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Virtual Pediatric Hospital: Paediapaedia: Hypoplastic Left Heart Syndrome (HLHS) Paediapaedia: Cardiovascular Diseases

Hypoplastic Left Heart Syndrome (HLHS)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Clinical Presentation:
Symptoms are early congestive heart failure, profound metabolic acidosis, and shock. Is the most frequent congen heart lesion associated with congestive heart failure and death in the newborn.

The components include aortic valve atresia, rudimentary left ventricle with or without mitral valve atresia, and hypoplastic ascending aorta that acts as a common coronary with retrograde flow via the ductus arterious. Pulmonary venous return is shunted to the right atrium by a small ASD. Cyanosis is due to poor peripheral perfusion and lung congestion.

Not applicable

Imaging Findings:
Cardiomegaly due to a dilated right heart and pulmonary trunk. Pulmonary venous hypertension is also see.


Pulmonary venous hypertension in the newborn - due to a congenital cardiovascular lesion obstruction blood flow distal to the pulmonary veins.

See References Chapter.

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