What is Hypoplastic Left Heart Syndrome?

Posted on Posted in Diagnosis Information, Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome (HLHS) means left-side ventricle and ascending aorta of the heart are underdeveloped. This severe congenital heart defect prevents the heart from pumping a sufficient amount of oxygenated blood to the body, and is fatal without heart surgery very shortly after birth.

While in the womb, a baby with HLHS receives the mother’s oxygenated blood through via patent ductus arteriosus (PDA). The PDA typically closes within hours after birth. Where HLHS is present, the right side of the heart can’t pump blood to the rest of the body once the PDA closes.

There are surgical treatments for HLHS including heart transplant, the Norwood series of three surgeries. New treatments for HLHS are also being explored.

According to the Children’s Hospital of Philadelphia, Children who have had surgical reconstruction for HLHS require life-long care by a cardiologist experienced in congenital heart disease. Sometimes they experience serious health problems. Many remain on medication, and additional surgeries may be required.

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