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Hypoplastic Left Heart Syndrome and Pulmonary Veno-occlusive Disease in an Infant

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Abstract

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.

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Author information

Correspondence to Marise D’Souza.

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D’Souza, M., Vergales, J. & Anitha Jayakumar, K. Hypoplastic Left Heart Syndrome and Pulmonary Veno-occlusive Disease in an Infant. Pediatr Cardiol 34, 1949–1951 (2013). https://doi.org/10.1007/s00246-012-0498-y

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Keywords

  • Hypoplastic left heart syndrome (HLHS)
  • Total anomalous pulmonary venous return (TAPVR)
  • Lymphangiectasia
  • Pulmonary veno-occlusive (PVOD)