Q23.4 Hypoplastic left heart syndrome

ICD10CM code

#### Name of the Condition
- Hypoplastic Left Heart Syndrome (HLHS)
- ICD-10 Code: Q23.4

#### Summary
Hypoplastic Left Heart Syndrome (HLHS) is a rare, congenital heart defect where the left side of the heart is underdeveloped. This structural abnormality can severely hinder the heart's ability to effectively pump blood throughout the body.

#### Causes
HLHS is a congenital condition, meaning it is present at birth. The exact cause is unknown, but it is believed to arise during fetal development. Most cases occur sporadically, though certain genetic factors may contribute.

#### Risk Factors
- Family history of congenital heart defects.
- Certain genetic conditions, such as Turner syndrome.
- Maternal factors such as obesity, diabetes, or medication use during pregnancy.

#### Symptoms
- Cyanosis (bluish skin coloration due to lack of oxygen).
- Rapid breathing or trouble breathing.
- Poor feeding and lack of energy.
- Cold extremities and weak pulse.

#### Diagnosis
- Prenatal fetal echocardiogram or ultrasound to identify structural heart abnormalities.
- Physical examination after birth.
- Echocardiography, chest X-ray, and MRI for detailed heart imaging.

#### Treatment Options
- Surgical interventions, such as the Norwood Procedure, Glenn Shunt, and Fontan Procedure, are performed in stages to improve blood flow.
- Heart transplantation may be necessary in severe cases.
- Medications to strengthen heart function and manage symptoms, such as diuretics or inotropes.

#### Prognosis and Follow-Up
- With surgical intervention, children with HLHS can survive into adulthood, but they require lifelong cardiac care.
- Regular follow-up with a pediatric cardiologist is crucial to monitor heart function and detect complications early.

#### Complications
- Heart failure.
- Arrhythmias (irregular heartbeats).
- Increased risk of stroke or blood clots.
- Endocarditis (heart infection).

#### Lifestyle & Prevention
- There are no known preventive measures for HLHS due to its congenital nature, but maintaining a healthy pregnancy might reduce the risk of other complications.
- Genetic counseling may be beneficial for families with a history of congenital heart defects.

#### When to Seek Professional Help
- Immediate medical care is needed if the newborn shows signs of distress, such as difficulty breathing or cyanosis.

#### Additional Resources
- American Heart Association [heart.org]
- Children’s Heart Foundation [childrensheartfoundation.org]
- The Pediatric Congenital Heart Association [conqueringchd.org]

#### Tips for Medical Coders
- Accurately document the specific surgical procedures performed for precise coding.
- Be aware of associated conditions and comorbidities for comprehensive coding.
- Double-check for correct ICD-10 code (Q23.4) and maintain consistency in documentation.

Medical Policies and Guidelines for Hypoplastic left heart syndrome

Related policies from health plans

Similar Codes