Hypoplastic Left Heart Syndrome (HLHS) in children

Hypoplastic Left Heart Syndrome (HLHS) is a complex and serious congenital heart defect. In HLHS, the left side of the heart is underdeveloped, including the left ventricle, mitral valve, aortic valve, and aorta. This condition severely affects the heart’s ability to pump blood effectively to the body.

Symptoms of HLHS

Symptoms usually manifest soon after birth and may include:

Cyanosis: A blue tint to the skin, lips, and nails, indicating low oxygen levels.
Rapid or Difficulty Breathing.
Poor Feeding: Difficulty feeding or poor weight gain.
Lethargy.
Cold hands and feet.

Diagnosis

Echocardiogram: Main diagnostic tool, shows the structure and function of the heart.
Chest X-ray: Assesses the size and shape of the heart and lungs.
Electrocardiogram (ECG): Records the electrical activity of the heart.
Cardiac Catheterization: Provides detailed information about the heart’s structures and pressures.

Treatment

HLHS requires a series of complex surgical procedures or a heart transplant:

Surgical Approach:
- Norwood Procedure: Performed shortly after birth, it reconstructs the heart so that the right ventricle pumps blood to both the lungs and the body.
- Glenn Procedure: Typically performed when the child is a few months old, it redirects blood from the upper body directly to the lungs.
- Fontan Procedure: Usually performed between 18 months and 4 years of age, it directs blood from the lower body to the lungs.
Heart Transplant: In some cases, a heart transplant may be considered as an alternative to the three-stage surgery.

Prognosis

With advances in surgical techniques, the survival rates for children with HLHS have improved, but the condition still poses significant risks.
Children with HLHS require lifelong cardiac care, and many face additional surgeries or interventions in the future.

Complications

Heart Failure.
Neurodevelopmental Delays.
Blood Clots and Stroke.
Arrhythmias (abnormal heart rhythms).
Liver Problems due to chronic venous congestion.

Preventive Measures

The exact cause of HLHS is not known, and it is not generally considered preventable.
Good prenatal care, including fetal ultrasounds, can help in early detection.

HLHS is one of the most challenging congenital heart defects to manage. Children with HLHS require specialized medical care throughout their lives, and their management involves a multidisciplinary approach including cardiology, cardiac surgery, nursing, nutrition, and developmental services.