Medical knowledge, Patient Education
Hypoplastic Left Heart Syndrome
Hypoplastic left heart syndrome (HLHS) is a congenital heart defect that occurs in some babies. It involves underdevelopment of the left side of the heart, which plays a crucial role in pumping oxygen-rich blood to the body.
The heart is divided into two sides – left and right. The right side receives deoxygenated blood from the body and sends it to the lungs for oxygenation. After picking up oxygen, the blood returns to the left side of the heart, which then pumps it back out to the body to supply vital oxygen to the tissues.
In HLHS, several important structures on the left side of the heart are either small or missing, making it difficult for the heart to pump enough oxygen-rich blood to the body. Common issues associated with HLHS include a small or absent left ventricle (the left pumping chamber), abnormal valves between the heart and major blood vessels, an atrial septal defect (a hole between the right and left sides of the heart), and a small aorta.
Symptoms of HLHS usually become apparent within hours or days after birth and may include trouble breathing, fast breathing, weak pulse or pounding heartbeat, pale or blue skin, cold hands and feet, poor feeding, and lethargy.
The exact cause of HLHS is often unknown, but potential factors may include changes in genes or chromosomes during fetal development and environmental influences or maternal factors during pregnancy, such as diet or medications.
Certain factors increase the risk of HLHS, including being male, maternal low folic acid levels during pregnancy, and having a previous child with HLHS.
Treating HLHS requires a series of three surgeries that aim to improve blood flow from the right side of the heart to the rest of the body. After the surgeries, ongoing follow-up care with a cardiologist is crucial throughout the child’s life. In some cases, a heart transplant may be necessary in the future.
The three surgeries performed are as follows:
1. Norwood procedure: Done within the first two weeks after birth to aid the right side of the heart in pumping blood to both the lungs and the body.
2. Bidirectional Glenn shunt or hemi-Fontan procedure: Performed at around 4-6 months old to allow blood returning from the upper body to go straight to the lungs, reducing the workload of the right side of the heart.
3. Fontan procedure: Conducted at around 3 years old, enabling blood returning from the lower body to go directly to the lungs.
Prior to surgery, medications may be prescribed to strengthen the heartbeat, reduce blood pressure, and prevent fluid buildup in the lungs and body. Children with HLHS may also require specialized feeding and nutrition care, such as the use of feeding tubes or high-calorie formulas, to ensure they receive proper nutrition.
Parents and caregivers should work closely with healthcare providers, including nutrition specialists, to ensure the child’s well-being. It is essential to keep all follow-up visits with the healthcare team for ongoing monitoring and support.
If any concerning symptoms or issues arise, such as fever, poor feeding, rapid breathing, lethargy, or skin color changes, medical attention should be sought promptly. In cases of severe symptoms, immediate medical help should be sought through emergency services (911 in the U.S.).
While this information provides valuable insight, it is not intended to replace professional medical advice. Any questions or concerns should be discussed with a healthcare provider to ensure appropriate care and guidance for the child with HLHS.
