Partial anomalous pulmonary venous return

Last revised by Dr Joshua Yap on 21 Apr 2022

Partial anomalous pulmonary venous return (PAPVR), also known as partial anomalous pulmonary venous connection (PAPVC), is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the systemic circulation rather than in the left atrium.

The overall prevalence of PAPVR is 0.4-0.7% 9

Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. Cases of secondary pulmonary arterial hypertension have been reported 6,7.

Four types of PAPVR have been described:

Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children 3. It is unclear if this is because of a higher proportion of symptomatic manifestation of the latter. The left upper lobe vein anomaly is thought to be most common.

Chest radiographic features are particular to each subtype of PAPVR. The abnormal vein is rarely identified, except in cases of Scimitar syndrome. Pulmonary venous congestion can be seen if the venous drainage is obstructed.

Cardiomegaly can also be seen if significant abnormal intracardiac venous drainage occurs.

Utilization of contrast-enhanced studies with MDCT technology enables both detection and characterization of the anomalies. It is considered the imaging modality of choice 3,4.

Therapeutic options include surgical repair with ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation and Warden procedure inter alia.

Imaging differential considerations include:

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Cases and figures

  • Case 1: supracardiac into RSVC
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  • Case 2
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  • Case 3
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  • Case 4: Scimitar syndrome
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  • Case 5: anomalous left upper lobe vein
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  • Case 6: left-upper lobe PAPVR
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  • Case 7: supra cardiac PAPVR
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  • Case 8: supra-cardiac PAPVR
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  • Case 9: supracardiac
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  • Case 10: left upper with IJV catheter
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  • Case 11: Left-upper lobe partial anomalous pulmonary venous return
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  • Case 12: cardiac type
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  • Case 13: IJV catheter in left upper lobe PAPVR
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  • Case 14
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  • Case 15
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