Hypoplastic Left Heart Syndrome

Overview

Hypoplastic left heart syndrome (HLHS) is a congenital abnormality in which the left side of the heart is severely underdeveloped. Without treatment, the defect is usually fatal within the first several days of life. Mayo specialists have extensive experience treating children with HLHS. Mayo Clinic has a unique, team approach to the management of babies with HLHS. Physicians from various specialties see the child at the same time, enhancing communication among the medical team and improving continuity of care.

A diagnosis of HLHS can be frightening and overwhelming. It is important to know that are there are options for babies with HLHS. A baby with HLHS requires urgent medical evaluation once symptoms develop and will need to be treated in a center with experienced pediatric cardiologists and cardiovascular surgeons. If HLHS is diagnosed during pregnancy, the parents should choose to have the baby delivered in a facility equipped to treat babies with HLHS.

Diagnosis

Hypoplastic left heart syndrome is usually diagnosed with an echocardiogram (ultrasound of the heart). Read more about hypoplastic left heart syndrome diagnosis.

Treatment Options

There are two major treatment options for babies with hypoplastic left heart syndrome — a three-stage surgical procedure, which reconstructs the child's heart so it can work using only two of the heart's four chambers and heart transplant. Read more about hypoplastic left heart syndrome treatment options.

About HLHS

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Hypoplastic left heart syndrome is an abnormality in which the left side of the heart (left ventricle, aortic valve and aorta) is severely underdeveloped. The condition is congenital (present at birth).

When the left-side heart structures are underdeveloped, the right side of the heart must pump blood to both the lungs and to the rest of the body. In the womb, a baby's body receives oxygen-rich blood through a communication (connection) called the patent ductus arteriosus (PDA). After birth, the PDA spontaneously begins to close. An undeveloped heart cannot pump efficiently enough to support life and that is when a baby with HLHS gets very sick. Without treatment, the defect is usually fatal within the first several days of life.