Hypoplastic Left Heart Syndrome and Pulmonary Veno-occlusive Disease in an Infant
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This report describes an infant with heterotaxy syndrome and severe hypoplasia of the left heart who presented with profound cyanosis at birth despite a large patent ductus arteriosus. Pulmonary venous return was difficult to demonstrate by echocardiography. Angiography showed total anomalous pulmonary venous return via a plexus that drained through the paravertebral veins and bilateral superior vena cavae. Autopsy confirmed these findings, and histopathology demonstrated severe occlusive changes within the pulmonary veins.
KeywordsHypoplastic left heart syndrome (HLHS) Total anomalous pulmonary venous return (TAPVR) Lymphangiectasia Pulmonary veno-occlusive (PVOD)
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