Cigna Transthoracic Echocardiography in Adults - (0510) Form

The following Coverage Policy applies to health benefit plans administered by Cigna Companies. Certain Cigna Companies and/or lines of business only provide utilization review services to clients and do not make coverage determinations. References to standard benefit plan language and coverage determinations do not apply to those clients. Coverage Policies are intended to provide guidance in interpreting certain standard benefit plans administered by Cigna Companies. Please note, the terms of a customer’s particular benefit plan document [Group Service Agreement, Evidence of Coverage, Certificate of Coverage, Summary Plan Description (SPD) or similar plan document] may differ significantly from the standard benefit plans upon which these Coverage Policies are based. For example, a customer’s benefit plan document may contain a specific exclusion related to a topic addressed in a Coverage Policy. In the event of a conflict, a customer’s benefit plan document always supersedes the information in the Coverage Policies. In the absence of a controlling federal or state coverage mandate, benefits are ultimately determined by the terms of the applicable benefit plan document. Coverage determinations in each specific instance require consideration of 1) the terms of the applicable benefit plan document in effect on the date of service; 2) any applicable laws/regulations; 3) any relevant collateral source materials including Coverage Policies and; 4) the specific facts of the particular situation. Each coverage request should be reviewed on its own merits. Medical directors are expected to exercise clinical judgment where appropriate and have discretion in making individual coverage determinations. Where coverage for care or services does not depend on specific circumstances, reimbursement will only be provided if a requested service(s) is submitted in accordance with the relevant criteria outlined in the applicable Coverage Policy, including covered diagnosis and/or procedure code(s). Reimbursement is not allowed for services when billed for conditions or diagnoses that are not covered under this Coverage Policy (see “Coding Information” below). When billing, providers must use the most appropriate codes as of the effective date of the submission. Claims submitted for services that are not accompanied by covered code(s) under the applicable Coverage Policy will be denied as not covered. Coverage Policies relate exclusively to the administration of health benefit plans. Coverage Policies are not recommendations for treatment and should never be used Medical Coverage Policy: 0510 as treatment guidelines. In certain markets, delegated vendor guidelines may be used to support medical necessity and other coverage determinations. This Coverage Policy addresses non-stress transthoracic echocardiography (TTE) in an adult age 18 and older. Coverage Policy Nonvalvular heart disease INITIAL EVALUATION OF AN ASYMPTOMATIC PATIENT in Nonvalvular Heart Disease Transthoracic echocardiography (TTE) (with or without three-dimensional [3D]; with contrast as needed) is considered medically necessary: Initial cardiac evaluation of a known systemic, congenital, or acquired disease that could be associated with structural heart disease Screening evaluation for structure and function in first-degree relatives of a patient with an inherited cardiomyopathy Initial evaluation prior to exposure to medications/radiation that could result in cardiotoxicity/heart failure Pre-operative evaluation of cardiac structure and function prior to non-cardiac solid organ transplantation Evaluation of the ascending aorta in the setting of a known or suspected connective tissue disease or genetic condition that predisposes to aortic aneurysm or dissection (e.g., Marfan syndrome, Loeys-Dietz syndrome, Turner's syndrome) Screening evaluation in relatives of a patient with known aortic aneurysm or dissection or carriers of same genetic variant Preparticipation assessment of an asymptomatic athlete with ≥1 of the following: abnormal examination, abnormal electrocardiogram (ECG), or definite (or high suspicion for) family history of inheritable heart disease Evaluation of suspected pulmonary arterial hypertension, including evaluation of right ventricular function and estimated pulmonary artery pressure in a patient at risk for developing pulmonary arterial hypertension Individual taking FINTEPLA® (fenfluramine) for a FDA-approved indication (e.g., Dravet syndrome) TTE (with or without 3D; with contrast as needed) is not covered or reimbursable: Preparticipation athlete assessment in a patient with no symptoms, normal examination, and no family history of inheritable heart disease As a screening study prior to starting Attention-deficit/Hyperactivity disorder (ADHD) drugs Infrequent atrial premature contractions (APCs), infrequent ventricular premature contractions (VPCs) without other evidence of heart disease, or asymptomatic isolated sinus bradycardia Medical Coverage Policy: 0510 INITIAL EVALUATION OF A PATIENT WITH CLINICAL SIGNS AND/OR SYMPTOMS in Nonvalvular Heart Disease TTE (with or without 3D; with contrast as needed) is considered medically necessary: Initial evaluation when symptoms or signs suggest heart disease Arrhythmias or Conduction Disorders Newly diagnosed left bundle branch block (LBBB) • Newly diagnosed right bundle branch block (RBBB) • Frequent ventricular premature contractions (VPCs) without other evidence of heart disease Nonsustained ventricular tachycardia (VT) • Sustained VT or ventricular fibrillation (VF) • Evaluation of the patient with episodes of supraventricular tachycardia (SVT) without other evidence of heart disease Atrial fibrillation/flutter (not for purposes of Precardioversion evaluation) Palpitations/Presyncope/Syncope Clinical symptoms or signs consistent with a cardiac diagnosis known to cause presyncope/syncope (including but not limited to hypertrophic cardiomyopathy and heart failure [HF]) Palpitations without other symptoms or signs of cardiovascular disease • Presyncope without other symptoms or signs of cardiovascular disease • Syncope without other symptoms or signs of cardiovascular disease Hypotension or Hemodynamic Instability Hypotension or hemodynamic instability of uncertain or suspected cardiac etiology • Assessment of volume status in a critically ill patient Hypertensive Heart Disease Initial evaluation of suspected hypertensive heart disease Acute Coronary Syndrome (ACS) Evaluation of left ventricular (LV) function during initial presentation with acute coronary syndrome Suspected complication of myocardial ischemia/ infarction, including but not limited to acute mitral regurgitation, ventricular septal defect, free-wall rupture/tamponade, shock, right ventricular involvement, HF, or intraventricular thrombus Respiratory Failure/Exertional Shortness of Breath Exertional shortness of breath/dyspnea or hypoxemia of uncertain etiology Heart Failure/Cardiomyopathy Initial evaluation of known or suspected HF (systolic or diastolic) based on symptoms, signs, or abnormal test results to assess systolic or diastolic function and to assess for possible etiology (coronary artery disease [CAD], valvular disease) Suspected inherited or acquired cardiomyopathy (e.g., restrictive, infiltrative, dilated, hypertrophic) Evaluation of LV function in patients who are scheduled for or who have received chemotherapy Evaluation for acute chest pain and suspected myopericarditis for assessment of ventricular function and wall motion Pulmonary Hypertension Evaluation of suspected pulmonary hypertension including evaluation of right ventricular function and estimated pulmonary artery pressure Device Therapy Evaluation after appropriate time interval following revascularization and/or optimal medical therapy to determine candidacy for implantable cardioverter-defibrillator (ICD)/ cardiac resynchronization therapy (CRT) and/or to determine optimal choice of device Initial evaluation for CRT device optimization after implantation Medical Coverage Policy: 0510 Known implanted pacing/ ICD/CRT device with symptoms possibly due to suboptimal device settings To determine candidacy for ventricular assist device • Optimization of ventricular assist device settings Cardiac Transplantation Monitoring for rejection or coronary arteriopathy in a cardiac transplant recipient • Cardiac structure and function evaluation in a potential heart donor Other Suspected pericardial diseases • Initial evaluation of cardiac mass, suspected tumor or thrombus, or potential cardiac source of emboli Suspected acute aortic pathology including acute aortic syndrome • Multisystem Inflammatory Syndrome (MIS) associated with SARS-CoV-2 (COVID-19) infection TTE (with or without 3D; with contrast as needed) is not covered or reimbursable: Hypertensive Heart Disease Routine evaluation of systemic hypertension without symptoms or signs of hypertensive heart disease Respiratory Failure/Exertional Shortness of Breath Exertional shortness of breath /dyspnea or hypoxemia when a noncardiac etiology of dyspnea has been established SEQUENTIAL OR FOLLOW-UP TESTING TO CLARIFY INITIAL DIAGNOSTIC TESTING in Nonvalvular Heart Disease TTE (with or without 3D; with contrast as needed) is not covered or reimbursable: Further anatomic characterization of anomalous coronary arteries identified by invasive coronary angiography SEQUENTIAL OR FOLLOW-UP TESTING: ASYMPTOMATIC OR STABLE SYMPTOMS in Nonvalvular Heart Disease TTE (with or without 3D; with contrast as needed) is considered medically necessary: Re-evaluation in 6 months after initial TTE in connective tissue disease or genetic condition that predisposes to aortic aneurysm or dissection, to determine the rate of aortic growth; if the aortic diameters are stable, an annual surveillance TTE is recommended Re-evaluation (<1 y) in a patient previously or currently undergoing therapy with potentially cardiotoxic agents Re-evaluation (≥1 y) of known moderate or greater pulmonary hypertension without change in clinical status or cardiac examination Re-evaluation of chronic asymptomatic pericardial effusion when findings would potentially alter therapy Re-evaluation of intracardiac mass when findings would potentially alter therapy TTE (with or without 3D; with contrast as needed) is not covered or reimbursable: Re-evaluation (<1 y) in a patient at risk for heart failure (HF) without structural heart disease on prior TTE and no change in clinical status or cardiac examination (stage A) • Re-evaluation of known hypertensive heart disease without a change in clinical status or cardiac examination (stage A) (<1 y) Medical Coverage Policy: 0510 Re-evaluation (<1 y) of HF (systolic or diastolic) cardiomyopathy or HF without a change in clinical status or cardiac examination Re-evaluation (<1 y) of known aortic dilatation at baseline study to assess changes in rate of expansion or size in patient without bicuspid aortic valve Re-evaluation (<1 y) of known moderate or greater pulmonary hypertension without change in clinical status or cardiac examination Further clarification of suspected pericardial constriction when findings of TTE including tissue Doppler is unclear Re-evaluation of prior TEE findings for interval change (e.g., resolution of atrial thrombus after anticoagulation) when no change in therapy is anticipated. SEQUENTIAL OR FOLLOW-UP TESTING: NEW OR WORSENING SYMPTOMS OR TO GUIDE THERAPY in Nonvalvular Heart Disease TTE (with or without 3D; with contrast as needed) is considered medically necessary: • Re-evaluation of known structural heart disease with change in clinical status or cardiac examination or to guide therapy (assume ischemic work-up has been performed and remains valid) Re-evaluation of prior TEE findings for interval change (e.g., reduction or resolution of atrial thrombus after anticoagulation or intracardiac evaluation of cardiac mass when a change in therapy is anticipated Re-evaluation of known cardiomyopathy with a change in clinical status or cardiac examination or to guide therapy (assume ischemic work-up has been done, performed, and remains valid) Re-evaluation of known HF (systolic or diastolic) with a change in clinical status or cardiac examination without a clear precipitating change in medication or diet • Periodic re-evaluation in a patient undergoing therapy with cardiotoxic agents and worsening symptoms Re-evaluation after revascularization and/or optimal medical therapy to determine candidacy for device therapy and/ or to determine optimal choice of device Re-evaluation for CRT device optimization in a patient with worsening HF (*Gated-SPECT for this indication only) Re-evaluation for ventricular assist device-related complication or infection is suspected (*FDG PET in this indication is for infection detection) Re-evaluation for progression of pericardial effusion size or development of tamponade • Re-evaluation for progression of pericardial constriction • Re-evaluation of known ascending aortic dilatation or history of aortic dissection with a change in clinical status (excluding acute coronary syndrome) or cardiac examination or when findings may alter management or therapy Re-evaluation of known pulmonary hypertension with change in clinical status or cardiac examination or to guide therapy Evaluation of patient with pericardial mass and symptoms suggestive of expansion • Individual taking FINTEPLA® (fenfluramine) for a FDA-approved indication (e.g., Dravet syndrome) Multisystem Inflammatory Syndrome (MIS) associated with SARS-CoV-2 (COVID-19) infection TTE (with or without 3D; with contrast as needed) is not covered or reimbursable: • Re-evaluation of known HF (systolic or diastolic) with a change in clinical status or cardiac examination with a clear precipitating change in medication or diet Medical Coverage Policy: 0510 UNDERGOING TRANSCATHETER INTERVENTION, IMAGING FOR THE EVALUATION OF TRANSIENT ISCHEMIC ATTACK (TIA) OR ISCHEMIC STROKE in Nonvalvular Heart Disease TTE (with agitated saline injection; with or without 3D; with contrast as needed) is considered medically necessary: Initial evaluation of patient to exclude cardiac origin of TIA or ischemic stroke: (Intracardiac masses (thrombus, vegetation);Valvular pathology Provocative maneuvers (Valsalva, cough) to assess for the presence of: Right-to-left intracardiac shunt IMAGING FOR THE EVALUATION OF PATENT FORAMEN OVALE (PFO) OR ATRIAL SEPTAL DEFECT (ASD), PREPROCEDURAL EVALUATION FOR CLOSURE OF PFO OR ASD in Nonvalvular Heart Disease TTE (with agitated saline injection; with or without 3D; with contrast as needed) is considered medically necessary: Preprocedure assessment for PFO: Atrial appendage thrombus; Spontaneous echo contrast (slow blood flow); Aortic atheroma; Cardiac masses; Vegetations • Preprocedure assessment for: Atrial septum anatomy; Atrial septum aneurysm; Suitability for percutaneous device closure IMAGING FOR THE EVALUATION OF PATENT FORAMEN OVALE (PFO) OR ATRIAL SEPTAL DEFECT (ASD), INTRA-PROCEDURAL GUIDANCE FOR CLOSURE OF PFO OR ASD in Nonvalvular Heart Disease TTE (with agitated saline injection; with or without 3D; with contrast as needed) is not covered or reimbursable: Intraprocedural guidance in patient with either: ASD of simple anatomy; No aneurysmal atrial septum; PFO with short tunnel Intraprocedural guidance in patient with either: ASD with complex anatomy; Aneurysmal interatrial septum; PFO with long tunnel IMAGING FOR THE EVALUATION OF PATENT FORAMEN OVALE (PFO) OR ATRIAL SEPTAL DEFECT (ASD), ASSESSMENT FOLLOWING CLOSURE OF PFO OR ASD in Nonvalvular Heart Disease TTE (with agitated saline injection; with or without 3D; with contrast as needed) is considered medically necessary: 30 days and 6-month routine scheduled follow-up ASD/PFO device closure for position of device and integrity of device; PFO patency; Thrombus formation Nonroutine follow up of ASD/PFO device closure and clinical concern for infection, malposition, embolization or persistent shunt. IMAGING FOR THE EVALUATION OF LEFT ATRIAL APPENDAGE (LAA) OCCLUSION DEVICE, EVALUATION BEFORE, DURING, AFTER LAA OCCLUSION in Nonvalvular Heart Disease TTE (with or without 3D; with contrast as needed) is considered medically necessary: Pre-Procedural evaluation for LAA occlusion • Intraprocedural guidance for LAA occlusion Medical Coverage Policy: 0510 TTE (with or without 3D) is considered medically necessary: • Prior to discharge for Assessment following LAA occlusion • Surveillance at 45 days or FDA guidance/guidelines following LAA occlusion Valvular heart disease INITIAL EVALUATION OF AN ASYMPTOMATIC PATIENT In Valvular Heart Disease (VHD) Transthoracic echocardiography (TTE) (without contrast) is considered medically necessary: Unexplained murmur or abnormal heart sounds • Reasonable suspicion of valvular heart disease (VHD) • History of rheumatic heart disease • Known systemic or acquired disease associated with VHD • First-degree family history of a bicuspid aortic valve • Exposure to medications that could result in development of VHD 3D TTE is not covered or reimbursable: Unexplained murmur or abnormal heart sounds • Reasonable suspicion of valvular heart disease (VHD) • History of rheumatic heart disease • Known systemic or acquired disease associated with VHD • First-degree family history of a bicuspid aortic valve • Exposure to medications that could result in development of VHD INITIAL EVALUATION OF A PATIENT WITH CLINICAL SIGNS AND/OR SYMPTOMS In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Arrhythmias Palpitations AND No other symptoms or signs of cardiovascular disease Presyncope/Syncope Presyncope AND No other symptoms or signs of cardiovascular disease • Syncope AND No other symptoms or signs of cardiovascular disease Hypotension or Hemodynamic Instability Hypotension or hemodynamic instability AND Uncertain or suspected cardiac etiology • Assessment of volume status in a critically ill patient • Suspected acute mitral or aortic regurgitation Respiratory Failure Respiratory failure or hypoxemia of uncertain etiology Heart Failure (HF) Initial evaluation in patients presented with HF to exclude the presence of primary or secondary valve disease Bacteremia/Endocarditis Suspected infective endocarditis (IE) (native valve, prosthetic valve, endocardial lead) Cardiac Mass/Cardiac Source of Emboli Suspected cardiac mass, suspected tumor or thrombus, or potential cardiac source of emboli Medical Coverage Policy: 0510 TTE (without contrast) is not covered or reimbursable: Respiratory Failure Respiratory failure or hypoxemia AND Noncardiac etiology of respiratory failure has been established 3D TTE is considered medically necessary: Hypotension or Hemodynamic Instability Suspected acute mitral or aortic regurgitation Cardiac Mass/Cardiac Source of Emboli Suspected cardiac mass, suspected tumor or thrombus, or potential cardiac source of emboli 3D TTE is not covered or reimbursable: Arrhythmias Palpitations AND No other symptoms or signs of cardiovascular disease Presyncope/Syncope Presyncope AND No other symptoms or signs of cardiovascular disease • Syncope AND No other symptoms or signs of cardiovascular disease Hypotension or Hemodynamic Instability Hypotension or hemodynamic instability AND Uncertain or suspected cardiac etiology • Assessment of volume status in a critically ill patient Respiratory Failure Respiratory failure or hypoxemia of uncertain etiology • Respiratory failure or hypoxemia AND Noncardiac etiology of respiratory failure has been established Heart Failure (HF) Initial evaluation in patients presented with HF to exclude the presence of primary or secondary valve disease Bacteremia/Endocarditis Transient fever AND No evidence of bacteremia or a new murmur • Transient bacteremia AND Pathogen not typically associated with IE and/or a documented nonendovascular source or infection PRIOR TESTING, ADDITIONAL TESTING TO CLARIFY DIAGNOSIS In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Valvular Mass Further evaluation of valvular mass (including incidental findings noted on noncardiac imaging studies) TTE (with contrast) is considered medically necessary: Inadequate TTE Images Inadequate TTE images for the evaluation of possible valvular heart disease due to patient characteristics TTE (with contrast) is not covered or reimbursable: Inadequate TTE Images Characterization of native or prosthetic valves with clinical signs or symptoms suggesting valve dysfunction Suspected Endocarditis With Negative TTE Aortic Stenosis (AS) Medical Coverage Policy: 0510 Symptomatic, severe aortic stenosis (AS) by calculated valve area (stage D2) AND Low flow/low gradient AND Low left ventricular ejection fraction (LVEF) Severe AS, by calculated valve area AND Low flow/low gradient AND Preserved LVEF and for assessment of morphology, including calcification Moderate or asymptomatic severe AS (stages B and C), for measurement of changes in valve hemodynamics with exercise or pharmacological stress Symptomatic severe AS (stage D), for measurement of changes in valve hemodynamics with exercise or pharmacological stress Mitral Stenosis Discrepancy between resting Doppler echocardiographic findings and clinical symptoms or signs to evaluate mean mitral gradient and pulmonary artery pressure Mitral Regurgitation (MR) Severe mitral regurgitation (MR) suspected clinically AND Potentially underestimated on TTE despite optimal images OR Better imaging of MR jet needed Chronic symptomatic primary MR with discrepancy between exertional symptoms and the severity of MR at rest OR Symptoms are disproportionate to the severity of MR determined at rest Chronic asymptomatic patient, to distinguish between moderate or severe primary MR • Chronic secondary MR (stages B to D), to establish etiology, including a possible ischemic etiology Chronic secondary MR (stages B to D), to assess myocardial viability Aortic Regurgitation (AR) Dilated aortic sinuses or ascending aorta or a bicuspid aortic valve (stages A and B), to evaluate the presence and severity of AR assuming optimal TTE images • Discordance between clinical assessment and TTE about the severity of AR • Assessment of symptoms and functional capacity in patients with moderate or severe AR Other Valvular Regurgitation Severe tricuspid regurgitation (stages C and D) and suboptimal TTE images, for assessment of RV systolic function and systolic and diastolic volumes Assessment of pulmonary pressures during stress in patient with severe asymptomatic valve regurgitation prior to pregnancy Valvular Mass Further evaluation of valvular mass (including incidental findings noted on noncardiac imaging studies) 3D TTE is considered medically necessary: Mitral Regurgitation (MR) Severe mitral regurgitation (MR) suspected clinically AND Potentially underestimated on TTE despite optimal images OR Better imaging of MR jet needed Chronic secondary MR (stages B to D), to establish etiology, including a possible ischemic etiology Valvular Mass Further evaluation of valvular mass (including incidental findings noted on noncardiac imaging studies) 3D TTE is not covered or reimbursable: Inadequate TTE Images Inadequate TTE images for the evaluation of possible valvular heart disease due to patient characteristics Characterization of native or prosthetic valves with clinical signs or symptoms suggesting valve dysfunction Suspected Endocarditis With Negative TTE Medical Coverage Policy: 0510 Suspected infective endocarditis (IE) with moderate to high pretest probability (i.e., staph bacteremia, fungemia, prosthetic heart valve, or intracardiac device) Aortic Stenosis (AS) Symptomatic, severe aortic stenosis (AS) by calculated valve area (stage D2) AND Low flow/low gradient AND Low left ventricular ejection fraction (LVEF) Severe AS, by calculated valve area AND Low flow/low gradient AND Preserved LVEF and for assessment of morphology, including calcification Moderate or asymptomatic severe AS (stages B and C), for measurement of changes in valve hemodynamics with exercise or pharmacological stress Symptomatic severe AS (stage D), for measurement of changes in valve hemodynamics with exercise or pharmacological stress Mitral Stenosis Discrepancy between resting Doppler echocardiographic findings and clinical symptoms or signs to evaluate mean mitral gradient and pulmonary artery pressure Mitral Regurgitation (MR) Chronic symptomatic primary MR with discrepancy between exertional symptoms and the severity of MR at rest OR Symptoms are disproportionate to the severity of MR determined at rest Chronic asymptomatic patient, to distinguish between moderate or severe primary MR • Chronic secondary MR (stages B to D), to assess myocardial viability Aortic Regurgitation (AR) Dilated aortic sinuses or ascending aorta or a bicuspid aortic valve (stages A and B), to evaluate the presence and severity of AR assuming optimal TTE images • Discordance between clinical assessment and TTE about the severity of AR • Assessment of symptoms and functional capacity in patients with moderate or severe AR Other Valvular Regurgitation Severe tricuspid regurgitation (stages C and D) and suboptimal TTE images, for assessment of RV systolic function and systolic and diastolic volumes Assessment of pulmonary pressures during stress in patient with severe asymptomatic valve regurgitation prior to pregnancy PRIOR TESTING, SEQUENTIAL OR FOLLOW-UP TESTING: ASYMPTOMATIC OR STABLE SYMPTOMS In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Stage A VHD Routine surveillance (every 3–5 y) for patients with stage A (bicuspid aortic valve (AV) or aortic sclerosis) for exclusion of progression to stage B. Mild or Moderate VHD Re-evaluation (3–5 y) of mild (stage B) valvular regurgitation • Re-evaluation (1–2 y) of moderate (stage B) VHD without a change in clinical status of cardiac examination Re-evaluation (<1 y) in patients with moderate AS who will be subjected to increased hemodynamic demands (e.g., noncardiac surgery, pregnancy) Severe VHD Re-evaluation (6–12 m) of asymptomatic severe (stage C1) aortic stenosis (AS) without a change in clinical status or cardiac examination Re-evaluation (every 1 y) for asymptomatic (stage C1) patients with AS • Re-evaluation (6–12 m) of stage C1 patients with asymptomatic severe aortic regurgitation (AR) with preserved ejection fraction and normal LV size Re-evaluation (<1 y) in patients with severe AS who will be subjected to increased hemodynamic demands (e.g., noncardiac surgery, pregnancy) Medical Coverage Policy: 0510 Re-evaluation after control of hypertension in patients with low-flow/low-gradient severe AS with preserved left ventricular ejection fraction (LVEF) Re-evaluation (6–12 m) of asymptomatic severe (stage B) mitral valve regurgitation (MR) Re-evaluation (every 6–12 m) of stage C1 patients with asymptomatic severe mitral regurgitation (MR) Dilated Thoracic Aorta Re-evaluation in 6 to 12 months is reasonable to determine the rate of aortic enlargement; if stable, surveillance imaging every 6 to 24 months (depending on aortic diameter) Endocarditis Re-evaluation of prior TTE/TEE finding for interval change (e.g., resolution of vegetation after antibiotic therapy) when a change in therapy is anticipated Re-evaluation of patient with infective endocarditis (IE) at high risk of progression or complications (e.g., extensive infective tissue/ large vegetation on initial echocardiogram, or staphylococcal, enterococcal, or fungal infections) in the absence of clinical change TTE (without contrast) is not covered or reimbursable: Mild or Moderate VHD Re-evaluation (1–2 y) of mild (stage B) VHD without a change in clinical status or cardiac examination Endocarditis Re-evaluation of prior TTE/TEE finding for interval change (e.g., resolution of vegetation after antibiotic therapy) when no change in therapy is anticipated 3D TTE is not covered or reimbursable: Stage A VHD Routine surveillance (every 3–5 y) for patients with stage A (bicuspid aortic valve (AV) or aortic sclerosis) for exclusion of progression to stage B. Mild or Moderate VHD Re-evaluation (3–5 y) of mild (stage B) valvular regurgitation • Re-evaluation (1–2 y) of mild (stage B) VHD without a change in clinical status or cardiac examination Re-evaluation (1–2 y) of moderate (stage B) VHD without a change in clinical status of cardiac examination Re-evaluation (<1 y) in patients with moderate AS who will be subjected to increased hemodynamic demands (e.g., noncardiac surgery, pregnancy) Severe VHD Re-evaluation (6–12 m) of asymptomatic severe (stage C1) aortic stenosis (AS) without a change in clinical status or cardiac examination Re-evaluation (every 1 y) for asymptomatic (stage C1) patients with AS • Re-evaluation (6–12 m) of stage C1 patients with asymptomatic severe aortic regurgitation (AR) with preserved ejection fraction and normal LV size Re-evaluation (<1 y) in patients with severe AS who will be subjected to increased hemodynamic demands (e.g., noncardiac surgery, pregnancy) Re-evaluation after control of hypertension in patients with low-flow/low-gradient severe AS with preserved left ventricular ejection fraction (LVEF) Re-evaluation (6–12 m) of asymptomatic severe (stage B) mitral valve regurgitation • Re-evaluation (every 6–12 m) of stage C1 patients with asymptomatic severe mitral regurgitation (MR) Medical Coverage Policy: 0510 Endocarditis Re-evaluation of prior TTE/TEE finding for interval change (e.g., resolution of vegetation after antibiotic therapy) when no change in therapy is anticipated Re-evaluation of prior TTE/TEE finding for interval change (e.g., resolution of vegetation after antibiotic therapy) when a change in therapy is anticipated Re-evaluation of patient with infective endocarditis (IE) at high risk of progression or complications (e.g., extensive infective tissue/ large vegetation on initial echocardiogram, or staphylococcal, enterococcal, or fungal infections) in the absence of clinical change PRIOR TESTING, SEQUENTIAL OR FOLLOW-UP TESTING OF NEW OR WORSENING SYMPTOMS OR TO GUIDE THERAPY In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: General Re-evaluation of known VHD with a change in clinical status or cardiac examination or to guide therapy Endocarditis Re-evaluation of infective endocarditis (IE) in a patient with a change in clinical status or cardiac examination (e.g., new murmur, embolism, persistent fever, heart failure (HF), abscess, or atrioventricular heart block) 3D TTE is not covered or reimbursable: General Re-evaluation of known VHD with a change in clinical status or cardiac examination or to guide therapy Endocarditis Re-evaluation of infective endocarditis (IE) in a patient with a change in clinical status or cardiac examination (e.g., new murmur, embolism, persistent fever, heart failure (HF), abscess, or atrioventricular heart block) PRIOR TESTING, POSTOPERATIVE IMAGING AFTER SURGICAL VALVE REPLACEMENT OR REPAIR In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Surgical Valve Replacement (No or Stable Symptoms) Initial postoperative evaluation of bioprosthetic or mechanical valve for establishment of baseline (6 wk to 3 mo postoperative) Re-evaluation in patients with a bioprosthetic valve at 5 years, at 10 years, and then annually Surgical Valve Replacement (Suspicion of Valve Dysfunction) Characterization of mechanical prosthetic valve if clinical signs or symptoms suggest valve dysfunction Characterization of bioprosthetic valve if clinical signs or symptoms suggest valve dysfunction Re-evaluation of known prosthetic valve dysfunction when it would change management or guide therapy Evaluation of documented prosthetic valve IE when medical management is considered, in a patient who is at high risk for progression or complication or with a change in clinical status or cardiac examination Surgical Mitral Valve Repair Medical Coverage Policy: 0510 Initial postoperative assessment of valve repair (6 wk to 3 mo postoperatively) • Re-evaluation (≥3 y) in patients without suspected repaired valve dysfunction • Re-evaluation (<3 y) for suspected repaired valve dysfunction Transcatheter Mitral Valve Repair Initial postoperative assessment of valve repair • Re-evaluation yearly in asymptomatic individuals • Re-evaluation if clinical signs or symptoms suggest valve dysfunction TTE (without contrast) is not covered or reimbursable: Surgical Mitral Valve Repair Re-evaluation (<3 y) in patients without suspected repaired valve dysfunction 3D TTE is considered medically necessary: Surgical Valve Replacement (Suspicion of Valve Dysfunction) Characterization of mechanical prosthetic valve if clinical signs or symptoms suggest valve dysfunction Characterization of bioprosthetic valve if clinical signs or symptoms suggest valve dysfunction Re-evaluation of known prosthetic valve dysfunction when it would change management or guide therapy Evaluation of documented prosthetic valve IE when medical management is considered, in a patient who is at high risk for progression or complication or with a change in clinical status or cardiac examination Mitral Valve Repair (transcatheter and surgical) Re-evaluation for suspected repaired valve dysfunction 3D TTE is not covered or reimbursable: Surgical Valve Replacement (No or Stable Symptoms) Initial postoperative evaluation of bioprosthetic or mechanical valve for establishment of baseline (6 wk to 3 mo postoperative) Re-evaluation in patients with a bioprosthetic or mechanical valve who are asymptomatic and/or clinically stable Surgical Valve Replacement (Suspicion of Valve Dysfunction) Characterization of bioprosthetic valve if suspected clinically significant valvular dysfunction and inadequate images from TTE or TEE Characterization of mechanical prosthetic valve if suspected clinically significant valvular dysfunction and inadequate images from TTE or TEE Mitral Valve Repair (transcatheter and surgical) • Re-evaluation in individuals who are asymptomatic and/or clinically stable Initial postoperative assessment of valve repair TRANSCATHETER INTERVENTION FOR VHD, PRE-TRANSCATHETER AORTIC VALVE REPLACEMENT (TAVR)/ TRANSCATHETER AORTIC VALVE IMPLANTATION (TAVI) EVALUATION In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: • Assessment of number of cusps and degree of calcification TTE (without contrast) is not covered or reimbursable: • Assessment for concomitant coronary artery disease Medical Coverage Policy: 0510 Accurate assessment of annular size and shape* *Multimodality imaging might improve the accuracy of the measurements Measurement of the distance between annulus and the coronary ostia • Precise coaxial alignment of the implant within the centerline of the aortic valve • Assessment of aortic dimensions • Assessment of aortic atherosclerotic burden • Assessment of iliofemoral vessels 3D TTE is considered medically necessary: Assessment of number of cusps and degree of calcification 3D TTE is not covered or reimbursable: Assessment for concomitant coronary artery disease • Accurate assessment of annular size and shape* *Multimodality imaging might improve the accuracy of the measurements Measurement of the distance between annulus and the coronary ostia • Precise coaxial alignment of the implant within the centerline of the aortic valve • Assessment of aortic dimensions • Assessment of aortic atherosclerotic burden • Assessment of iliofemoral vessels TRANSCATHETER INTERVENTION FOR VHD, INTRAPROCEDURAL EVALUATION DURING TRANSCATHETER AORTIC VALVE REPLACEMENT (TAVR)/ TRANSCATHETER AORTIC VALVE IMPLANTATION (TAVI) In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Guidewire placement into the LV • Valve placement • Postdeployment assessment (position, function, regurgitation) • Evaluate immediate complications • Hypotension • Coronary occlusion • LV depression from rapid pacing • LV outflow tract obstruction • Severe MR • Prosthesis dislodgment • Tamponade • Right ventricular perforation • Air embolism • Aortic dissection (paravalvular leak needs to be excluded) 3D TTE is considered medically necessary: Guidewire placement into the LV • Valve placement • Postdeployment assessment (position, function, regurgitation) • Evaluate immediate complications • Hypotension • Coronary occlusion • LV depression from rapid pacing • LV outflow tract obstruction • Severe MR • Prosthesis dislodgment Medical Coverage Policy: 0510 Tamponade • Right ventricular perforation • Air embolism • Aortic dissection (paravalvular leak needs to be excluded) TRANSCATHETER INTERVENTION FOR VHD, POSTPROCEDURAL ASSESSMENT AFTER TAVR/TAVI In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Assessment of degree of aortic regurgitation (including valvular and paravalvular) with suspicion of valve dysfunction Assessment of stroke with suspicion of valve dysfunction • Assessment within the immediate post-procedure period, at 30 days, at one year, and then annually regardless of symptoms 3D TTE is considered medically necessary: Assessment of degree of aortic regurgitation (including valvular and paravalvular) with suspicion of valve dysfunction 3D TTE is not covered or reimbursable: Assessment of stroke with suspicion of valve dysfunction TRANSCATHETER INTERVENTION FOR VHD, EVALUATION PRIOR TO PERCUTANEOUS MITRAL VALVE REPAIR In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Determine patient eligibility* *Currently, MitraClip is the only FDA-approved device available. TTE (without contrast) is not covered or reimbursable: Exclude the presence of intracardiac mass, thrombus, or vegetation prior to (within 3 d of the procedure) 3D TTE is considered medically necessary: Determine patient eligibility* *Currently, MitraClip is the only FDA-approved device available. Exclude the presence of intracardiac mass, thrombus, or vegetation prior to (within 3 d of the procedure) TRANSCATHETER INTERVENTION FOR VHD, INTRAPROCEDURAL EVALUATION DURING PERCUTANEOUS MITRAL VALVE REPAIR In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Assess for presence of mitral stenosis TTE (without contrast) is not covered or reimbursable: Alignment of the device over the origin of the regurgitant jet and advance to the LV • Guidance for grasping the mitral valve leaflets and device closure • Assess for adequacy in the reduction of the MR Medical Coverage Policy: 0510 3D TTE is considered medically necessary: Alignment of the device over the origin of the regurgitant jet and advance to the LV • Guidance for grasping the mitral valve leaflets and device closure • Assess for adequacy in the reduction of the MR • Assess for presence of mitral stenosis TRANSCATHETER INTERVENTION FOR VHD, POSTPROCEDURAL ASSESSMENT AFTER PERCUTANEOUS MITRAL VALVE REPAIR (OUT OF PROCEDURE) In Valvular Heart Disease (VHD) TTE (without contrast) is considered medically necessary: Reassessment for degree of MR and left ventricular function (predischarge at 1, 6, and 12 mo, and then annually to 5 y) 3D TTE is considered medically necessary: Reassessment for degree of MR and left ventricular function (predischarge at 1, 6, and 12 mo, and then annually to 5 y) Congenital heart disease ESTABLISHED CONGENITAL HEART DISEASE Transthoracic echocardiography (TTE) is considered medically necessary according to the American College of Cardiology (ACC) 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease (detailed in the General Background below), which may include: Patent foramen ovale (PFO) • Atrial septal defects • Partial anomalous pulmonary venous connection • Ventricular septal defects • Atrioventricular septal defects • Patent ductus arteriosus • Total anomalous pulmonary venous connection • Eisenmenger Syndrome • Pulmonary hypertension associated with congenital heart disease • Ebstein anomaly • Tricuspid valve dysplasia • Pulmonary stenosis • Pulmonary atresia with intact ventricular septum • Mitral valve disease • Left ventricular outflow tract (LVOT) lesions • Aortic coarctation and Interrupted aortic arch • Coronary anomalies • Tetralogy of Fallot (TOF) • Double outlet right ventricle (DORV) • D-Loop transposition of the great arteries (D-Loop TGA) • Congenitally corrected transposition of the great arteries (ccTGA) • Truncus arteriosus • Single-ventricle heart disease Medical Coverage Policy: 0510 Pregnancy PREGNANCY Transthoracic echocardiography (TTE) (without contrast) is considered medically necessary for: Initial cardiac evaluation of a known systemic, congenital, or acquired disease that could be associated with structural heart disease in an individual who is considering becoming pregnant or is pregnant and has had no echocardiography within the past year Initial screening evaluation for structure and function in an individual who is considering becoming pregnant or is pregnant who has a relative with an inherited cardiomyopathy, known aortic aneurysm or dissection, connective tissue disease or genetic condition that predisposes to aortic aneurysm or dissection and has had no echocardiography within the past year. Evaluation prior to pregnancy in patients with a prosthetic valve and no echocardiography within the past year Reevaluation in an individual with an aortopathic condition or a dilated aortic root or ascending aorta, is recommended each trimester and again several weeks postpartum Frequency of TTE FREQUENCY OF TTE More than two transthoracic echocardiograms (TTE) submitted within a rolling twelve months are not covered or reimbursable, with the exception of: • diagnoses without frequency limits listed in the specified Coding section table below those diagnoses with frequency limits indicated in the policy above, OR Myocardial strain imaging MYOCARDIAL STRAIN IMAGING (CPT® 93356) using speckle tracking-derived assessment of myocardial mechanics Myocardial strain imaging is considered medically necessary if the primary TTE (93303, 93304, 93306, 93307, 93308) on the same date of service is medically necessary AND EITHER of the following criteria are met: prior to, during or following exposure to medications/radiation that could result in cardiotoxicity to evaluate hypertrophic cardiomyopathy Myocardial strain imaging is not covered or reimbursable for any other indication. Medical Coverage Policy: 0510 General Background Echocardiography is the most frequently employed cardiac imaging test for evaluation of cardiovascular disease related to a structural, functional or hemodynamic abnormality of the heart or great vessels. Echocardiography allows ultrasonic visualization of cardiac structures in real time from multiple planes, and Doppler and color flow imaging allows a reliable assessment of cardiac hemodynamics and blood flow. A transthoracic echocardiography (TTE) examination begins with real-time two dimensional (2D) echocardiography, which provides high-resolution images of cardiac structures and their movements. TTE technique has evolved from a simple M-mode tracing to a family of technologies that include 2D imaging, pulsed and continuous wave spectral Doppler, color flow Doppler, tissue Doppler, 3-dimensional (3D) imaging, and myocardial strain imaging using speckle tracking. Myocardial strain is the deformation produced by the application of a force; myocardial strain represents percent change in myocardial length from relaxed to contractile state. The main limitation remains that strain values vary among methods, modalities and software version. The most prevalent use of myocardial strain imaging evaluated in current literature is for identifying potential cancer therapy-related cardiac dysfunction. Myocardial strain imaging in individuals with exposure to medications/radiation that could result in cardiotoxicity is supported by the American College of Cardiology and current peer-reviewed literature (Oikonomou, et al., 2019; Amzulescu, et al., 2019; Thavendiranathan, et al., 2014). Diagnostic procedures used as alternatives to TTE for cardiac diagnosis and assessment vary, depending on the clinical situation and other factors, and may include electrocardiogram (ECG), chest x-ray, stress TTE, transesophageal echocardiography (TEE), magnetic resonance imaging (MRI), computed tomography (CT), computed tomography angiography (CTA), magnetic resonance angiography (MRA), single photon emission computed tomography (SPECT), coronary arteriography, and positron emission tomography (PET). In some cases TTE may be the sole diagnostic procedure, while in other situations additional testing is required. Although TTE is technically demanding, the diagnostic accuracy, cost effectiveness, availability, and noninvasive nature of the test have made it a powerful diagnostic tool in cardiology. Professional society recommendations have been published in an effort to guide appropriate use of this imaging modality for selected patient indications. Professional Societies/Organizations This Cigna Coverage Policy is primarily based upon the following American College of Cardiology (ACC) Appropriate Use Criteria (AUC): 1. Multimodality Imaging in the Assessment of Cardiac Structure and Function in Nonvalvular Heart Disease (Doherty, et al., 2019) 2. Multimodality Imaging in Valvular Heart Disease (Doherty, et al., 2017) 3. Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease (Sachdeva, et al., 2020) And the following ACC/American Heart Association (AHA) Guidelines: 1. Guideline for the Evaluation and Diagnosis of Chest Pain (Gulati, et al., 2021) 2. Guideline for the Management of Patients With Valvular Heart Disease (Otto, et al., 2021). 3. Guideline for the Management of Heart Failure (Heidenreich, et al., 2022) 4. Guideline for the Diagnosis and Management of Aortic Disease (2022) Medical Coverage Policy: 0510 2019 American College of Cardiology (ACC) Appropriate Use Criteria (AUC) for Multimodality Imaging in the Assessment of Cardiac Structure and Function in Nonvalvular Heart Disease The American College of Cardiology Appropriate Use Criteria Task Force, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for CardiovascularMagnetic Resonance, and the Society of Thoracic Surgeons published the 2019 Appropriate Use Criteria (AUC) for Multimodality Imaging in the Assessment of Cardiac Structure and Function in Nonvalvular Heart Disease (Doherty, et al., 2019). Noteworthy: • This document is the second of two companion appropriate use criteria (AUC) documents. The first document addresses the evaluation and use of multimodality imaging in the diagnosis and management of valvular heart disease (Doherty, et al., 2017) whereas this document addresses this topic with regard to structural (nonvalvular) heart disease (Doherty, et al., 2019). Includes 103 separate TTE indications Where appropriate, the scenarios were developed on the basis of the most current American College of Cardiology/American Heart Association Clinical Practice Guidelines. Ratings: A = Appropriate. Median Score 7 to 9: Appropriate test for specific indication (test is generally acceptable and is a reasonable approach for the indication). M = May be appropriate. Median Score 4 to 6: May Be Appropriate test for specific indication (test may be generally acceptable and may be a reasonable approach for the indication). May Be Appropriate also implies that more research and/or patient information is needed to classify the indication definitively. R = Rarely appropriate. Median Score 1 to 3: Rarely Appropriate test for specific indication (test is not generally acceptable and is not a reasonable approach for the indication). Table 1: Nonvalvular Heart Disease, Initial Evaluation of an Asymptomatic Patient Initial cardiac evaluation of a known systemic, congenital, or acquired disease that could be associated with structural heart disease Screening evaluation for structure and function in first- degree relatives of a patient with an inherited cardiomyopathy Initial evaluation prior to exposure to medications/radiation that could result in cardiotoxicity/heart failure Evaluation of the ascending aorta in the setting of a known or suspected connective tissue disease or genetic condition that predisposes to aortic aneurysm or dissection (e.g., Marfan syndrome) TTE (With or Without 3D; With Contrast as Needed) (9) A (9) A (9) A (8) A Strain/Strain Rate Imaging by Speckle or Tissue Doppler (5) M (4) M (7) A (1) R Medical Coverage Policy: 0510 Screening evaluation in relatives of a patient with known aortic aneurysm or dissection Preparticipation athlete assessment in a patient with no symptoms, normal examination, and no family history of inheritable heart disease Preparticipation assessment of an asymptomatic athlete with ≥1 of the following: abnormal examination, abnormal electrocardiogram (ECG), or definite (or high suspicion for) family history of inheritable heart disease Evaluation of suspected pulmonary arterial hypertension, including evaluation of right ventricular function and estimated pulmonary artery pressure in a patient at risk for developing pulmonary arterial hypertension TTE (With or Without 3D; With Contrast as Needed) (8) A (3) R (9) A (9) A Strain/Strain Rate Imaging by Speckle or Tissue Doppler (1) R (1) R (4) M (2) R Table 2: Nonvalvular Heart Disease, Initial Evaluation of a Patient With Clinical Signs and/or Symptoms of Heart Disease Initial evaluation when symptoms or signs suggest heart disease TTE (With or Without 3D; With Contrast as Needed) (9) A Strain/Strain Rate Imaging by Speckle or Tissue Doppler (5) M Arrhythmias or Conduction Disorders Newly diagnosed left bundle branch block (LBBB) Newly diagnosed right bundle branch block (RBBB) Frequent ventricular premature contractions (VPCs) without other evidence of heart disease Nonsustained ventricular tachycardia (VT) Sustained VT or ventricular fibrillation (VF) Evaluation of the patient with episodes of supraventricular tachycardia (SVT) without other evidence of heart disease Atrial fibrillation/flutter (not for purposes of Precardioversion evaluation) 7 (A) 5 (M) 7 (A) 8 (A) 9 (A) 6 (M) 8 (A) (4) M (2) R (2) R (4) M (3) R (1) R (3) R Palpitations/Presyncope/Syncope Clinical symptoms or signs consistent with a cardiac diagnosis known to cause presyncope/syncope (including but not limited to hypertrophic cardiomyopathy and heart failure [HF]) 9 (A) (4) M Medical Coverage Policy: 0510 Palpitations without other symptoms or signs of cardiovascular disease Presyncope without other symptoms or signs of cardiovascular disease Syncope without other symptoms or signs of cardiovascular disease Hypotension or Hemodynamic Instability Hypotension or hemodynamic instability of uncertain or suspected cardiac etiology Assessment of volume status in a critically ill patient Hypertensive Heart Disease Initial evaluation of suspected hypertensive heart disease Routine evaluation of systemic hypertension without symptoms or signs of hypertensive heart disease Acute Coronary Syndrome (ACS) Evaluation of left ventricular (LV) function during initial presentation with acute coronary syndrome Suspected complication of myocardial ischemia/ infarction, including but not limited to acute mitral regurgitation, ventricular septal defect, free-wall rupture/tamponade, shock, right ventricular involvement, HF, or intraventricular thrombus Respiratory Failure/Exertional Shortness of Breath Exertional shortness of breath/dyspnea or hypoxemia of uncertain etiology Exertional shortness of breath /dyspnea or hypoxemia when a noncardiac etiology of dyspnea has been established Heart Failure/Cardiomyopathy Initial evaluation of known or suspected HF (systolic or diastolic) based on symptoms, signs, or abnormal test results to assess systolic or diastolic function and to assess for possible etiology (coronary artery disease [CAD], valvular disease) Suspected inherited or acquired cardiomyopathy (e.g., restrictive, infiltrative, dilated, hypertrophic) Evaluation of LV function in patients who are scheduled for or who have received chemotherapy Pulmonary Hypertension Medical Coverage Policy: 0510 TTE (With or Without 3D; With Contrast as Needed) 6 (M) 7 (A) 8 (A) 8 (A) 7 (A) 8 (A) 5 (M) 8 (A) 9 (A) 8 (A) 4 (M) 9 (A) 9 (A) 9 (A) Strain/Strain Rate Imaging by Speckle or Tissue Doppler (2) R (2) R (2) R (1) R (1) R (3) R (1) R (1) R (1) R (4) M (1) R (6) M (6) M (6) M Evaluation of suspected pulmonary hypertension including evaluation of right ventricular function and estimated pulmonary artery pressure TTE (With or Without 3D; With Contrast as Needed) 9 (A) Strain/Strain Rate Imaging by Speckle or Tissue Doppler (3) R Device Therapy Evaluation after appropriate time interval following revascularization and/or optimal medical therapy to determine candidacy for implantable cardioverter- defibrillator (ICD)/ cardiac resynchronization therapy (CRT) and/or to determine optimal choice of device Initial evaluation for CRT device optimization after implantation Known implanted pacing/ ICD/CRT device with symptoms possibly due to suboptimal device settings To determine candidacy for ventricular assist device Optimization of ventricular assist device settings 9 (A) 7 (A) 8 (A) 9 (A) 8 (A) (2) R (3) R (4) M (1) R (1) R Cardiac Transplantation Monitoring for rejection or coronary arteriopathy in a cardiac transplant recipient Cardiac structure and function evaluation in a potential heart donor 8 (A) 9 (A) (4) M (1) R Other Suspected pericardial diseases Initial evaluation of cardiac mass, suspected tumor or thrombus, or potential cardiac source of emboli Suspected acute aortic pathology including acute aortic syndrome 9 (A) 9 (A) 7 (A) (5) M (1) R (1) R Table 3: Nonvalvular Heart Disease, Sequential or Follow-Up Testing to Clarify Initial Diagnostic Testing Comprehensive further evaluation of undefined cardiomyopathy Evaluation of suspected cardiac amyloidosis Evaluation of suspected hypertrophic cardiomyopathy Further anatomic characterization of anomalous coronary arteries identified by invasive coronary angiography TTE (With or Without 3D; With Contrast as Needed) Not rated Not rated Not rated 2 (R) Strain/Strain Rate Imaging by Speckle or Tissue Doppler (5) M (6) M 7 (A) (1) R Medical Coverage Policy: 0510 Table 4: Nonvalvular Heart Disease, Sequential or Follow-Up Testing: Asymptomatic or Stable Symptoms Re-evaluation (<1 y) in a patient at risk for heart failure (HF) without structural heart disease on prior TTE and no change in clinical status or cardiac examination (stage A) Re-evaluation of known hypertensive heart disease without a change in clinical status or cardiac examination (stage A) (<1 y) Re-evaluation (<1 y) of HF (systolic or diastolic) cardiomyopathy or HF without a change in clinical status or cardiac examination Re-evaluation (<1 y) in a patient previously or currently undergoing therapy with potentially cardiotoxic agents Re-evaluation (<1 y) of known aortic dilatation at baseline study to assess changes in rate of expansion or size in patient without bicuspid aortic valve Re-evaluation (<1 y) of the size and morphology of the aortic sinuses and ascending aorta in patients with a bicuspid aortic valve and an aortic diameter >4 cm without characteristics mentioned in the indication below Re-evaluation (<1 y) of the size and morphology of the aortic sinuses and ascending aorta in patients with a bicuspid aortic valve and an aortic diameter >4 cm with one of the following: Aortic dilatation >4.5 cm; Rapid rate of change in aortic diameter; Family history of aortic dissection Re-evaluation (<1 y) of known moderate or greater pulmonary hypertension without change in clinical status or cardiac examination Re-evaluation (≥1 y) of known moderate or greater pulmonary hypertension without change in clinical status or cardiac examination Re-evaluation of chronic asymptomatic pericardial effusion when findings would potentially alter therapy Further clarification of suspected pericardial constriction when findings of TTE including tissue Doppler is unclear Re-evaluation of intracardiac mass when findings would potentially alter therapy Re-evaluation of prior TEE findings for interval change (e.g., resolution of atrial thrombus after anticoagulation) when no change in therapy is anticipated. TTE (With or Without 3D; With Contrast as Needed) 2 (R) 2 (R) 2 (R) 7 (A) 3 (R) 2 (R) 3 (R) 4 (M) 7 (A) 7 (A) 1 (R) 8 (A) Strain/Strain Rate Imaging by Speckle or Tissue Doppler 1 (R) 1 (R) 1 (R) 7 (A) 1 (R) 1 (R) 1 (R) 1 (R) 1 (R) 1 (R) 1 (R) 1 (R) 1 (R) 1 (R) Medical Coverage Policy: 0510 Table 5: Nonvalvular Heart Disease, Sequential or Follow-Up Testing: New or Worsening Symptoms or to Guide Therapy Re-evaluation of known structural heart disease with change in clinical status or cardiac examination or to guide therapy (assume ischemic work-up has been performed and remains valid) Re-evaluation of prior TEE findings for interval change (e.g., reduction or resolution of atrial thrombus after anticoagulation or intracardiac evaluation of cardiac mass when a change in therapy is anticipated Re-evaluation of known cardiomyopathy with a change in clinical status or cardiac examination or to guide therapy (assume ischemic work-up has been done, performed, and remains valid) Re-evaluation of known HF (systolic or diastolic) with a change in clinical status or cardiac examination without a clear precipitating change in medication or diet Re-evaluation of known HF (systolic or diastolic) with a change in clinical status or cardiac examination with a clear precipitating change in medication or diet Periodic re-evaluation in a patient undergoing therapy with cardiotoxic agents and worsening symptoms Re-evaluation after revascularization and/or optimal medical therapy to determine candidacy for device therapy and/ or to determine optimal choice of device Re-evaluation for CRT device optimization in a patient with worsening HF (*Gated-SPECT for this indication only) Re-evaluation for ventricular assist device-related complication or infection is suspected (*FDG PET in this indication is for infection detection) Re-evaluation for progression of pericardial effusion size or development of tamponade Re-evaluation for progression of pericardial constriction Evaluation of patient with pericardial mass and symptoms suggestive of expansion Re-evaluation of known ascending aortic dilatation or history of aortic dissection with a change in clinical status (excluding acute coronary syndrome) or cardiac examination or when findings may alter management or therapy TTE (With or Without 3D; With Contrast as Needed) 8 (A) 5 (M) 8 (A) 8 (A) 4 (M) 9 (A) 8 (A) 8 (A) 8 (A) 9 (A) 8 (A) 8 (A) 8 (A) Strain/Strain Rate Imaging by Speckle or Tissue Doppler (4) M (1) R (5) M (4) M (1) R 7 (A) 1 (R) (4) M 1 (R) 1 (R) 1 (R) 1 (R) 1 (R) Medical Coverage Policy: 0510 Re-evaluation of known pulmonary hypertension with change in clinical status or cardiac examination or to guide therapy TTE (With or Without 3D; With Contrast as Needed) 8 (A) Strain/Strain Rate Imaging by Speckle or Tissue Doppler 1 (R) Table 6: Nonvalvular Heart Disease, Patients Undergoing Transcatheter Intervention, Imaging for the Evaluation of transient ischemic attack (TIA) or Ischemic Stroke Initial evaluation of patient to exclude cardiac origin of TIA or ischemic stroke: (Intracardiac masses (thrombus, vegetation);Valvular pathology Provocative maneuvers (Valsalva, cough) to assess for the presence of: Right-to-left intracardiac shunt TTE (with agitated saline injection; with or without 3D; with contrast as needed) 8 (A) 8 (A) Table 7A: Nonvalvular Heart Disease, Imaging for the Evaluation of Patent Foramen Ovale or Atrial Septal Defect, Preprocedural Evaluation for Closure of PFO or Atrial Septal Defect Preprocedure assessment for PFO: Atrial appendage thrombus; Spontaneous echo contrast (slow blood flow); Aortic atheroma; Cardiac masses; Vegetations Preprocedure assessment for: Atrial septum anatomy; Atrial septum aneurysm; Suitability for percutaneous device closure TTE (with agitated saline injection; with or without 3D; with contrast as needed) 7 (A) 7 (A) Table 7B: Nonvalvular Heart Disease, Imaging for the Evaluation of Patent Foramen Ovale or Atrial Septal Defect, Intra-Procedural Guidance for Closure of PFO or ASD Intraprocedural guidance in patient with either: ASD of simple anatomy; No aneurysmal atrial septum; PFO with short tunnel Intraprocedural guidance in patient with either: ASD with complex anatomy; Aneurysmal interatrial septum; PFO with long tunnel TTE (with agitated saline injection; with or without 3D; with contrast as needed) 3 (R) 3 (R) Table 7C: Nonvalvular Heart Disease, Imaging for the Evaluation of Patent Foramen Ovale or Atrial Septal Defect, Assessment Following Closure of PFO or ASD Medical Coverage Policy: 0510 6-month routine scheduled follow-up ASD/PFO device closure for position of device and integrity of device; PFO patency; Thrombus formation Nonroutine follow up of ASD/PFO device closure and clinical concern for infection, malposition, embolization or persistent shunt. TTE (with agitated saline injection; with or without 3D; with contrast as needed) 7 (A) 8 (A) Table 8A: Nonvalvular Heart Disease, Imaging for the Evaluation of Left Atrial Appendage (LAA) Occlusion Device, Pre-Procedural Evaluation for LAA Occlusion Evaluate for: All cardiac chambers; LV function; Interatrial septum; Valve function Evaluate for: LA/LAA thrombus; Spontaneous echo contrast/slow blood flow Assess: LAA morphology; Baseline LAA dimensions; Ostial morphology and dimension; Maximum length of dominant lobe TTE (with or without 3D; with contrast as needed) 8 (A) 5 (M) 6 (M) Table 8B: Nonvalvular Heart Disease, Imaging for the Evaluation of Left Atrial Appendage (LAA) Occlusion Device, Intraprocedural Guidance for LAA Occlusion Screen for procedural complications TTE (with or without 3D; with contrast as needed) 7 (A) Table 8C: Nonvalvular Heart Disease, Imaging for the Evaluation of Left Atrial Appendage (LAA) Occlusion Device, Assessment Following LAA Occlusion TTE (with or without 3D) Prior to discharge to assess: Device position; Presence of pericardial effusion; Presence of thrombus around the device; Mitral valve function; LV function Surveillance at 45 days or FDA guidance/guidelines for follow- up: Assess device stability; Exclude migration, displacement, or erosion; Assess device leak Long-term follow-up (assume device integrity) 6 (M) 4 (M) 5 (M) AHA/ACC/HFSA Guideline for the Management of Heart Failure (Heidenreich, et al., 2022): Medical Coverage Policy: 0510 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure Class of Recommendation (COR) and Level of Evidence (LOE)* 4.4. Evaluation With Cardiac Imaging Recommendations for Evaluation With Cardiac Imaging COR: 1; LOE: C-LD Recommendation: In patients with suspected or newly diagnosed heart failure, transthoracic echocardiography (TTE) should be performed during initial evaluation to assess cardiac structure and function. See Appendix for ACC/AHA Class of Recommendation and Level of Evidence definitions ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease (2022): Class of Recommendation (COR) and Level of Evidence (LOE) 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease 6.1.2.1. Heritable thoracic aortic disease (HTAD): Genetic Testing and Screening of Family Members for thoracic aortic disease (TAD) Recommendations for HTAD: Genetic Testing and Screening of Family Members for TAD: COR:1; LOE: B-NR In patients with TAD who have a pathogenic/likely pathogenic variant, genetic testing of at-risk biological relatives (ie, cascade testing) is recommended. In family members who are found by genetic screening to have inherited the pathogenic/likely pathogenic variant, aortic imaging with TTE (if aortic root and ascending aorta are adequately visualized, otherwise with CT or MRI) is recommended. 6.1.2.2.1. Diagnostic and Surveillance Aortic Imaging in Marfan Syndrome Initial Diagnosis and Surveillance Imaging Recommendations for Diagnostic and Surveillance Aortic Imaging in Marfan Syndrome: In patients with Marfan syndrome, a TTE is recommended at the time of initial diagnosis, to determine the diameters of the aortic root and ascending aorta, and 6 months thereafter, to determine the rate of aortic growth; if the aortic diameters are stable, an annual surveillance TTE is recommended. If the aortic root, ascending aorta, or both are not COR:1; LOE: C-EO Medical Coverage Policy: 0510 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease adequately visualized on TTE, a CT or MRI of the thoracic aorta is recommended. 6.1.2.3.1. Imaging in Loeys-Dietz Syndrome Recommendations for Imaging in Loeys-Dietz Syndrome: In patients with Loeys-Dietz syndrome, a baseline TTE is recommended to determine the diameters of the aortic root and ascending aorta, and 6 months thereafter to determine the rate of aortic growth; if the aortic diameters are stable, annual surveillance TTE is recommended. 6.1.2.5. Turner Syndrome Recommendations for Diagnostic Testing, Surveillance, and Surgical Intervention for Aortic Dilation in Turner Syndrome: In patients with Turner syndrome, TTE and cardiac MRI are recommended at the time of diagnosis to evaluate for bicuspid aortic valve (BAV), aortic root and ascending aortic dilation, aortic coarctation, and other congenital heart defects. In patients with Turner syndrome without risk factors for aortic dissection, surveillance imaging with TTE or MRI to evaluate the aorta is recommended every 5 years in children and every 10 years in adults, as well as before planning a pregnancy. 6.1.3. Bicuspid aortic valve (BAV) Aortopathy Recommendations for BAV Aortopathy: In patients with a BAV, TTE is indicated to evaluate valve morphology and function, to evaluate the diameter of the aortic root and ascending aorta, and to evaluate for aortic coarctation and other associated cardiovascular defects. In patients with a BAV and a dilated aortic root or ascending aorta, screening of all first-degree relatives by TTE is recommended to evaluate for the presence of a BAV, dilation of the aortic root and ascending aorta, or both; if the diameter and morphology of the aortic root, ascending aorta, or both cannot be assessed accurately or completely by TTE, a cardiac gated CT or MRI of the thoracic aorta is indicated. Medical Coverage Policy: 0510 Class of Recommendation (COR) and Level of Evidence (LOE) COR:1; LOE: C-EO COR:1; LOE: B-NR COR:1; LOE: C-LD COR:1; LOE: B-NR COR:1; LOE: C-LD COR:2a; LOE: B-NR 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease In patients with a BAV, screening of all first-degree relatives by TTE is reasonable to evaluate for the presence of a BAV, dilation of the aortic root and ascending aorta, or both. 6.1.3.1. Routine Follow-Up of BAV Disease Aortopathy Recommendations for Routine Follow-Up of BAV Disease Aortopathy: In patients with a BAV who have undergone previous aortic valve repair or replacement and have a diameter of the aortic root, ascending aortic, or both of ≥4.0 cm, lifelong surveillance imaging of the aortic root and ascending aorta by TTE, CT, or MRI is recommended at an interval dependent on aortic diameter and rate of growth. In patients with a BAV and a diameter of the aortic root, ascending aorta, or both of ≥4.0 cm, lifelong surveillance imaging of the aortic root and ascending aorta by TTE, CT, or MRI is recommended at an interval dependent on aortic diameter and rate of growth. 6.4.3.1. Surveillance of Thoracic Aortic Dilation and Aneurysm Recommendations for Surveillance of Thoracic Aortic Dilation and Aneurysm: In patients with a dilated thoracic aorta, a TTE is recommended at the time of diagnosis to assess aortic valve anatomy, aortic valve function, and thoracic aortic diameters. In patients with a dilated thoracic aorta, follow-up imaging (with TTE, CT, or MRI, as appropriate based on individual anatomy) in 6 to 12 months is reasonable to determine the rate of aortic enlargement; if stable, surveillance imaging every 6 to 24 months (depending on aortic diameter) is reasonable. 8.1. Counseling and Management of Aortic Disease in Pregnancy and Postpartum Recommendations for Counseling and Management of Aortic Disease in Pregnancy and Postpartum: Pre-pregnancy: In patients with syndromic and nonsyndromic heritable thoracic aortic disease (nsHTAD), Turner syndrome, BAV with aortic dilation, Medical Coverage Policy: 0510 Class of Recommendation (COR) and Level of Evidence (LOE) COR:1; LOE: B-NR COR:1; LOE: C-LD COR:1; LOE: C-LD COR:2a; LOE: C-LD COR:1; LOE: C-LD 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease Class of Recommendation (COR) and Level of Evidence (LOE) and other aortopathy conditions, aortic imaging (with TTE, MRI or CT, or both as appropriate) before pregnancy is recommended to determine aortic diameters. COR:1; LOE: C-LD During Pregnancy: In pregnant patients with an aortopathic condition or a dilated aortic root or ascending aorta, surveillance TTE to monitor aortic diameters and aortic valve function is recommended each trimester and again several weeks postpartum, although imaging may be more frequent depending on aortic diameter, aortic growth rate, and underlying condition. 2022 American Society of Echocardiography (ASE) Recommendations for Multimodality Cardiovascular Imaging of Patients with Hypertrophic Cardiomyopathy (Nagueh, et al., 2022): ASE Recommendations for Multimodality Cardiovascular Imaging of Patients with Hypertrophic Cardiomyopathy Assessment of Left Ventricular Systolic Function: Assessment of global longitudinal strain adds important prognostic data and may be performed in centers with experience and expertise with using strain echocardiography. 2022 American Society of Echocardiography (ASE): Non-Invasive Imaging in Coronary Syndromes (Edvardsen, et al., 2022): ASE Non-Invasive Imaging in Coronary Syndromes Left Ventricular Function Assessment TTE should be used as the initial imaging modality for the assessment of LV systolic function. If initial echocardiographic images are of limited quality (two or more contiguous segments cannot be properly seen), an ultrasound enhancing agents (UEA) should be used for better endocardial delineation. 2017 American College of Cardiology (ACC) Appropriate Use Criteria (AUC) for Multimodality Imaging in Valvular Heart Disease (VHD) The American College of Cardiology Appropriate Use Criteria Task Force, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons published the 2017 Appropriate Use Criteria (AUC) for Multimodality Imaging in Valvular Heart Disease (VHD) (Doherty, et al., 2017). Noteworthy: Medical Coverage Policy: 0510 • This document is the first of two companion appropriate use criteria (AUC) documents. • The criteria are divided into three primary sections: 1) initial evaluation for VHD; 2) prior testing; and 3) transcatheter intervention for VHD. Includes 92 separate TTE indications Where appropriate, the scenarios were developed on the basis of the most current American College of Cardiology/American Heart Association guidelines. Ratings: A = Appropriate. Median Score 7 to 9: Appropriate test for specific indication (test is generally acceptable and is a reasonable approach for the indication). M = May be appropriate. Median Score 4 to 6: May Be Appropriate test for specific indication (test may be generally acceptable and may be a reasonable approach for the indication). May Be Appropriate also implies that more research and/or patient information is needed to classify the indication definitively. R = Rarely appropriate. Median Score 1 to 3: Rarely Appropriate test for specific indication (test is not generally acceptable and is not a reasonable approach for the indication). Table 1: Initial Evaluation for Valvular Heart Disease (VHD) of an Asymptomatic Patient Unexplained murmur or abnormal heart sounds Reasonable suspicion of valvular heart disease (VHD) History of rheumatic heart disease Known systemic or acquired disease associated with VHD First-degree family history of a bicuspid aortic valve Exposure to medications that could result in development of VHD TTE A (9) A (9) A (9) A (9) A (8) A (7) 3D TTE R (3) M (4) M (4) R (3) R (1) R (1) Table 2: Initial Evaluation for VHD of a Patient with Clinical Signs and/or Symptoms TTE 3D TTE Arrhythmias Palpitations AND No other symptoms or signs of cardiovascular disease M (4) R (1) Presyncope/Syncope Presyncope AND No other symptoms or signs of cardiovascular disease Syncope AND No other symptoms or signs of cardiovascular disease M (6) A (8) R (1) R (1) Hypotension or Hemodynamic Instability Hypotension or hemodynamic instability AND Uncertain or suspected cardiac etiology Assessment of volume status in a critically ill patient Suspected acute mitral or aortic regurgitation A (9) M (6) A (9) R (3) R (2) M (6) Respiratory Failure Respiratory failure or hypoxemia of uncertain etiology Respiratory failure or hypoxemia AND Noncardiac etiology of respiratory failure has been established A (8) M (4) R (2) R (1) Heart Failure (HF) Initial evaluation in patients presented with HF to exclude the presence of primary or secondary valve disease A (9) R (3) Medical Coverage Policy: 0510 Bacteremia/Endocarditis Suspected infective endocarditis (IE) (native valve, prosthetic valve, endocardial lead) AND Positive blood cultures or a new murmur Transient fever AND No evidence of bacteremia or a new murmur Transient bacteremia AND Pathogen not typically associated with IE and/or a documented nonendovascular source or infection A (9) R (2) R (3) Cardiac Mass/Cardiac Source of Emboli Suspected cardiac mass, suspected tumor or thrombus, or potential cardiac source of emboli A (9) Table 3: Valvular Heart Disease, Prior testing, Additional Testing to Clarify Diagnosis TTE with contrast Inadequate TTE Images Inadequate TTE images for the evaluation of possible valvular heart disease due to patient characteristics Characterization of native or prosthetic valves with clinical signs or symptoms suggesting valve dysfunction M (5) M (4) Suspected Endocarditis With Negative TTE Suspected infective endocarditis (IE) with moderate to high pretest probability (i.e., staph bacteremia, fungemia, prosthetic heart valve, or intracardiac device) R (2) Aortic Stenosis (AS) Symptomatic, severe aortic stenosis (AS) by calculated valve area (stage D2) AND Low flow/low gradient AND Low left ventricular ejection fraction (LVEF) Severe AS, by calculated valve area AND Low flow/low gradient AND Preserved LVEF and for assessment of morphology, including calcification Moderate or asymptomatic severe AS (stages B and C), for measurement of changes in valve hemodynamics with exercise or pharmacological stress Symptomatic severe AS (stage D), for measurement of changes in valve hemodynamics with exercise or pharmacological stress R (2) R (2) R (1) R (1) Mitral Stenosis Discrepancy between resting Doppler echocardiographic findings and clinical symptoms or signs to evaluate mean mitral gradient and pulmonary artery pressure R (3) Mitral Regurgitation (MR) Severe mitral regurgitation (MR) suspected clinically AND Potentially underestimated on TTE despite optimal images OR Better imaging of MR jet needed R (2) Medical Coverage Policy: 0510 M (4) R (1) R (1) M (5) 3D TTE R (2) R (2) R (2) R (1) R (3) R (1) R (1) M (4) M (5) Chronic symptomatic primary MR with discrepancy between exertional symptoms and the severity of MR at rest OR Symptoms are disproportionate to the severity of MR determined at rest Chronic asymptomatic patient, to distinguish between moderate or severe primary MR Chronic secondary MR (stages B to D), to establish etiology, including a possible ischemic etiology Chronic secondary MR (stages B to D), to assess myocardial viability R (1) R (1) M (4) R (1) Aortic Regurgitation (AR) Dilated aortic sinuses or ascending aorta or a bicuspid aortic valve (stages A and B), to evaluate the presence and severity of AR assuming optimal TTE images Discordance between clinical assessment and TTE about the severity of AR Assessment of symptoms and functional capacity in patients with moderate or severe AR R (1) R (1) R (1) Other Valvular Regurgitation Severe tricuspid regurgitation (stages C and D) and suboptimal TTE images, for assessment of RV systolic function and systolic and diastolic volumes Assessment of pulmonary pressures during stress in patient with severe asymptomatic valve regurgitation prior to pregnancy R (3) R (1) Valvular Mass Further evaluation of valvular mass (including incidental findings noted on noncardiac imaging studies) A (9) for TTE without contrast; M (4) for TTE with contrast Table 4: Valvular Heart Disease, Prior testing, Sequential or Follow-Up Testing: Asymptomatic or Stable Symptoms TTE Stage A valvular heart disease (VHD) Routine surveillance (every 3–5 y) for patients with stage A (bicuspid aortic valve (AV) or aortic sclerosis) for exclusion of progression to stage B. A (9) Mild or Moderate VHD Re-evaluation (3–5 y) of mild (stage B) valvular regurgitation Re-evaluation (1–2 y) of mild (stage B) VHD without a change in clinical status or cardiac examination Re-evaluation (1–2 y) of moderate (stage B) VHD without a change in clinical status of cardiac examination A (8) M (4) A (7) Medical Coverage Policy: 0510 M (4) M (4) M (5) R (1) R (3) R (3) R (1) R (3) R (1) M (5) 3D TTE R (1) R (1) R (1) R (1) Re-evaluation (<1 y) in patients with moderate AS who will be subjected to increased hemodynamic demands (e.g., noncardiac surgery, pregnancy) Severe VHD Re-evaluation (6–12 m) of asymptomatic severe (stage C1) aortic stenosis (AS) without a change in clinical status or cardiac examination Re-evaluation (every 1 y) for asymptomatic (stage C1) patients with AS Re-evaluation (6–12 m) of stage C1 patients with asymptomatic severe aortic regurgitation (AR) with preserved ejection fraction and normal LV size Re-evaluation (every 6–12 m) of stage C1 patients with asymptomatic severe mitral regurgitation (MR) Re-evaluation (<1 y) in patients with severe AS who will be subjected to increased hemodynamic demands (e.g., noncardiac surgery, pregnancy) Re-evaluation after control of hypertension in patients with low-flow/low-gradient severe AS with preserved left ventricular ejection fraction (LVEF) Bicuspid aortic valve (AV) With Dilated Aorta Re-evaluation (<1 y) of the size and morphology of the aortic sinuses and ascending aorta in patients with a bicuspid aortic valve (AV) and an ascending aortic diameter >4 cm with 1 of the following: • aortic diameter >4.5 cm • rapid rate of change in aortic diameter • family history (first-degree relative) of aortic dissection Re-evaluation (<1 y) of the size and morphology of the aortic sinuses and ascending aorta in patients with a bicuspid AV and an aortic diameter of 4.0–4.5 cm without any of the risk factors listed in the indication above. Endocarditis Re-evaluation of prior TTE/TEE finding for interval change (e.g., resolution of vegetation after antibiotic therapy) when no change in therapy is anticipated Re-evaluation of prior TTE/TEE finding for interval change (e.g., resolution of vegetation after antibiotic therapy) when a change in therapy is anticipated Re-evaluation of patient with infective endocarditis (IE) at high risk of progression or complications (e.g., extensive infective tissue/ large vegetation on initial echocardiogram, or staphylococcal, enterococcal, or fungal infections) in the absence of clinical change Medical Coverage Policy: 0510 M (6) M (6) A (8) M (6) A (7) M (6) A (7) A (7) R (2) M (4) A (8) A (7) R (1) R (1) R (1) R (1) R (1) R (1) R (1) R (3) R (1) R (1) R (3) R (1) Table 5: Valvular Heart Disease, Prior testing, Sequential or Follow-Up Testing of New or Worsening Symptoms or to Guide Therapy TTE 3D TTE General Re-evaluation of known VHD with a change in clinical status or cardiac examination or to guide therapy A (9) R (1) Endocarditis Re-evaluation of infective endocarditis (IE) in a patient with a change in clinical status or cardiac examination (e.g., new murmur, embolism, persistent fever, heart failure (HF), abscess, or atrioventricular heart block) A (8) R (3) Table 6: Valvular Heart Disease, Prior testing, Postoperative Imaging After Surgical Valve Replacement or Repair TTE 3D TTE Surgical Valve Replacement (No or Stable Symptoms) Initial postoperative evaluation of bioprosthetic or mechanical valve for establishment of baseline (6 wk to 3 mo postoperative) Re-evaluation (<3 y after valve implantation) of bioprosthetic or mechanical valve if no known or suspected valve dysfunction Re-evaluation (≥3 y after valve implantation) of bioprosthetic or mechanical valve if no known or suspected valve dysfunction Re-evaluation in patients with a bioprosthetic valve after the first 10 years, even in the absence of a change in clinical status Evaluation prior to pregnancy in patients with a prosthetic valve and no echocardiography within the past year A (9) M (5) A (7) A (9) A (9) R (1) R (1) R (1) R (1) R (1) Surgical Valve Replacement (Suspicion of Valve Dysfunction) Characterization of mechanical prosthetic valve if clinical signs or symptoms suggesting valve dysfunction Characterization of bioprosthetic valve if clinical signs or symptoms suggesting valve dysfunction Characterization of bioprosthetic valve if suspected clinically significant valvular dysfunction and inadequate images from TTE or TEE Characterization of mechanical prosthetic valve if suspected clinically significant valvular dysfunction and inadequate images from TTE or TEE Re-evaluation of known prosthetic valve dysfunction when it would change management or guide therapy Evaluation of documented prosthetic valve IE when medical management is considered, in a patient who is at high risk for progression or complication or with a change in clinical status or cardiac examination A (9) A (9) Not rated Not rated A (9) A (9) M (6) M (6) R (2) R (1) M (5) M (5) Mitral Valve Repair Initial postoperative assessment of valve repair (6 wk to 3 mo postoperatively) A (9) M (4) Medical Coverage Policy: 0510 Re-evaluation (<3 y) in patients without suspected repaired valve dysfunction Re-evaluation (≥3 y) in patients without suspected repaired valve dysfunction Re-evaluation (<3 y) for suspected repaired valve dysfunction R (1) A (8) A (9) R (1) M (4) M (6) Table 7A: Transcatheter Intervention for VHD, Pre-Transcatheter aortic valve replacement (TAVR) Evaluation Assessment for concomitant coronary artery disease Accurate assessment of annular size and shape* *Multimodality imaging might improve the accuracy of the measurements Assessment of number of cusps and degree of calcification Measurement of the distance between annulus and the coronary ostia Precise coaxial alignment of the implant within the centerline of the aortic valve Assessment of aortic dimensions Assessment of aortic atherosclerotic burden Assessment of iliofemoral vessels TTE R (1) R (3) A (7) R (1) R (1) R (1) R (1) R (1) 3D TTE R (1) M (4) M (6) R (1) R (1) R (1) R (1) R (1) Table 7B: Transcatheter Intervention for VHD, Intraprocedural Evaluation During TAVR Guidewire placement into the LV Valve placement Postdeployment assessment (position, function, regurgitation) Evaluate immediate complications • Hypotension • Coronary occlusion • LV depression from rapid pacing • LV outflow tract obstruction • Severe MR • Prosthesis dislodgment • Tamponade • Right ventricular perforation • Air embolism • Aortic dissection (paravalvular leak needs to be excluded) TTE A (7) A (7) A (7) A (8) 3D TTE M (5) M (6) A (7) A (7) Table 7C: Transcatheter Intervention for VHD, Postprocedural Assessment After TAVR (Out of Procedure and <30 days) Assessment of degree of aortic regurgitation (including valvular and paravalvular) with suspicion of valve dysfunction Assessment of stroke with suspicion of valve dysfunction TTE A (8) A (7) 3D TTE M (5) R (3) Medical Coverage Policy: 0510 Table 8A: Transcatheter Intervention for VHD, Evaluation Prior to Percutaneous Mitral Valve Repair Determine patient eligibility* * * Currently, MitraClip is the only FDA-approved device available. Exclude the presence of intracardiac mass, thrombus, or vegetation prior to (within 3 d of the procedure) TTE A (8) M (4) 3D TTE A (7) M (5) Table 8B: Transcatheter Intervention for VHD, Intraprocedural Evaluation During Percutaneous Mitral Valve Repair Alignment of the device over the origin of the regurgitant jet and advance to the LV Guidance for grasping the mitral valve leaflets and device closure Assess for adequacy in the reduction of the MR Assess for presence of mitral stenosis TTE R (1) R (1) M (4) M (5) 3D TTE A (9) A (9) A (9) A (9) Table 8C: Transcatheter Intervention for VHD, Postprocedural Assessment After Percutaneous Mitral Valve Repair (Out of Procedure) Reassessment for degree of MR and left ventricular function (predischarge at 1, 6, and 12 mo, and then annually to 5 y) TTE A (9) 3D TTE M (5) 2020 American College of Cardiology (ACC) Appropriate Use Criteria (AUC) for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease (CHD) The American College of Cardiology (ACC) Solution Set Oversight Committee and Appropriate Use Criteria (AUC) Task Force, American Heart Association (AHA), American Society of Echocardiography (ASE), Heart Rhythm Society (HRS), International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography published the 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease (Sachdeva, et al., 2020). Noteworthy: • Addresses cardiac imaging in adult and pediatric patients with established Congenital Heart Disease. Includes 324 separate TTE indications Addresses only the follow-up of patients with established CHD using various cardiovascular imaging modalities. It is assumed that a complete anatomic cardiac diagnosis has been established. The initial evaluation by TTE prompted by signs and symptoms suggesting CHD has been addressed in the 2014 AUC for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology. Ratings: A = Appropriate. Median Score 7 to 9: Appropriate test for specific indication (test is generally acceptable and is a reasonable approach for the indication). M = May be appropriate. Median Score 4 to 6: May Be Appropriate test for specific indication (test may be generally acceptable and may be a reasonable approach for the Medical Coverage Policy: 0510 indication). May Be Appropriate also implies that more research and/or patient information is needed to classify the indication definitively. R = Rarely appropriate. Median Score 1 to 3: Rarely Appropriate test for specific indication (test is not generally acceptable and is not a reasonable approach for the indication). Table 1: Congenital Heart Disease (CHD), Patent Foramen Ovale, Atrial Septal Defects (ASD) and Partial Anomalous Pulmonary Venous Connection (PAPVC) Patent Foramen Ovale (PFO) Routine surveillance of an asymptomatic patient with a PFO TTE R (1) TTE with contrast R (1) Unrepaired Routine surveillance (1–2 years) in an asymptomatic patient with a small atrial septal defects (ASD) or Partial anomalous pulmonary venous connection (PAPVC) involving a single pulmonary vein Routine surveillance (3–5 years) in an asymptomatic patient with a small ASD or PAPVC involving a single pulmonary vein Routine surveillance (1–2 years) in an asymptomatic patient with ≥ moderate ASD or PAPVC involving >1 pulmonary vein Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation to determine the method of closure of isolated secundum ASD Evaluation prior to planned repair of sinus venosus defect and/or PAPVC M (4) A (7) A (8) A (9) A (9) A (9) Not rated Not rated Not rated M (5) M (4) M (4) Postprocedural: Surgical or catheter-based Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance within 1 week following device closure of ASD in an asymptomatic patient with no or mild sequelae Routine surveillance at 1 month following device closure of ASD in an asymptomatic patient with no or mild sequelae Routine surveillance at 3–6 months following device closure of ASD in an asymptomatic patient with no or mild sequelae Routine surveillance at 1 year following device closure of ASD in an asymptomatic patient with no or mild sequelae Routine surveillance (2–5 years) after the first year following device closure of ASD in an asymptomatic patient with no or mild sequelae Routine surveillance within a year following surgical ASD closure or PAPVC repair in an asymptomatic patient with no or mild sequelae Routine surveillance (annually) after the first year following surgical ASD closure or PAPVC repair in an asymptomatic patient with no or mild sequelae A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) M (6) M (5) M (6) R (3) R (3) R (3) R (3) R (2) R (2) R (2) Medical Coverage Policy: 0510 Routine surveillance (2–5 years) after the first year following surgical ASD closure or PAPVC repair in an asymptomatic patient with no or mild sequelae Routine surveillance (3–12 months) following surgical or device closure of ASD in a patient with significant residual shunt, valvular or ventricular dysfunction, arrhythmias, and/or pulmonary hypertension Routine surveillance (3–12 months) following repair of PAPVC in a patient with systemic or pulmonary venous obstruction, valvular or ventricular dysfunction, arrhythmias, and/or pulmonary hypertension Table 2: Congenital Heart Disease (CHD), Ventricular Septal Defects (VSD) Unrepaired Routine surveillance (1–2 years) in an asymptomatic child with a small muscular VSD Routine surveillance (3–5 years) in an asymptomatic child with a small muscular VSD Routine surveillance (3–5 years) in an asymptomatic adult with a small muscular VSD Routine surveillance (1–2 years) in an asymptomatic child with a small VSD in a location other than muscular septum Routine surveillance (3–5 years) in an asymptomatic adult with a small VSD in a location other than muscular septum Routine surveillance (1–3 months) in an infant with ≥ moderate VSD on medical management Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postprocedural: Surgical or Catheter-Based Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance within a year following surgical or device VSD closure in an asymptomatic patient with no or mild sequelae Routine surveillance (2–3 years) after the first year following device closure of VSD in an asymptomatic patient with no or mild sequelae Routine surveillance (annually) after the first year following surgical VSD closure in an asymptomatic patient with no or mild sequelae Routine surveillance (2–3 years) after the first year following surgical VSD closure in an asymptomatic patient with no or mild sequelae Routine surveillance (2–3 years) following surgical or device closure in a patient with small residual shunt, ≤ mild valvular dysfunction, no ventricular dysfunction, arrhythmias, or pulmonary hypertension Routine surveillance (3–12 months) following surgical or device closure in a patient with significant residual shunt, valvular or ventricular dysfunction, arrhythmias, and/or pulmonary hypertension Medical Coverage Policy: 0510 A (9) A (9) A (9) R (2) M (4) M (5) TTE R (3) A (7) A (7) A (7) A (8) A (9) A (9) A (9) A (9) A (9) A (8) A (9) M (5) A (8) A (9) A (9) Table 3: Congenital Heart Disease (CHD), Atrioventricular Septal Defects Unrepaired: Partial/Transitional Routine surveillance (3–6 months) in an asymptomatic infant Routine surveillance (1–2 years) in an asymptomatic child Unrepaired: Complete Routine surveillance (1–3 months) in an infant Unrepaired: All Types Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postoperative Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance within a year after atrioventricular septal defects (AVSD) repair in an asymptomatic patient with no or mild sequelae Routine surveillance (1–3 years) after the first year following repair in an asymptomatic patient with no or mild sequelae Routine surveillance (3–12 months) in a patient with significant residual shunt, valvular or ventricular dysfunction, left ventricular outflow tract (LVOT) obstruction, arrhythmias, and/or pulmonary hypertension Routine surveillance (3–12 months) in a patient with heart failure symptoms Table 4: Congenital Heart Disease (CHD), Patent Ductus Arteriosus (PDA) Unrepaired Routine surveillance (3–5 years) in an asymptomatic patient with a trivial, silent PDA Routine surveillance (3–6 months) in an infant with ≥ moderate PDA Routine surveillance (3–6 months) in an infant with a small, audible PDA until closure Routine surveillance (1–2 years) in an infant or child with a small, audible PDA until closure Routine surveillance (3–5 years) in an adult with a small PDA Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postprocedural: Surgical or Catheter-Based Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (annually) within 2 years following PDA closure in an asymptomatic patient with no or mild sequelae Routine surveillance (5 years) after the first 2 years following surgical closure in an asymptomatic patient with no or mild sequelae Medical Coverage Policy: 0510 TTE A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) TTE R (3) A (9) A (7) A (8) A (9) A (9) A (9) A (9) A (9) A (8) R (3) Routine surveillance (5 years) after the first 2 years following device closure in an asymptomatic patient with no or mild sequelae Routine surveillance (1–2 years) in a patient with postprocedural left pulmonary artery stenosis Routine surveillance (1–2 years) in a patient with postprocedural aortic obstruction Table 5: Congenital Heart Disease (CHD), Total Anomalous Pulmonary Venous Connection Unrepaired Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postoperative Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) in an asymptomatic infant with no or mild sequelae Routine surveillance (1–2 years) in an asymptomatic child with no or mild sequelae Table 6: Congenital Heart Disease (CHD), Eisenmenger Syndrome (ES) and Pulmonary Hypertension Associated With CHD Eisenmenger Syndrome (ES) Initial evaluation with suspicion of ES Evaluation due to change in clinical status and/or new concerning signs or symptoms in a patient with ES Evaluation due to change in pulmonary arterial hypertension-targeted therapy in a patient with ES Routine surveillance (3 months) in a stable child with ES Routine surveillance (6–12 months) in a stable child with ES Routine surveillance (3 months) in a stable adult with ES Routine surveillance (6–12 months) in a stable adult with ES Pulmonary Hypertension (PH) Associated With Congenital heart disease (CHD) Initial evaluation with suspicion of pulmonary hypertension following CHD surgery Evaluation due to change in clinical status and/or new concerning signs or symptoms in a patient with postoperative PH Evaluation due to change in pulmonary arterial hypertension-targeted therapy in a patient with postoperative PH Routine surveillance (3 months) in a stable child with postoperative PH Routine surveillance (6–12 months) in a stable child with post-operative PH Routine surveillance (3 months) in a stable adult with postoperative PH Routine surveillance (6–12 months) in a stable adult postoperative PH Medical Coverage Policy: 0510 A (7) A (9) A (9) TTE A (9) A (9) A (9) A (9) A (8) A (8) TTE A (9) A (9) A (9) M (6) A (9) R (3) A (9) A (9) A (9) A (9) A (7) M (5) A (9) A (9) Table 7: Congenital Heart Disease (CHD), Ebstein Anomaly and Tricuspid Valve Dysplasia Unrepaired TTE Routine surveillance (1–2 years) in an asymptomatic infant or child with mild tricuspid regurgitation (TR) Routine surveillance (3–5 years) in an asymptomatic adult with mild TR Routine surveillance (3–6 months) in an asymptomatic infant with ≥ moderate TR without hypoxemia Routine surveillance (6–12 months) in an asymptomatic patient with ≥ moderate TR and previously stable RV size and/or function without hypoxemia Evaluation due to change in clinical status and/or new concerning signs and symptoms Evaluation of an atrial septal defect (ASD) for device closure in a patient with mild or moderate TR, right ventricle (RV) enlargement, and no hypoxemia Evaluation prior to planned repair A (9) A (9) A (9) A (9) A (9) A (9) A (9) Postprocedural: Surgical or Cathether-Based Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (1–2 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (6–12 months) in an asymptomatic child with valvular or ventricular dysfunction or arrhythmias Routine surveillance (1–2 years) in an asymptomatic adult with valvular or ventricular dysfunction or arrhythmias Routine surveillance (3–12 months) in a patient with symptoms of heart failure and/or atrial arrhythmias A (9) A (9) A (9) A (9) A (9) A (9) Table 8: Congenital Heart Disease (CHD), Pulmonary Stenosis (PS) Unrepaired Routine surveillance (3–6 months) in an asymptomatic infant with mild PS Routine surveillance (1–2 years) in an asymptomatic child with mild PS Routine surveillance (3–5 years) in an asymptomatic adult with mild PS Routine surveillance (3–6 months) in an asymptomatic infant with ≥ moderate PS Routine surveillance (1–2 years) in an asymptomatic child or adult with ≥ moderate PS Routine surveillance (3–5 years) in an asymptomatic adult with PS and pulmonary artery dilation Medical Coverage Policy: 0510 TTE with contrast Not rated M (5) Not rated M (4) A (7) M (6) M (6) M (6) A (7) Not rated Not rated Not rated Not rated Not rated TTE A (8) A (8) A (9) A (9) A (9) Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postprocedural: Surgical or Catheter-Based Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (1–2 years) in an asymptomatic child with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic adult with no or mild sequelae Routine surveillance (6–12 months) in an asymptomatic child with moderate or severe sequelae Routine surveillance (1–3 years) in an asymptomatic adult with moderate or severe sequelae Routine surveillance (3–12 months) in a patient with heart failure symptoms Table 9: Congenital Heart Disease (CHD), Pulmonary Atresia With Intact Ventricular Septum Unrepaired Evaluation prior to planned repair Postprocedural: Palliation Routine postprocedural evaluation (within 30 days) Routine surveillance (1–3 months) in an asymptomatic patient Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postprocedural: Complete Repair Routine postprocedural evaluation (within 30 days) Evaluation due to a change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) in an asymptomatic infant Routine surveillance (1–2 years) in an asymptomatic child with no or mild sequelae Routine surveillance (2–3 years) in an asymptomatic adult with no or mild sequelae Routine surveillance (6–12 months) in an asymptomatic child with ≥ moderate sequelae Routine surveillance (1–3 years) in an asymptomatic adult with ≥ moderate sequelae Routine surveillance (3–12 months) in a patient with heart failure symptoms Table 10: Congenital Heart Disease (CHD), Mitral Valve Disease Unrepaired Congenital Mitral Stenosis (MS) Medical Coverage Policy: 0510 A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) TTE A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) TTE Routine surveillance (1–4 weeks) in an infant <3 months with any degree of MS Routine surveillance (3–6 months) in an infant ≥3 months with mild MS Routine surveillance (1–3 months) in an infant ≥3 months with ≥ moderate MS Routine surveillance (1–2 years) in an asymptomatic child with mild MS Routine surveillance (3–12 months) in an asymptomatic child with ≥ moderate MS Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair A (8) A (8) A (9) A (9) A (9) A (9) A (9) Unrepaired: Congenital Mitral Regurgitation (MR) including Mitral Valve Prolapse (MVP) Routine surveillance (6–12 months) in an asymptomatic infant with mild MR Routine surveillance (1–3 months) in an asymptomatic infant with ≥ moderate MR Routine surveillance (2–5 years) in a child with mild MR, normal LV size and systolic function Routine surveillance (6–12 months) in a child with ≥ moderate MR Routine surveillance (1–2 years) in an asymptomatic child with MVP and mild MR Routine surveillance (3–5 years) in an asymptomatic child with MVP and mild MR Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair A (9) A (9) A (9) A (9) M (5) A (9) A (9) A (9) Postprocedural: Surgical or Catheter-Based Routine postprocedural evaluation (within 30 days) Evaluation in an infant or child due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) in an infant with mild MS or MR, and no LV dysfunction Routine surveillance (1–3 months) in an infant with ≥ moderate MS or MR, dilated LV, and no LV dysfunction Routine surveillance (1–2 years) in a child with mild MS or MR, and no LV dysfunction Routine surveillance (3–12 months) in a child with ≥ moderate MS or MR, dilated LV, and no LV dysfunction Routine surveillance (annually) in a child with normal prosthetic mitral valve function and no LV dysfunction Routine surveillance (3–12 months) in a child with prosthetic mitral valve or ventricular dysfunction, and/or arrhythmias A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) Table 11: Congenital Heart Disease (CHD), Left ventricular outflow tract (LVOT) lesions Unrepaired: Subvalvular Aortic Stenosis (AS) TTE Medical Coverage Policy: 0510 Routine surveillance (1–3 months) in an infant with any degree of subvalvular aortic stenosis (AS) and ≤ mild aortic regurgitation (AR) Routine surveillance (1–2 years) in a child or adult with mild subvalvular AS and no AR Routine surveillance (6–12 months) in a child or adult with ≥ moderate subvalvular AS and/or ≤ mild AR Routine surveillance (3–5 years) in an asymptomatic adult with ≥ moderate subvalvular AS Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postoperative Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) in an infant with ≤ mild stenosis and/or AR Routine surveillance (1–3 months) in an infant with ≥ moderate stenosis and/or AR Routine surveillance (1–2 years) in a child or adult with ≤ mild stenosis and/or AR Routine surveillance (6–12 months) in a child or adult with ≥ moderate stenosis and/or AR Routine surveillance (3–12 months) in an adult with heart failure symptoms or ≥ moderate stenosis and/or AR Unrepaired: Aortic Valve Stenosis and/or Regurgitation* *This part of the table does not include indications for adults: Routine surveillance (1–4 weeks) in an infant (<3 months old) with any degree of AS and/or AR not requiring neonatal surgery Routine surveillance (3–6 months) in an infant (3–12 months old) with mild AS and/or mild AR Routine surveillance (1–3 months) in an infant (3-12 months old) with ≥ moderate AS and/or ≥ moderate AR Routine surveillance (6 months) in an asymptomatic child with mild AS and/or mild AR without aortic dilation Routine surveillance (1–2 years) in an asymptomatic child with mild AS and/or mild AR without aortic dilation Routine surveillance (6–12 months) in an asymptomatic child with ≥ moderate AS and/or ≥ moderate AR Routine surveillance (3–5 years) in a child with a bicuspid aortic valve with trivial or mild valvular dysfunction with no aortic sinus and/or ascending aortic dilation Routine surveillance (2–3 years) in a child with aortic sinus and/or ascending aortic dilation with stable z-scores Routine surveillance (6–12 months) in a child with aortic sinus and/or ascending aortic dilation with increasing z-scores Medical Coverage Policy: 0510 A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) R (3) A (9) A (9) A (9) A (9) A (9) Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postprocedural: Surgical or Catheter-Based* *This part of the table does not include indications for adults: Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) in an infant following neonatal intervention with ≤ mild AS and/or AR and no LV dysfunction Routine surveillance (1–3 months) in an infant following neonatal intervention with ≥ moderate AS and/or regurgitation, and/or LV dysfunction Routine surveillance (1–2 years) in a child with ≤ mild AS and/or AR following repair or normal prosthetic valve function Routine surveillance (6–12 months) in a child with ≥ moderate AS or AR Routine surveillance (3–12 months) in a child with heart failure symptoms and/or ventricular dysfunction Unrepaired: Supravalvular Aortic Stenosis (AS) Routine surveillance (3–6 months) in an infant with any degree of supravalvular AS Routine surveillance (1–2 years) in an asymptomatic child or adult with mild supravalvular AS Routine surveillance (6–12 months) in an asymptomatic child or adult with moderate supravalvular AS Routine surveillance (2–5 years) in an asymptomatic adult with moderate supravalvular AS Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postoperative Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (2–5 years) in a patient with no or mild supravalvular AS Routine surveillance (6–12 months) in a patient with ≥ moderate supravalvular AS Table 12: Congenital Heart Disease (CHD), Aortic Coarctation and Interrupted Aortic Arch Unrepaired Routine surveillance (3–6 months) in an infant with mild aortic coarctation in the absence of a Patent ductus arteriosus (PDA) Routine surveillance (1–2 years) in a child or adult with mild aortic coarctation Medical Coverage Policy: 0510 A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) TTE A (9) A (9) Routine surveillance (3–5 years) in a child or adult with mild aortic coarctation Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postprocedural: Surgical or Catheter-Based Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) within the first year following surgical or catheter-based intervention in an asymptomatic patient with no or mild sequelae Routine surveillance (6–12 months) within the first year following catheter-based intervention in an asymptomatic patient with no or mild sequelae Routine surveillance (6 months) after the first year following surgical or catheter-based intervention in an asymptomatic patient with no or mild sequelae Routine surveillance (1–2 years) after the first year following surgical or catheter-based intervention in an asymptomatic patient with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic patient to evaluate for aortic arch aneurysms, in-stent stenosis, stent fracture, or endoleak Routine surveillance (3–12 months) in a patient with heart failure symptoms Table 13: Congenital Heart Disease (CHD), Coronary Anomalies Unrepaired Routine surveillance (annually) in an asymptomatic patient with anomalous right coronary artery from the left aortic sinus Routine surveillance (2–5 years) in an asymptomatic patient with anomalous right coronary artery from the left aortic sinus Routine surveillance (annually) in an asymptomatic patient with small coronary fistula Routine surveillance (2–5 years) in an asymptomatic patient with small coronary fistula Routine surveillance (1–2 years) in an asymptomatic patient with moderate or large coronary fistula Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postprocedural: Surgical or Catheter-Based Routine post–procedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Medical Coverage Policy: 0510 Not rated A (9) A (9) A (9) A (9) Not rated Not rated A (9) A (9) Not rated A (9) TTE R (3) A (7) R (3) A (8) A (9) A (9) A (9) A (9) A (9) Evaluation within 1 year after surgery or catheter-based intervention with no or mild sequelae Routine surveillance (1–3 months) within the first year following repair Routine surveillance (3–6 months) in an infant with or without ventricular or valvular dysfunction Routine surveillance (3–6 months) in a child or adult with ventricular or valvular dysfunction Routine surveillance (annually) with no or mild sequelae Routine surveillance (2–5 years) with no or mild sequelae Table 14: Congenital Heart Disease (CHD), Tetralogy of Fallot (TOF) Unrepaired Routine surveillance (1–3 months) in an infant before complete repair Routine surveillance (1–3 months) in an infant following valvuloplasty, patent ductus arteriosus (PDA) and/or right ventricular outflow tract (RVOT) stenting, or shunt placement before complete repair Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postoperative: Initial Repair Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (annually) in an asymptomatic patient with no or mild sequelae or PR of any severity Routine surveillance (6–12 months) in a patient with valvular dysfunction other than pulmonary valve, RVOT obstruction, branch pulmonary artery stenosis, arrhythmias, or presence of a right ventricle to pulmonary artery (RV-to-PA) conduit Routine surveillance (2–3 years) in a patient with pulmonary regurgitation (PR) and preserved ventricular function Routine surveillance (3–12 months) in a patient with heart failure symptoms Evaluation prior to planned pulmonary valve replacement (percutaneous or surgical) Postprocedural: Surgical or Catheter-based Pulmonary Valve Replacement Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation at 1 year following transcatheter or surgical pulmonary valve replacement Routine surveillance at 1 and 6 month(s) in an asymptomatic patient following transcatheter pulmonary valve replacement Routine surveillance (annually) in an asymptomatic patient following transcatheter pulmonary valve replacement Routine surveillance (annually) in an asymptomatic patient with no or mild sequelae Medical Coverage Policy: 0510 A (9) A (7) A (9) A (9) A (7) Not rated TTE A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) Not rated A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) Routine surveillance (6–12 months) in a patient with RV-to-PA conduit dysfunction, valvular or ventricular dysfunction, branch pulmonary artery stenosis, or arrhythmias Routine surveillance (2–3 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (2–3 years) in a patient with valvular or ventricular dysfunction, RVOT obstruction, branch pulmonary artery stenosis, arrhythmias, or presence of an RV-to- PA conduit Routine surveillance (3–12 months) in a patient with heart failure symptoms A (9) Not rated A (9) A (9) Table 15: Congenital Heart Disease (CHD), Double Outlet Right Ventricle (DORV) Unrepaired Routine surveillance (1–3 months) in an infant with balanced systemic and pulmonary circulation Routine surveillance (3–6 months) in a child with balanced systemic and pulmonary circulation Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair TTE A (9) A (9) A (9) A (9) Postoperative Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (6 months) within a year following repair in an asymptomatic infant or child with no or mild sequelae Routine surveillance (1–2 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (3–12 months) in a patient with valvular or ventricular dysfunction, right or left ventricular outflow tract obstruction, branch pulmonary artery stenosis, arrhythmias, or presence of an right ventricle to pulmonary artery (RV-to-PA) conduit Routine surveillance (3–5 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (3–12 months) in a patient with heart failure symptoms A (9) A (9) A (9) A (9) A (9) Not rated A (9) Table 16: Congenital Heart Disease (CHD), D-Loop Transposition of the Great Arteries (D-Loop TGA) Unrepaired Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair TTE A (9) A (9) Postoperative: Arterial Switch Operation Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms A (9) A (9) Medical Coverage Policy: 0510 Evaluation for coronary imaging in an asymptomatic patient Routine surveillance (1–3 months) in an asymptomatic infant with moderate sequelae Routine surveillance (3–6 months) in an asymptomatic infant with no or mild sequelae Routine surveillance (3–12 months) in an asymptomatic child or adult with ≥ moderate valvular or ventricular dysfunction, right or left ventricular outflow tract obstruction, branch pulmonary artery stenosis, or arrhythmias Routine surveillance (1–2 years) in an asymptomatic child or adult with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic patient Routine surveillance (1–2 years) in a patient with dilated neoaortic root with increasing Z scores, or neoaortic regurgitation Routine surveillance (3–12 months) in a patient with heart failure symptoms Postoperative: Rastelli Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) within the first year following repair Routine surveillance (6 months) after the first year following repair in an asymptomatic patient with no or mild sequelae Routine surveillance (1–2 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic patient Routine surveillance (3–12 months) in a patient with ≥ moderate valvular dysfunction, LVOT obstruction, presence of an right ventricle to pulmonary artery (RV-to-PA) conduit, branch pulmonary artery stenosis, or arrhythmias Routine surveillance (3–12 months) in a patient with heart failure symptoms Postoperative: Atrial Switch Operation Evaluation due to concerning signs or symptoms and/or change in clinical status Routine surveillance (6 months) in an asymptomatic patient with no or mild sequelae Routine surveillance (1–2 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic patient Routine surveillance (3–12 months) in a patient with ≥ moderate systemic AV valve regurgitation, systemic RV dysfunction, LVOT obstruction, or arrhythmias Routine surveillance (3–12 months) in a patient with heart failure symptoms Medical Coverage Policy: 0510 Not rated A (9) A (9) A (9) A (9) Not rated A (9) A (9) A (9) A (9) A (9) A (9) A (9) Not rated A (9) A (9) A (9) R (3) A (9) Not rated A (9) A (9) Table 17: Congenital Heart Disease (CHD), Congenitally Corrected Transposition of the Great Arteries (ccTGA) Unrepaired Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) in an asymptomatic infant Routine surveillance (1–2 years) in a patient with < moderate systemic atrioventricular (AV) valve regurgitation Routine surveillance (6–12 months) in a patient with ≥ moderate systemic AV valve regurgitation Routine surveillance (3–5 years) in an asymptomatic patient Routine surveillance (3–12 months) in a patient with heart failure symptoms Evaluation prior to planned repair TTE A (9) A (9) A (9) A (9) A (9) A (9) TTE with contrast Not rated Not rated Not rated Not rated Not rated Not rated A (9) Not rated Postoperative: Anatomic Repair Routine post–operative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) within a year following repair in an asymptomatic patient with no or mild sequelae Routine surveillance (1–2 years) after the first year following repair in an asymptomatic patient with no or mild sequelae Routine surveillance (6–12 months) in a patient with valvular or ventricular dysfunction, right or left ventricular outflow tract obstruction, or presence of a right ventricle to pulmonary artery (RV-to-PA) conduit Routine surveillance (3–5 years) in an asymptomatic patient Routine surveillance (3–12 months) in a patient with heart failure symptoms A (9) A (9) M (5) M (5) A (9) Not rated A (9) Not rated A (9) Not rated Not rated Not rated Not rated A (9) Postoperative: Physiological Repair With Ventricular septal defect (VSD) Closure and/or Left ventricle to Pulmonary artery (LV-to-PA) Conduit Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) within a year following repair in an asymptomatic patient with no or mild sequelae Routine surveillance (1–2 years) in an asymptomatic patient with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic patient with no or mild A (9) A (9) A (9) A (9) A (9) Not rated Not rated Not rated Not rated Not rated Medical Coverage Policy: 0510 sequelae Routine surveillance (3–12 months) in a patient with ≥ moderate systemic AV valve regurgitation, systemic RV dysfunction, and/or LV-to-PA conduit dysfunction Routine surveillance (3–12 months) in a patient with heart failure symptoms A (9) A (9) Table 18: Congenital Heart Disease (CHD), Truncus Arteriosus Unrepaired Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned repair Postoperative Routine postprocedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (1–3 months) within the first year following repair in an asymptomatic patient Routine surveillance (6–12 months) after the first year following repair in an asymptomatic child or adult with no or mild sequelae Routine surveillance (3–5 years) in an asymptomatic child or adult with no or mild sequelae Routine surveillance (3–6 months) in an asymptomatic child or adult with ≥ moderate truncal stenosis and/or regurgitation Routine surveillance (1–2 years) in an asymptomatic child or adult with ≥ moderate truncal stenosis and/or regurgitation Routine surveillance (3–12 months) in a patient with known residual VSD, presence of an right ventricle to pulmonary artery RV-to-PA conduit, or branch pulmonary artery obstruction Routine surveillance (3–12 months) in a patient with heart failure symptoms Table 19: Congenital Heart Disease (CHD), Single-Ventricle Heart Disease Unrepaired TTE Routine surveillance (1–4 weeks) in a patient with balanced systemic and pulmonary circulation not requiring neonatal surgery Evaluation due to change in clinical status and/or new concerning signs or symptoms Evaluation prior to planned surgical palliation A (9) A (9) A (9) Postprocedural: Surgical and/or Catheter-Based (Stage 1 Palliation) Routine post-procedural evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms A (9) A (9) Medical Coverage Policy: 0510 Not rated Not rated TTE A (9) A (9) A (9) A (9) A (9) A (9) Not rated A (9) Not rated A (9) A (9) TTE with contrast Not rated Not rated Not rated Not rated Not rated Routine surveillance (1–4 weeks) in an asymptomatic infant Evaluation prior to planned stage 2 palliation A (9) A (9) Not rated Not rated Postoperative: Stage 2 Palliation Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (1–6 months) in an asymptomatic infant or child Routine surveillance (1–2 years) in an asymptomatic adult Evaluation prior to planned stage 3 palliation A (9) A (9) A (9) A (9) A (9) Not rated M (6) Not rated Not rated M (5) Postoperative: Stage 3 Palliation Routine postoperative evaluation (within 30 days) Evaluation due to change in clinical status and/or new concerning signs or symptoms Routine surveillance (3–6 months) within a year following stage 3 palliation in an asymptomatic patient Routine surveillance (6–12 months) after the first year following stage 3 palliation in an asymptomatic patient Routine surveillance (3–5 years) in an asymptomatic patient Routine surveillance (3–12 months) in a patient with valvular or ventricular dysfunction, arrhythmias, or other cardiac complications Routine surveillance (3–12 months) in a patient with heart failure symptoms A (9) A (9) A (9) A (9) A (9) A (9) A (9) R (3) M (6) Not rated Not rated Not rated Not rated Not rated 2021 American Heart Association (AHA)/ American College of Cardiology (ACC) Guideline for the Evaluation and Diagnosis of Chest Pain (Gulati, et al., 2021) 4.1.2. Intermediate-Risk Patients With Acute Chest Pain For intermediate-risk patients with acute chest pain, TTE is recommended as a rapid, bedside test to establish baseline ventricular and valvular function, evaluate for wall motion abnormalities, and to assess for pericardial effusion. (COR*: 1; LOE*: C-EO) 4.2. Evaluation of Acute Chest Pain With Nonischemic Cardiac Pathologies In patients with acute chest pain in whom other potentially life-threatening nonischemic cardiac conditions are suspected (e.g., aortic pathology, pericardial effusion, endocarditis), TTE is recommended for diagnosis. (COR: 1; LOE: C-EO) 4.2.3. Acute Chest Pain With Suspected Myopericarditis In patients with acute chest pain and suspected myopericarditis, TTE is effective to determine the presence of ventricular wall motion abnormalities, pericardial effusion, valvular abnormalities, or restrictive physiology. (COR: 1; LOE: C-EO) 4.2.4. Acute Chest Pain With Valvular Heart Disease (VHD) In patients presenting with acute chest pain with suspected or known history of VHD, TTE is useful in determining the presence, severity, and cause of VHD. (COR: 1; LOE: C-EO) Medical Coverage Policy: 0510 In patients presenting with acute chest pain with suspected or known VHD in whom TTE diagnostic quality is inadequate, TEE (with 3D imaging if available) is useful in determining the severity and cause of VHD.(COR: 1; LOE: C-EO) In patients presenting with acute chest pain with known or suspected VHD, CMR imaging is reasonable as an alternative to TTE and/or TEE is nondiagnostic. (COR: 2a; LOE: C-EO) 5.1.3. Intermediate-High Risk Patients With Stable Chest Pain and No Known CAD Assessment of Left Ventricular Function: In intermediate-high risk patients with stable chest pain who have pathological Q waves, symptoms or signs suggestive of heart failure, complex ventricular arrhythmias, or a heart murmur with unclear diagnosis, use of TTE is effective for diagnosis of resting left ventricular systolic and diastolic ventricular function and detection of myocardial, valvular, and pericardial abnormalities. (COR: 1; LOE: B- NR) See Appendix for ACC/AHA Class of Recommendation and Level of Evidence 2020 American College of Cardiology (ACC) / American Heart Association (AHA) Guideline for the Management of Patients With Valvular Heart Disease (Otto, et al., 2021) TTE is the standard initial diagnostic test in the initial evaluation of patients with known or suspected VHD. 2.7.4. Periodic Imaging After Valve Intervention In asymptomatic patients with any type of valve intervention, a baseline postprocedural TTE followed by periodic monitoring with TTE is recommended, depending on type of intervention, length of time after intervention, ventricular function, and concurrent cardiac conditions. (COR*: 1; LOE*: C-EO) 3.2.1.1. Diagnostic Testing: Initial Diagnosis of Aortic Stenosis (AS) In patients with signs or symptoms of AS or a bicuspid aortic valves (BAV), TTE is indicated for accurate diagnosis of the cause of AS, assessment of hemodynamic severity, measurement of LV size and systolic function, and determination of prognosis and timing of valve intervention. (COR: 1; LOE: A) In patients with suspected low-flow, low-gradient severe AS with normal LVEF (Stage D3), optimization of blood pressure control is recommended before measurement of AS severity by TTE, TEE, cardiac catheterization, or CMR (COR: 1; LOE: B-NR) 4.3.1. Diagnosis of Chronic Aortic Regurgitation (AR) In patients with signs or symptoms of AR, TTE is indicated for assessment of the cause and severity of regurgitation, LV size and systolic function, prognosis, and timing of valve intervention (COR: 1; LOE: B-NR) In patients with a BAV or with known dilation of the aortic sinuses or ascending aorta, TTE is indicated to evaluate the presence and severity of AR. (COR: 1; LOE: B-NR) Medical Coverage Policy: 0510 In patients with moderate or severe AR and suboptimal TTE images or a discrepancy between clinical and TTE findings, TEE, CMR, or cardiac catheterization is indicated for the assessment of LV systolic function, systolic and diastolic volumes, aortic size, and AR severity (COR: 1; LOE: B-NR) 5.1.1.1. Diagnostic Testing: Initial Diagnosis of BAV In patients with a known BAV, TTE is indicated to evaluate valve morphology, measure severity of AS and AR, assess the shape and diameter of the aortic sinuses and ascending aorta, and evaluate for the presence of aortic coarctation for prediction of clinical outcome and to determine timing of intervention (COR: 1; LOE: B-NR) In first-degree relatives of patients with a known BAV, a screening TTE might be considered to look for the presence of a BAV or asymptomatic dilation of the aortic sinuses and ascending aorta. (COR: 2b; LOE: B-NR) 6.2.1.1. Diagnostic Testing: Initial Diagnosis of Rheumatic MS In patients with signs or symptoms of rheumatic MS, TTE is indicated to establish the diagnosis, quantify hemodynamic severity, assess concomitant valvular lesions, and demonstrate valve morphology (to determine suitability for mitral commissurotomy) (COR: 1; LOE: B-NR) 7.2.2.1. Diagnostic Testing: Initial Diagnosis of Chronic MR In patients with known or suspected primary MR, TTE is indicated for baseline evaluation of LV size and function, RV function, LA size, pulmonary artery pressure, and the mechanism and severity of primary MR (Stages A to D) (COR: 1; LOE: B- NR) In patients with primary MR, when TTE provides insufficient or discordant information, TEE is indicated for evaluation of the severity of MR, mechanism of MR, and status of LV function (Stages B to D). (COR: 1; LOE: C-EO) 7.2.2.2. Diagnostic Testing: Changing Signs or Symptoms in Patients With Primary MR In patients with primary MR (Stages B to D) and new-onset or changing symptoms, TTE is indicated to evaluate the mitral valve apparatus and LV function (COR: 1; LOE: B-NR) 7.2.2.3. Diagnostic Testing: Routine Follow-Up for Chronic Primary MR For asymptomatic patients with severe primary MR (Stages B and C1), TTE is indicated every 6 to 12 months for surveillance of LV function (estimated by LVEF, LVEDD, and LVESD) and assessment of pulmonary artery pressure (COR: 1; LOE: B-NR) 7.3.2. Diagnosis of Chronic Secondary MR In patients with chronic secondary MR (Stages B to D), TTE is useful to establish the etiology and to assess the extent of regional and global LV remodeling and systolic dysfunction, severity of MR, and magnitude of pulmonary hypertension. (COR: 1; LOE: B-NR) 8.2.1. Diagnosis of Tricuspid Regurgitation (TR) In patients with TR, TTE is indicated to evaluate the presence and severity of TR, determine the etiology, measure the sizes of the right-sided chambers and inferior Medical Coverage Policy: 0510 vena cava, assess RV systolic function, estimate pulmonary artery systolic pressure, and characterize any associated left-sided heart disease (COR: 1; LOE: C-LD) 10.1. Diagnosis of Mixed VHD For patients with mixed valve disease, TTE is recommended to assess the etiology, severity, and pathophysiological impact. (COR: 1; LOE: C-EO) 11.1.1. Diagnosis and Follow-Up of Prosthetic Valves In patients with a surgical or transcatheter prosthetic valve and in patients who have had valve repair, an initial postprocedural TTE study is recommended for evaluation of valve hemodynamics and ventricular function (COR: 1; LOE: B-NR) In patients with a prosthetic valve or prior valve repair and a change in clinical symptoms or signs suggesting valve dysfunction, repeat TTE is recommended. (COR: 1; LOE: C-EO) 11.1.1. Diagnosis and Follow-Up of Prosthetic Valves In patients with a bioprosthetic surgical valve, TTE at 5 and 10 years and then annually after implantation is reasonable, even in the absence of a change in clinical status. (COR: 2a; LOE: C-LD) In patients with a bioprosthetic TAVI, TTE annually is reasonable.(COR: 2a; LOE: C- LD) 11.6.1. Diagnosis of Acute Mechanical Valve Thrombosis In patients with suspected mechanical prosthetic valve thrombosis, urgent evaluation with TTE, TEE, fluoroscopy, and/or multidetector CT imaging is indicated to assess valve function, leaflet motion, and the presence and extent of thrombus (COR: 1; LOE: B-NR) 11.8.1. Diagnosis of Prosthetic Valve Stenosis In patients with suspected mechanical or bioprosthetic valve stenosis, TTE and TEE are recommended to diagnosis the cause and severity of valve obstruction, assess ventricular function, and estimate pulmonaryartery systolic pressure (COR: 1; LOE: B-NR) 11.9.1. Diagnosis of Prosthetic Valve Regurgitation In patients with suspected mechanical or bioprosthetic valve regurgitation, TTE and TEE are recommended to determine the cause and severity of the leak, assess ventricular function, and estimate pulmonary artery systolic pressure (COR: 1; LOE: B-NR) 12.2. Diagnosis of IE In patients with suspected IE, TTE is recommended to identify vegetations, characterize the hemodynamic severity of valvular lesions, assess ventricular function and pulmonary pressures, and detect complications (COR: 1; LOE: B-NR) In patients with IE who have a change in clinical signs or symptoms (eg, new murmur, embolism, persistent fever, HF, abscess, or atrioventricular heart block) and in patients at high risk of complications (eg, extensive infected tissue, large vegetation on initial echocardiogram, or staphylococcal, enterococcal, or fungal infections), TTE and/or TEE are recommended for reevaluation (COR: 1; LOE: B-NR) Medical Coverage Policy: 0510 13.1. Initial Management of Women With VHD Before and During Pregnancy Women with suspected valve disease who are considering pregnancy should undergo a clinical evaluation and TTE before pregnancy (COR: 1; LOE: B-NR) See Appendix for ACC/AHA Class of Recommendation and Level of Evidence American Academy of Pediatrics The AAP Clinical Practice Guideline for the Diagnosis, Evaluation, and Treatment of Attention- Deficit/Hyperactivity Disorder in Children and Adolescents (Wolraich, et al., 2019) states the following: Stimulant medications, on average, increase patient heart rate (HR) and blood pressure (BP) to a mild and clinically insignificant degree. However, because stimulants have been linked to more substantial increases in HR and BP in a subset of individuals (5%–15%), clinicians are encouraged to monitor these vital signs in patients receiving stimulant treatment. Although concerns have been raised about sudden cardiac death among children and adolescents using stimulant and medications, it is an extremely rare occurrence. In fact, stimulant medications have not been shown to increase the risk of sudden death beyond that observed in children who are not receiving stimulants. Nevertheless, before initiating therapy with stimulant medications, it is important to obtain the child or adolescent’s history of specific cardiac symptoms in addition to the family history of sudden death, cardiovascular symptoms, Wolff-Parkinson-White syndrome, hypertrophic cardiomyopathy, and long QT syndrome. If any of these risk factors are present, clinicians should obtain additional evaluation to ascertain and address potential safety concerns of stimulant medication use by the child or adolescent. Among nonstimulants, the risk of serious cardiovascular events is extremely low, as it is for stimulants. Clinicians are recommended to not only obtain the personal and family cardiac history, as detailed above, but also to perform additional evaluation if risk factors are present before starting nonstimulant medications (ie, perform an electrocardiogram [ECG] and possibly refer to a pediatric cardiologist if the ECG is not normal). The American Board of Internal Medicine’s (ABIM) Foundation Choosing Wisely® Initiative American Society of Anesthesiologists (Five Things Physicians and Patients Should Question) (Released October 12, 2013; Last reviewed 2019): Don’t obtain baseline diagnostic cardiac testing (trans-thoracic/esophageal echocardiography – TTE/TEE) or cardiac stress testing in asymptomatic stable patients with known cardiac disease (e.g., CAD, valvular disease) undergoing low or moderate risk non- cardiac surgery. Use Outside the U.S. Recommendations for transthoracic echocardiography are contained in numerous European Society of Cardiology (ESC) guidelines, but a comprehensive TTE specific guideline or appropriate use criteria have not been published to date.