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

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.

SUBMITTER: El-Kersh K

PROVIDER: S-EPMC6456451 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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

El-Kersh Karim K Homsy Elie E Daniels Curt J CJ Smith J Shaun JS

Respiratory medicine case reports 20190403

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 synd ...[more]

PMID: 31008046

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