What Is Hypoplastic Left Heart Syndrome (HLHS)?
Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect where the left side of the heart is underdeveloped, making it difficult for the heart to pump blood. This congenital heart defect/blood vessel condition occurs during fetal development and is present at birth.
What Causes Hypoplastic Left Heart Syndrome?
The exact cause of HLHS is often unknown, but certain factors may increase the risk for congenital heart disease, including:
- Genetic conditions
- Family history of heart defects
- Maternal exposures during pregnancy
Early detection through prenatal screenings and diagnostic tests from healthcare professionals is crucial for understanding and managing HLHS.
How Is Hypoplastic Left Heart Syndrome Diagnosed?
HLHS is typically diagnosed during routine prenatal ultrasounds or shortly after birth. Our specialized pediatric cardiology team uses advanced imaging techniques and diagnostic tools to assess and confirm the diagnosis, ensuring a personalized and effective treatment plan. When a baby is not diagnosed during pregnancy, symptoms may develop within the first few hours or days of life. This can include rapid breathing and heartbeat, poor feeding, cold extremities, blue color of the skin, lips, and nailbeds and general weakness. Treatments after birth will focus on stabilizing your baby.
How Is Hypoplastic Left Heart Syndrome Treated?
Treatments for this heart problem can include fetal cardiac intervention, medication, and surgical options like biventricular repair, balloon atrial septostomy, and patent ductus arteriosus (PDA) stenting, a cutting-edge, minimally invasive treatment for babies born with a severe heart condition like HLHS.
Surgical treatment from healthcare professionals involves a series of staged surgical interventions. Our expert pediatric cardiac surgeons work closely with families to explain treatment options for the heart problem/blood flow issues, potential risks, and expected outcomes. These staged heart surgeries aim to reconstruct the heart’s anatomy, allowing for improved blood circulation. These procedures include:
- Norwood (usually done in the first days of life) – combines the pulmonary artery and aorta to direct flow coming back from the body and lungs back out to the body. An extra connection to the lungs is also made at this time to allow for adequate oxygenation.
- Bidirectional Glenn (done at 4 to 6 months) – The superior vena cava, which normally returns deoxygenated blood from the head and upper extremities back to the heart, is connected directly to the right pulmonary artery.
- Fontan (done at 18 months to 2 years) – The inferior vena cava, which returns blood from the lower extremities, liver, and kidneys, is connected via a conduit or baffle to the superior vena cava, which is already connected to the pulmonary arteries. At this point, ALL systemic venous return is going to the lungs, is oxygenated, returned to the pulmonary venous atrium to the single ventricle and out toward the body.
Post-Surgery Care and Follow-Up
After surgical intervention, ongoing care and monitoring are essential to help strengthen the heart and blood vessels. Our dedicated pediatric cardiology team will work with you to create a comprehensive post-surgery care plan. Regular check-ups, diagnostic tests, and therapeutic interventions may be recommended to ensure the best possible outcome for your child’s heart health.
Research and Innovation
As a leading healthcare institution, Rady Children’s actively participates in research and clinical trials aimed at advancing our understanding of HLHS as well as other heart defects and improving treatment outcomes. Our commitment to innovation ensures that your child receives the latest and most effective care available.