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.
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:
- persistent left superior vena cava
- right superior vena cava (most common)
- brachiocephalic veins (=innominate veins)
- right atrium
- coronary sinus
- a combination of two or more of the above anomalies
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.
- in ~40% of patients with right-sided PAPVC, an atrial septal defect is seen 3
- more rarely it is seen with ostium primum defect, a subtype of atrioventricular defects
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.
Utilisation 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.
Treatment and prognosis
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:
- 1. Chen JT. Radiologic demonstration of anomalous pulmonary venous connection and its clinical significance. CRC Crit Rev Diagn Imaging. 1979;11 (4): 383-422. - Pubmed citation
- 2. Cronin P, Sneider MB, Kazerooni EA et-al. MDCT of the left atrium and pulmonary veins in planning radiofrequency ablation for atrial fibrillation: a how-to guide. AJR Am J Roentgenol. 2004;183 (3): 767-78. doi:10.2214/ajr.183.3.1830767 - Pubmed citation
- 3. Ho ML, Bhalla S, Bierhals A et-al. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 2009;24 (2): 89-95. doi:10.1097/RTI.0b013e318194c942 - Pubmed citation
- 4. Porres DV, Morenza OP, Pallisa E et-al. Learning from the pulmonary veins. Radiographics. 2013;33 (4): 999-1022. doi:10.1148/rg.334125043 - Pubmed citation
- 5. Konen E, Raviv-Zilka L, Cohen RA et-al. Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 2003;23 (5): 1175-84. doi:10.1148/rg.235035004 - Pubmed citation
- 6. Sung WK, Au V, Rose A. Partial anomalous pulmonary venous return in patients with pulmonary hypertension. J Med Imaging Radiat Oncol. 2012;56 (3): 324-7. doi:10.1111/j.1754-9485.2012.02364.x - Pubmed citation
- 7. Sears EH, Aliotta JM, Klinger JR. Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension. Pulm Circ. 2012;2 (2): 250-5. doi:10.4103/2045-8932.97637 - Free text at pubmed - Pubmed citation