Hypoplastic left heart syndrome

Alternative Names

HLHS

Definition of Hypoplastic left heart syndrome

Hypoplastic left heart syndrome occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely. The condition is congenital (present at birth).

Causes, incidence, and risk factors

Hypoplastic left heart is a rare type of congenital heart disease. It is more common in males than in females.

Symptoms

At first, a newborn with hypoplastic left heart may appear normal. Symptoms usually occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include:

Signs and tests

A physical exam may show signs of heart failure:

Treatment

Once the diagnosis of hypoplastic left heart is made, the baby will be admitted to the neonatal intensive care unit. A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to keep blood circulating to the body by keeping the ductus arteriosus open.

Expectations (prognosis)

If left untreated, hypoplastic left heart syndrome is fatal. Survival rates for the staged repair continue to rise as surgical techniques and postoperative management improve. Survival after the first stage is more than 75%.

Review

Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 12/21/2009

Heart, section through the middle
Heart, front view

ADAM Medical Encyclopedia

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