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Hypoplastic left heart syndrome (HLHS) is one of several congenital (present at birth) heart defects in which the heart has only one fully functioning ventricle. These conditions are commonly referred to as single ventricle defects.
In an infant and child with a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, enters the right ventricle and is pumped through the pulmonary artery into the lungs to receive oxygen. The oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle and is pumped out to the body through the aorta. In HLHS, most of the structures on the left side of the heart are too small and underdeveloped (hypoplastic) to provide enough red blood flow for the body’s needs.
Wayne Tworetzky, MD, co-director of the Advanced Fetal Care Center at Boston Children's Hospital, describes the congenital heart defect HLHS
The most critical issue in HLHS is the small left ventricle, which needs to be large enough and strong enough to pump blood out to the body. If it’s too small, it simply can’t function effectively. Other left-heart structures can also be underdeveloped in varying degrees, including:
Despite the severity of the heart defect, the fetus or newborn with HLHS is able to survive because of two naturally occurring “holes in the heart” — patent ductus arteriosus and patent foramen ovale — which allow for the communication of blood between the left and right sides of the heart.
Once the PDA begins to close (a natural occurrence), the baby will become extremely sick, due to lack of blood supply to the body. At this point, treatment is necessary for the baby to survive.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”