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Table of Contents   
Year : 2019  |  Volume : 12  |  Issue : 2  |  Page : 135-137
Isolated agenesis of the right pulmonary veins with pulmonary sequestration

1 Department of Paediatric Cardiology, Care Hospital, Hyderabad, Telangana, India
2 Glenmark Cardiac Center, Mumbai, Maharashtra, India

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Date of Web Publication30-Apr-2019


We report two cases of agenesis of the right pulmonary veins (PVs) associated with sequestration of the right lung with systemic to pulmonary collateral. Both the children were referred for evaluation of recurrent lower respiratory tract infections. Transthoracic echocardiographic evaluation showed reversal of flow in the right pulmonary artery (PA) and absent right PVs. Cardiac catheterization confirmed the diagnosis of agenesis of the right PVs. Transcatheter closure of collaterals was performed in view of significant shunt from systemic to PA. Both the patients are asymptomatic on follow-up.

Keywords: Dextrocardia, pulmonary sequestration, pulmonary veins

How to cite this article:
Patil S, Sachdeva S, Bakhru S, Dalvi B, Koneti NR. Isolated agenesis of the right pulmonary veins with pulmonary sequestration. Ann Pediatr Card 2019;12:135-7

How to cite this URL:
Patil S, Sachdeva S, Bakhru S, Dalvi B, Koneti NR. Isolated agenesis of the right pulmonary veins with pulmonary sequestration. Ann Pediatr Card [serial online] 2019 [cited 2020 Sep 23];12:135-7. Available from:

   Introduction Top

Embryologically, pulmonary veins (PVs) are formed when the splanchnic plexus around the lung buds fuses with the common PV from the posterior wall of the left atrium (LA). Defects during early embryonic stage may result in PV agenesis.[1],[2] Recurrent lower respiratory infection (LRTI) is the most common presentation due to association with pulmonary sequestration and systemic to pulmonary collaterals.[2],[3],[4] Symptomatic patients may benefit from transcatheter closure of collaterals.[5] Here, we report our experience of two cases of isolated right PV agenesis.

   Case Reports Top

Case 1

A 15-month-old boy presented with symptoms of failure to thrive (weight < 5th percentile) and two episodes of LRTIs requiring hospitalization. Systemic examination was unremarkable. Chest X-ray revealed a shift of mediastinum with reduced pulmonary vascularity on the right side. Transthoracic echocardiogram (TTE) showed small right pulmonary artery (RPA: 'z' score -1.8) with complete reversal of flow on color flow mapping. Spectral Doppler showed a venous flow pattern in the RPA [Figure 1]. The right PVs were not visualized. Diagnostic cardiac catheterization showed normal PA pressures. The pulmonary angiogram revealed negative washout of contrast from RPA toward the left PA (LPA) [Figure 2]. Levophase showed a normal drainage of the left-sided PVs to LA and absence of right PVs. Aortogram showed multiple collaterals from the descending thoracic aorta supplying the right lung and subsequently filling the RPA. Levophase showed opacification of the RPA followed by contrast washout into LPA and subsequently left PVs into LA, leading to an arterioarterial circular shunt (physiological left-to-right shunt). This suggested complete absent of flow from the main PA to RPA. As the right lung was not contributing to oxygenation, the multiple aortopulmonary collaterals were closed using multiple Gianturco coils (Cook Medical, Inc., Bloomington, IN, USA).
Figure 1: (a) Flow reversal with centripetal flow pattern on color flow mapping in the right pulmonary artery. (b) Spectral Doppler revealed a venous flow pattern in the right pulmonary artery

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Figure 2: (a) Pulmonary angiogram revealed negative washout of contrast from right pulmonary artery toward the left pulmonary artery. (b) Aortogram shows multiple collaterals to the right lung

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Case 2

A 4-year-old boy was evaluated in view of recurrent LRTIs. Chest X-ray showed mediastinal shift to the right with differential vascularity [Figure 3]. TTE revealed hypoplastic RPA ('z' score -3.2). The right PVs were not visualized whereas the left PVs had an increased venous return. A collateral was seen originating from the celiac axis and supplying right lung. Computed tomography (CT) angiogram confirmed the findings of TTE. There was associated aberrant left subclavian artery and hypoplastic right lung with thickened interlobar fissure [Figure 4].
Figure 3: Dextroposition of heart. Reduced right lung vascularity than the left

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Figure 4: (a) Right pulmonary veins not seen. (b) Small right pulmonary artery than the left pulmonary artery

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Cardiac catheterization showed O2 step-up of 15% from mixed venous to LPA, with Qp: Qs of 1.8:1. The PA pressures were normal. Angiographic findings were similar to Case 1, except a single collateral arising from the celiac axis. It was closed with an 8-mm Amplatzer Vascular Plug 4 (St. Jude Medical, Inc., MN, USA) [Figure 5].
Figure 5: (a) Aortogram showing collateral from the celiac axis to the right lower lobe. (b) Subsequent opacification of the right pulmonary artery without pulmonary venous phase. (c) Pulmonary angiogram done with balloon occlusion of collateral showing agenesis of right-sided pulmonary veins. The right pulmonary artery is small. (d) Vascular plug occlusion to collateral

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   Discussion Top

Atresia of unilateral PVs is a rare anomaly and nearly 50 cases were reported in the literature.[6] About 30%–50% are associated with other abnormalities, namely septal defects and complex anomalies such as single ventricle, veno-occlusive disease, and Scimitar syndrome.[7],[8],[9]

Embryologic basis

Pulmonary vasculature develops from splanchnic plexus surrounding the primitive lung buds. The splanchnic plexus during its development drains to systemic venous channels and establishes its communication with common PV by the end of 4th week. The connection between splanchnic plexus and systemic veins involutes subsequently. Faulty incorporation of PVs after the involution of their connections with systemic veins leads to atresia of PVs.[1],[2]

Pathophysiology and clinical attributes

Failure of PVs to drain to LA leads to various histopathologic abnormalities, including intimal and medial thickening, fibrosis, and dilation of other PVs [Figure 5]. The ipsilateral PA and lung are underdeveloped and receive systemic collaterals, which can lead to left-to-right shunt. This may cause pulmonary hypertension, hemoptysis, or proneness to recurrent LRTIs. The clinical presentation may vary from asymptomatic state to severe pulmonary hypertension. This is essentially due to the balance between systemic collateral supply to affected lung and efficiency of its drainage and secondary changes in lung parenchyma due to associated congenital heart defects.[2],[4]


Chest X-ray (mediastinal shift) and TTE (reversal flow in the ipsilateral PA) provide subtle clues to the diagnosis. Definitive diagnosis can be made either on CT angiogram or on pulmonary arterial wedge angiography. Nonvisualization of confluence of PVs to LA, presence of systemic collateral, and dilated bronchial veins on CT angiogram are in favor of PV atresia.[3],[5],[10] Cardiac catheterization conclusively demonstrates atresia of PVs, presence of systemic collaterals, degree of left-to-right shunt, and severity of pulmonary hypertension.[1],[3],[7]


Various treatment modalities employed include conservative management, transcatheter closure of systemic collaterals, and pneumonectomy. Transcatheter closure of systemic collaterals is indicated in children with recurrent LRTI, presence of pulmonary hypertension, and recurrent episodes of hemoptysis.[5] Both our patients responded very well to transcatheter closure of collaterals. The rarity of the condition poses difficulty to fathom its natural history; however, high mortality to the tune of 50% has been reported in various case reports.[1] Timely intervention can improve symptomatic status and alleviate progression of pathologic changes.

Our report highlights the importance of appropriate diagnostic evaluation and management of isolated right PV agenesis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Cullen S, Deasy PF, Tempany E, Duff DF. Isolated pulmonary vein atresia. Br Heart J 1990;63:350-4.  Back to cited text no. 1
Pourmoghadam KK, Moore JW, Khan M, Geary EM, Madan N, Wolfson BJ, et al. Congenital unilateral pulmonary venous atresia: Definitive diagnosis and treatment. Pediatr Cardiol 2003;24:73-9.  Back to cited text no. 2
Mataciunas M, Gumbiene L, Cibiras S, Tarutis V, Tamosiunas AE. CT angiography of mildly symptomatic, isolated, unilateral right pulmonary vein atresia. Pediatr Radiol 2009;39:1087-90.  Back to cited text no. 3
Kim Y, Yoo IR, Ahn MI, Han DH. Asymptomatic adults with isolated, unilateral right pulmonary vein atresia: Multidetector CT findings. Br J Radiol 2011;84:e109-13.  Back to cited text no. 4
Heyneman LE, Nolan RL, Harrison JK, McAdams HP. Congenital unilateral pulmonary vein atresia: Radiologic findings in three adult patients. AJR Am J Roentgenol 2001;177:681-5.  Back to cited text no. 5
Savaş Bozbaş Ş, Varan B, Akçay Ş. Right pulmonary venous atresia: A case report and review of literature. Tuberk Toraks 2012;60:254-7.  Back to cited text no. 6
Lee HY, Hwang B, Lee PC, Jan SL, Meng CC. Congenital atresia of unilateral pulmonary veins associated with a single ventricle: A rare case report and literature review. Circ J 2008;72:1544-6.  Back to cited text no. 7
Shrivastava S, Moller JH, Edwards JE. Congenital unilateral pulmonary venous atresia with pulmonary veno-occlusive disease in contralateral lung: An unusual association. Pediatr Cardiol 1986;7:213-9.  Back to cited text no. 8
Namvar M, Zoroufian A, Shirani S, SalehiOmran A, Davoodi G, Yazdanifard P, et al. Scimitar vein anomaly with total right-side pulmonary vein return to inferior vena cava concomitant with atresia of upper right-side pulmonary vein and secundum atrial septal defect. Heart Surg Forum 2009;12:E235-7.  Back to cited text no. 9
Eifer DA, Arsego FV, Torres FS. Unilateral pulmonary vein atresia: Evaluation by computed tomography. Radiol Bras 2013;46:376-8.  Back to cited text no. 10

Correspondence Address:
Dr. Nageswara Rao Koneti
Care Hospital, Banjara Hills, Hyderabad, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/apc.APC_55_18

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]