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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.

Etiology/Pathophysiology:
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.

Pathology:
Not applicable

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

DDX:

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

References:
See References Chapter.

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