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Heart, section through the middle
Heart, section through the middle
Heart, front view
Heart, front view


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 blood circulation for the body. As a result, the right side of the heart must maintain the circulation for both the lungs and the body. 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: 5/17/2004
Reviewed By: Elchanan Bruckheimer MBBS, Director of Pediatric Cardiac Catheterization, Schneider Children's Medical Center Israel, Petach Tikva, Israel. Review provided by VeriMed Healthcare Network.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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