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Case Reports
. 2019 Apr 3:27:100833.
doi: 10.1016/j.rmcr.2019.100833. eCollection 2019.

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

Karim El-Kersh  1 , Elie Homsy  2 , Curt J Daniels  3   4 , J Shaun Smith  2
Affiliations
Case Reports

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

Karim El-Kersh et al. Respir Med Case Rep. .

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.

Keywords: ASD; CHD; PAH; PAPVR.

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Figures

Fig. 1
Fig. 1
CT chest with contrast shows A) Right sided SVC (white arrow) and left sided SVC (red arrow). B) PAPVR draining at the junction of right SVC and RA. C) Enlarged main pulmonary artery at 5.5 cm.
Fig. 2
Fig. 2
Chest CT with contrast and 3D Reconstruction shows A) Near complete anomalous pulmonary venous return on the left. The left pulmonary veins drains into the left innominate vein at the upper mediastinum via left vertical vein. B) The left lung had only one left lower lobe segmental branch draining into the left atrium (red arrow).
Fig. 3
Fig. 3
A- Chest CT with contrast and 3D reconstruction shows the anomalous pulmonary veins. B-Chest CT with IV contrast shows anomalous pulmonary veins and ASD.
Fig. 4
Fig. 4
A and B: Chest CT with contrast and 3D reconstruction shows PAPVR from RUL (red arrow) to mid SVC (white arrow).
Fig. 5
Fig. 5
Calculation of Qp/Qs. Formula (1) and (2) can be derived from the oxygen consumption formula (A). Formula (B) is Flamm equation for mixed venous saturation. (PV: pulmonary vein, PA: pulmonary artery, Ao: Aorta, MV: mixed venous, sat: saturation) [8].
See this image and copyright information in PMC

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