Partial anomalous pulmonary venous return: A case series with management approach

Karim El-Kersh, Elie Homsy, Curt J. Daniels, J. Shaun Smith

Research output: Contribution to journalArticlepeer-review

Abstract

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly that results in a left-to-right shunt. Based on the shunt fraction, PAPVR has a wide spectrum of presentations. If a significant left-to-right shunt is left unrepaired, pulmonary vascular remodeling can occur resulting in the development of pulmonary arterial hypertension (PAH). Furthermore, if the condition is associated with an atrial septal defect (ASD), the patient can develop shunt reversal and Eisenmenger's syndrome in setting of severe PAH. Management plans include close observation, surgical repair, and treatment with pulmonary artery vasodilator therapies. Here, we present multiple cases of PAPVR to highlight the wide spectrum of presentations and the individualized treatment for each case.

Original languageEnglish (US)
Article number100833
JournalRespiratory Medicine Case Reports
Volume27
DOIs
StatePublished - Jan 1 2019
Externally publishedYes

Keywords

  • ASD
  • CHD
  • PAH
  • PAPVR

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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