Hypoplastic Left Heart Syndrome (HLHS)

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

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.

• HLHS
• Critical aortic stenosis
• TAPVR (obstructed) - has a normal heart size
• Cor triatriatum
• Pulmonary venous atresia
• Non-cardiac AV fistula (vein of Galen aneurysm)
• Hemangioendothelioma
• Congenital myocarditis
• Arrythmias

References:
See References Chapter.

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See related Provider Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.

See related Patient Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.

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