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Heart, section through the middle
Heart, section through the middle
Heart, front view
Heart, front view
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Hypoplastic left heart

Definition:
Hypoplastic left heart describes the underdevelopment of the left side of the heart (left ventricle, aortic valve, and aorta). The condition is congenital (present at birth).

Alternative Names:
Hypoplastic left heart syndrome; HLHS

Causes, incidence, and risk factors:

Hypoplastic left heart is a rare type of congenital heart disease. The problem develops before birth when there is inadequate growth of the left ventricle and associated structures (aortic and mitral valves that guard the exit and entrance of the ventricle and the aorta which is the blood vessel that carries oxygenated blood from the left ventricle to the entire body).

In patients with this condition, the left side of the heart is unable to maintain circulation for the body. As a result, the right side of the heart must maintain both pulmonary (lung) and systemic (body) circulation. This extra workload eventually causes the heart to fail.

The only possibility of survival is a connection between the right and the left side of the heart through which blood may pass. This is called a shunt. Babies are normally born with two of these connections (the foramen ovale and the ductus arteriosus), which spontaneously close a few days after birth.

If these structures are allowed to close in a baby with hypoplastic left heart syndrome, however, the patient will quickly die because no blood will be pumped to the body.

There is no known cause of hypoplastic left heart syndrome. Up to 40% of patients with this condition have other birth defects.



Review Date: 5/7/2002
Reviewed By: Satish K. Rajagopal, M.D., Department of Pediatrics, Columbia Presbyterian Medical Center, The Children's Hospital of New York, New York, NY. Review provided by VeriMed Healthcare Network.

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