Cigna Transthoracic Echocardiography in Children - (0523) 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: 0523 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 the pediatric population (under age 18). Coverage Policy PALPITATIONS Initial outpatient transthoracic echocardiography (TTE) in an individual under age 18 is considered medically necessary: Palpitations with abnormal electrocardiogram (ECG) • Palpitations in an individual with known channelopathy • Palpitations with family history at a young age (before the age of 50 years) of sudden cardiac arrest or death and/or pacemaker or implantable defibrillator placement Palpitations with family history of cardiomyopathy • Palpitations in an individual with known cardiomyopathy Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Palpitations with no other symptoms or signs of cardiovascular disease, a benign family history, and no recent ECG or a normal ECG Palpitations with family history of a channelopathy ELECTROCARDIOGRAM (ECG) FINDINGS Initial outpatient TTE in an individual under age 18 is considered medically necessary: Supraventricular tachycardia • Premature ventricular contractions (PVC) in the prenatal or neonatal period • PVCs after the neonatal period • Ventricular tachycardia Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Premature atrial contractions (PAC) in the prenatal or neonatal period • PACs after the neonatal period • Sinus bradycardia • Sinus arrhythmia SYNCOPE Initial outpatient TTE in an individual under age 18 is considered medically necessary: Syncope with abnormal ECG • Syncope with family history of channelopathy • Syncope with family history at a young age (before the age of 50 years) of sudden cardiac arrest or death and/or pacemaker or implantable defibrillator placement Medical Coverage Policy: 0523 Syncope with family history of cardiomyopathy • Unexplained pre-syncope • Exertional syncope • Unexplained post-exertional syncope Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Syncope with or without palpitations and with no recent ECG • Syncope with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG Probable neurocardiogenic (vasovagal) syncope • Syncope or pre-syncope with a known non-cardiovascular cause CHEST PAIN Initial outpatient TTE in an individual under age 18 is considered medically necessary: Chest pain with other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG - Exertional chest pain • Non-exertional chest pain with abnormal ECG - • Chest pain with family history of sudden unexplained death or cardiomyopathy • Chest pain with family history of premature coronary artery disease • Chest pain with recent onset of fever • Chest pain with recent illicit drug use Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Chest pain with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG Non-exertional chest pain with no recent ECG or a normal ECG • Reproducible chest pain with palpation or deep inspiration MURMUR Initial outpatient TTE in an individual under age 18 is considered medically necessary: Presumptively innocent murmur with signs, symptoms, or findings of cardiovascular disease Pathologic murmur Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Presumptively innocent murmur with no symptoms, signs, or findings of cardiovascular disease and a benign family history OTHER / SYMPTOMS AND SIGNS Initial outpatient TTE in an individual under age 18 is considered medically necessary: Preparticipation assessment of an asymptomatic athlete with ≥1 of the following: abnormal examination, abnormal ECG, or family history of inheritable heart disease associated with sudden death Initial evaluation prior to exposure to medications/radiation that could result in cardiotoxicity/heart failure. Medical Coverage Policy: 0523 Pre-operative evaluation of cardiac structure and function prior to noncardiac solid organ transplantation. Monitoring for rejection in a cardiac transplant recipient • Cardiac structure and function evaluation in a potential heart donor • Re-evaluation (<1 y) in a patient previously or currently undergoing therapy with potentially cardiotoxic agents Periodic re-evaluation in a patient undergoing therapy with cardiotoxic agents and worsening symptoms Symptoms and/or signs suggestive of congestive heart failure, including but not limited to respiratory distress, poor peripheral pulses, feeding difficulty, decreased urine output, edema, and/or hepatomegaly Chest wall deformities and scoliosis pre-operatively • Suspected cardiac mass • Chest mass or concern for any impingement of structure on the heart • Signs and symptoms of endocarditis in the absence of blood culture data or a negative blood culture Unexplained fever without other evidence for cardiovascular or systemic involvement • Central cyanosis • Multisystem Inflammatory Syndrome (MIS) associated with SARS-CoV-2 (COVID-19) infection Individual taking FINTEPLA® (fenfluramine) for a FDA-approved indication (e.g., Dravet syndrome) Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Preparticipation athlete assessment in an individual patient no symptoms, normal examination, and no family history of inheritable heart disease Screening study prior to starting Attention-deficit/Hyperactivity disorder (ADHD) drugs • Fatigue with no other signs and symptoms of cardiovascular disease, a normal ECG, and a benign family history Isolated acrocyanosis PRIOR TEST RESULTS Initial outpatient TTE in an individual under age 18 is considered medically necessary: Known channelopathy • Genotype positive for cardiomyopathy • Abnormal chest X-ray findings suggestive of cardiovascular disease • Abnormal ECG without symptoms • Desaturation based on pulse oximetry • Previously normal echocardiogram with a change in cardiovascular status and/or a new family history suggestive of heritable heart disease Chromosomal abnormality known to be associated with cardiovascular disease • Chromosomal abnormality with undefined risk for cardiovascular disease • Positive blood cultures suggestive of infective endocarditis • Abnormal cardiac biomarkers • Abnormal barium swallow or bronchoscopy suggesting vascular ring Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Previously normal echocardiogram with no change in cardiovascular status or family history Elevated anti-streptolysin O titers without suspicion for rheumatic fever Medical Coverage Policy: 0523 SYSTEMIC DISORDERS Initial outpatient TTE in an individual under age 18 is considered medically necessary: Cancer without chemotherapy • Prior to, during or following chemotherapy in cancer • Sickle cell disease and other hemoglobinopathies • Connective tissue disorder such as Marfan, Loeys Dietz, and other aortopathy syndromes • Suspected connective tissue disorder • Clinically suspected syndrome or extracardiac congenital anomaly known to be associated with congenital heart disease • Human immunodeficiency virus infection • Suspected or confirmed Kawasaki disease • Suspected or confirmed Takayasu arteritis • Suspected or confirmed acute rheumatic fever • Systemic lupus erythematosis and autoimmune disorders • Muscular dystrophy • Systemic hypertension • Renal failure • Obesity with obstructive sleep apnea • Obesity with other cardiovascular risk factors • Stroke • Suspected pulmonary hypertension • Hepatic disorders • Failure to thrive • Storage diseases, mitochondrial and metabolic disorders • Abnormalities of visceral or cardiac situs Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Obesity without other cardiovascular risk factors • Diabetes mellitus • Lipid disorders • Seizures, other neurologic disorders, or psychiatric disorders • Gastrointestinal disorders, not otherwise specified FAMILY HISTORY OF CARDIOVASCULAR DISEASE IN PATIENTS WITHOUT SIGNS OR SYMPTOMS AND WITHOUT CONFIRMED CARDIAC DIAGNOSIS Initial outpatient TTE in an individual under age 18 is considered medically necessary: Family history of Unexplained sudden death before the age of 50 years - • Family history of Hypertrophic cardiomyopathy • Family history of Non-ischemic dilated cardiomyopathy • Family history of Other cardiomyopathies • Family history of Genetic disorder at high risk for cardiovascular involvement • Family history of Marfan or Loeys Dietz syndrome • Family history of Connective tissue disorder other than Marfan or Loeys Dietz syndrome • Family history of Congenital left-sided heart lesion, including but not limited to mitral stenosis, left ventricular outflow tract obstruction, bicuspid aortic valve, aortic coarctation, and/or hypoplastic left heart syndrome Family history of Congenital heart disease other than the congenital left-sided heart lesions Family history of Idiopathic pulmonary arterial hypertension Medical Coverage Policy: 0523 Family history of Heritable pulmonary arterial hypertension Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Family history of Premature coronary artery disease before the age of 50 years • Family history of Channelopathy • Family history of Unspecified cardiovascular disease • Family history of Disease at high risk for cardiovascular involvement, including but not limited to diabetes, systemic hypertension, obesity, stroke, and peripheral vascular disease Family history of Pulmonary arterial hypertension other than idiopathic and heritable • Family history of Consanguinity OUTPATIENT NEONATES WITHOUT POST-NATAL CARDIOLOGY EVALUATION Initial outpatient TTE in an individual under age 18 is considered medically necessary: Suspected cardiovascular abnormality on fetal echocardiogram • Maternal infection during pregnancy or delivery with potential fetal/neonatal cardiac sequelae Maternal diabetes with no prior fetal echocardiogram • Maternal diabetes with a normal fetal echocardiogram • Maternal phenylketonuria • Maternal autoimmune disorder • Maternal teratogen exposure Initial outpatient TTE in an individual under age 18 is not covered or reimbursable: Isolated echogenic focus on fetal ultrasound ESTABLISHED CONGENITAL HEART DISEASE 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, 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 Medical Coverage Policy: 0523 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 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. 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. Lack of radiation exposure and the non-invasive, painless nature of TTE make it an ideal diagnostic tool in the pediatric population. 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 chest x-ray, stress echocardiography, 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 Medical Coverage Policy: 0523 arteriography, and positron emission tomography (PET). In some cases TTE may be the sole diagnostic procedure, while in other situations additional testing is required. 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. Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology (Campbell, et al., 2014) 2. Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease (Sachdeva, et al., 2020) American College of Cardiology/American Academy of Pediatrics/American Heart Association The ACC/AAP/AHA 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology (Campbell, et al., 2014) addresses the initial use of outpatient TTE during pediatric (≤18 years of age) outpatient care. • Although TTE is an essential tool in hospitalized patients, discussion of indications for this use is beyond the scope of the 2014 document. Includes 103 separate TTE indications Additionally, the use of TTE in patients with previously known structural, functional, or primary electrical cardiac abnormalities is not addressed within this document. Ratings: Median score 7-9: Appropriate test for specific indication (test is generally acceptable and is a reasonable approach for the indication). Median score 4-6: 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. Median score 1-3: Rarely Appropriate test for specific indication (test is not generally acceptable and is not a reasonable approach for the indication). Note: Fifty-three indications were identified as Appropriate, 28 as May Be Appropriate, and 32 as Rarely Appropriate. PALPITATIONS Appropriate Palpitations with family history at a young age (before the age of 50 years) of sudden cardiac arrest or death and/or pacemaker or implantable defibrillator placement (7) Palpitations with family history of cardiomyopathy (9) • Palpitations in a patient with known cardiomyopathy (9) May Be Appropriate Palpitations with abnormal electrocardiogram (ECG) (6) • Palpitations in a patient with known channelopathy (4) Rarely Appropriate Palpitations with no other symptoms or signs of cardiovascular disease, a benign family history, and no recent ECG (2) Medical Coverage Policy: 0523 Palpitations with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG (1) Palpitations with family history of a channelopathy (3) ELECTROCARDIOGRAM (ECG) FINDINGS Appropriate Supraventricular tachycardia (7) • Ventricular tachycardia (9) May Be Appropriate Premature ventricular contractions (PVC) in the prenatal or neonatal period (6) • PVCs after the neonatal period (6) Rarely Appropriate Premature atrial contractions (PAC) in the prenatal or neonatal period (3) • PACs after the neonatal period (3) • Sinus bradycardia (2) • Sinus arrhythmia (1) SYNCOPE Appropriate Syncope with abnormal ECG (7) • Syncope with family history at a young age (before the age of 50 years) of sudden cardiac arrest or death and/or pacemaker or implantable defibrillator placement (9) Syncope with family history of cardiomyopathy (9) • Exertional syncope (9) • Unexplained post-exertional syncope (7) May Be Appropriate Syncope with family history of channelopathy (5) • Unexplained pre-syncope (4) Rarely Appropriate Syncope with or without palpitations and with no recent ECG (3) • Syncope with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG (2) Probable neurocardiogenic (vasovagal) syncope (2) • Syncope or pre-syncope with a known non-cardiovascular cause (2) CHEST PAIN Appropriate Exertional chest pain (8) • Non-exertional chest pain with abnormal ECG (7) • Chest pain with family history of sudden unexplained death or cardiomyopathy (8) May Be Appropriate Chest pain with other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG (6) Chest pain with family history of premature coronary artery disease (4) • Chest pain with recent onset of fever (6) • Chest pain with recent illicit drug use (6) Rarely Appropriate Chest pain with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG (2) Medical Coverage Policy: 0523 Non-exertional chest pain with no recent ECG (3) • Non-exertional chest pain with normal ECG (1) • Reproducible chest pain with palpation or deep inspiration (1) MURMUR Appropriate Presumptively innocent murmur with signs, symptoms, or findings of cardiovascular disease (7) Pathologic murmur (9) Rarely Appropriate Presumptively innocent murmur with no symptoms, signs, or findings of cardiovascular disease and a benign family history (1) OTHER / SYMPTOMS AND SIGNS Appropriate Symptoms and/or signs suggestive of congestive heart failure, including but not limited to respiratory distress, poor peripheral pulses, feeding difficulty, decreased urine output, edema, and/or hepatomegaly (9) Signs and symptoms of endocarditis in the absence of blood culture data or a negative blood culture (8) • Central cyanosis (8) May Be Appropriate Chest wall deformities and scoliosis pre-operatively (6) • Unexplained fever without other evidence for cardiovascular or systemic involvement (5) Rarely Appropriate Fatigue with no other signs and symptoms of cardiovascular disease, a normal ECG, and a benign family history (3) Isolated acrocyanosis (1) PRIOR TEST RESULTS Appropriate Genotype positive for cardiomyopathy (9) • Abnormal chest X-ray findings suggestive of cardiovascular disease (9) • Abnormal ECG without symptoms (7) • Desaturation based on pulse oximetry (9) • Previously normal echocardiogram with a change in cardiovascular status and/or a new family history suggestive of heritable heart disease (7) Chromosomal abnormality known to be associated with cardiovascular disease (9) • Positive blood cultures suggestive of infective endocarditis (9) • Abnormal cardiac biomarkers (9) • Abnormal barium swallow or bronchoscopy suggesting vascular ring (7) May Be Appropriate Known channelopathy (4) • Chromosomal abnormality with undefined risk for cardiovascular disease (5) Rarely Appropriate Previously normal echocardiogram with no change in cardiovascular status or family history (1) Elevated anti-streptolysin O titers without suspicion for rheumatic fever (3) SYSTEMIC DISORDERS Appropriate Medical Coverage Policy: 0523 Prior to or during chemotherapy in cancer (8) • Sickle cell disease and other hemoglobinopathies (8) • Connective tissue disorder such as Marfan, Loeys Dietz, and other aortopathy syndromes (9) Suspected connective tissue disorder (7) • Clinically suspected syndrome or extracardiac congenital anomaly known to be associated with congenital heart disease (9) • Human immunodeficiency virus infection (8) • Suspected or confirmed Kawasaki disease (9) • Suspected or confirmed Takayasu arteritis (9) • Suspected or confirmed acute rheumatic fever (9) • Systemic lupus erythematosis and autoimmune disorders (7) • Muscular dystrophy (9) • Systemic hypertension (9) • Renal failure (7) • Stroke (8) • Suspected pulmonary hypertension (9) • Storage diseases, mitochondrial and metabolic disorders (8) • Abnormalities of visceral or cardiac situs (9) May Be Appropriate Cancer without chemotherapy (5) • Obesity with obstructive sleep apnea (6) • Obesity with other cardiovascular risk factors (6) • Hepatic disorders (4) • Failure to thrive (5) Rarely Appropriate Obesity without other cardiovascular risk factors (2) • Diabetes mellitus (3) • Lipid disorders (3) • Seizures, other neurologic disorders, or psychiatric disorders (2) • Gastrointestinal disorders, not otherwise specified (2) FAMILY HISTORY OF CARDIOVASCULAR DISEASE IN PATIENTS WITHOUT SIGNS OR SYMPTOMS AND WITHOUT CONFIRMED CARDIAC DIAGNOSIS Appropriate Family history of Hypertrophic cardiomyopathy (9) • Family history of Non-ischemic dilated cardiomyopathy (9) • Family history of Other cardiomyopathies (8) • Family history of Genetic disorder at high risk for cardiovascular involvement(7) • Family history of Marfan or Loeys Dietz syndrome (7) • Family history of Heritable pulmonary arterial hypertension (8) May Be Appropriate Family history of Unexplained sudden death before the age of 50 years (6) • Family history of Connective tissue disorder other than Marfan or Loeys Dietz syndrome (6) Family history of Congenital left-sided heart lesion, including but not limited to mitral stenosis, left ventricular outflow tract obstruction, bicuspid aortic valve, aortic coarctation, and/or hypoplastic left heart syndrome (6) Family history of Congenital heart disease other than the congenital left-sided heart lesions (5) Family history of Idiopathic pulmonary arterial hypertension (5) Medical Coverage Policy: 0523 Rarely Appropriate Family history of Premature coronary artery disease before the age of 50 years (2) • Family history of Channelopathy (3) • Family history of Unspecified cardiovascular disease (3) • Family history of Disease at high risk for cardiovascular involvement, including but not limited to diabetes, systemic hypertension, obesity, stroke, and peripheral vascular disease (2) Family history of Pulmonary arterial hypertension other than idiopathic and heritable (3) • Family history of Consanguinity (3) OUTPATIENT NEONATES WITHOUT POST-NATAL CARDIOLOGY EVALUATION Appropriate Suspected cardiovascular abnormality on fetal echocardiogram (9) • Maternal infection during pregnancy or delivery with potential fetal/neonatal cardiac sequelae (7) Maternal phenylketonuria (7) May Be Appropriate Maternal diabetes with no prior fetal echocardiogram (6) • Maternal diabetes with a normal fetal echocardiogram (4) • Maternal autoimmune disorder (5) • Maternal teratogen exposure (6) Rarely Appropriate Isolated echogenic focus on fetal ultrasound (2) 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: Includes 324 separate TTE indications • Addresses cardiac imaging in adult and pediatric patients with established Congenital Heart Disease. 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: 0523 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 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 A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) M (6) A (9) A (9) A (9) M (5) M (6) R (3) R (3) R (3) R (3) R (2) R (2) R (2) R (2) M (4) M (5) Medical Coverage Policy: 0523 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 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 Medical Coverage Policy: 0523 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) TTE A (9) A (9) A (9) A (9) A (9) A (9) A (9) 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 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 Medical Coverage Policy: 0523 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) A (7) A (9) A (9) TTE A (9) A (9) A (9) A (9) A (8) A (8) 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 TTE A (9) A (9) A (9) M (6) A (9) R (3) A (9) 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 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 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 TTE A (9) A (9) A (9) A (9) A (9) A (9) TTE with contrast Not rated M (5) Not rated M (4) A (7) M (6) A (9) M (6) 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 A (9) A (9) A (9) A (9) M (6) A (7) Not rated Not rated Not rated Medical Coverage Policy: 0523 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) 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 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 Medical Coverage Policy: 0523 Not rated Not rated TTE A (8) A (8) 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) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) 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 A (9) A (9) A (9) Table 10: Congenital Heart Disease (CHD), Mitral Valve Disease Unrepaired Congenital Mitral Stenosis (MS) 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 TTE 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) Routine surveillance (1–3 months) in an infant with any degree of subvalvular aortic stenosis (AS) and ≤ mild aortic regurgitation (AR) TTE A (9) Medical Coverage Policy: 0523 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 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 Medical Coverage Policy: 0523 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) A (9) A (9) A (9) A (9) A (9) 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 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 Medical Coverage Policy: 0523 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) Not rated A (9) A (9) A (9) A (9) Not rated Not rated A (9) 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 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 Medical Coverage Policy: 0523 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) 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) 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 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 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 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 Medical Coverage Policy: 0523 A (9) Not rated A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) Not rated A (9) A (9) TTE A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) A (9) 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 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 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 A (9) A (9) Not rated A (9) A (9) A (9) A (9) Not rated A (9) A (9) 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 A (9) A (9) A (9) A (9) A (9) Not rated A (9) A (9) 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 A (9) R (3) A (9) Not rated A (9) Medical Coverage Policy: 0523 Routine surveillance (3–12 months) in a patient with heart failure symptoms 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 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 A (9) A (9) A (9) A (9) A (9) A (9) Not rated Not rated Not rated Not rated Not rated Not rated Medical Coverage Policy: 0523 Routine surveillance (3–12 months) in a patient with heart failure symptoms 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 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 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 Routine surveillance (1–4 weeks) in an asymptomatic infant Evaluation prior to planned stage 2 palliation 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 Postoperative: Stage 3 Palliation Routine postoperative evaluation (within 30 days) Medical Coverage Policy: 0523 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) 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 Not rated Not rated Not rated M (6) Not rated Not rated M (5) R (3) 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) M (6) Not rated Not rated Not rated Not rated Not rated Myocardial Strain Imaging Refer to Cigna Coverage Policy 0510 Transthoracic Echocardiography in Adults. 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). American Heart Association The AHA Pre-participation Cardiovascular Screening of Young Competitive Athletes: Policy Guidance (Updated 9/2021) notes that the AHA recommends the following with regards to preparticipation screening of young competitive athletes: Competitive athletic prescreening should happen annually and consist of a targeted personal history, family history and physical examination. This includes 14 key prescreening elements such as a history of elevated systemic blood pressure, knowledge of certain cardiac conditions in family members, and the presence of a heart murmur that are designed to identify, or at least raise the suspicion of, cardiovascular diseases that Medical Coverage Policy: 0523 place certain athletes at risk. Those athletes with positive findings should be referred for further evaluation and testing At this time, the AHA does not recommend the use of tests such as a 12-lead ECG or echocardiogram in mandatory preparticipation screening programs. Instead, these tests should be used as follow-up if an initial screening raises suspicions about the presence of a cardiovascular disease. American Academy of Pediatrics (AAP) The American Academy of Pediatrics, American Academy of Family Physicians, American College of Sports Medicine, American Medical Society for Sports Medicine, American Medical Society for Sports Medicine, American Orthopaedic Society for Sports Medicine, and American Osteopathic Academy of Sports Medicine published the Preparticipation Physical Evaluation (PPE), 5th Edition on May 1, 2019. The AAP notes although the PPE has been used for over 50 years, it lacks data on effectiveness and was not developed as an evidence-based process. The AAP Policy Statement ‘Sudden Death in the Young: Information for the Primary Care Provider’ (July 2021) states these Recommendations / Primary Take-away Points: 1. All children should be evaluated for conditions predisposing to sudden cardiac arrest (SCA) and sudden cardiac death (SCD) in the course of routine health care. 2. A thorough and detailed history, family history, and physical examination are necessary to begin assessing SCA and SCD risk. 3. The ECG should be the first test ordered when there is concern for SCA risk. The ECG should be interpreted by a physician trained in recognizing electrical heart disease (ie, a pediatric cardiologist or pediatric electrophysiologist). To provide optimal care, ECGs should not be performed in isolation without clinical history; referral to a specialist should be considered. 4. Do not trust the computer interpretation of the ECG (Erickson, et al., 2021). U.S. Preventive Services Task Force (USPSTF) The USPSTF does not list any pediatric guidelines addressing preparticipation screening or TTE. The American Board of Internal Medicine’s (ABIM) Foundation Choosing Wisely® Initiative: The American Academy of Pediatrics – Section on Cardiology and Cardiac Surgery states: Do not routinely order a screening ECG as part of a sports preparticipation examination in asymptomatic, otherwise healthy patients with no personal or family history of cardiac disease (Released November 2, 2020) Use Outside of the US Asian Pacific Society of Cardiology: The Asian Pacific Society of Cardiology Consensus Recommendations for Pre-participation Screening in Young Competitive Athletes (Wang, et al., 2021) states that the routine use of TTE is not recommended as a first-line investigation pre- participation screening by any professional societies. TTE plays an integral role in the evaluation of competitive athletes with suspected or confirmed cardiovascular disease.