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 Table of Contents  
Year : 2018  |  Volume : 4  |  Issue : 3  |  Page : 250-253

Atrioventricular septal defect with anomalous systemic venous and pulmonary venous connection

Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication11-Jan-2019

Correspondence Address:
Prof. Sachin Talwar
Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcs.jpcs_64_18

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An anomalous pulmonary venous and systemic connection associated with a partial atrioventricular septal defect (AVSD) is rare. A 23-year-old patient, who presented with dyspnea and cyanosis on exertion, was found to have an anomalous pulmonary and systemic venous drainage with separate drainage of the left superior caval vein and hepatic veins into the left atrium and a partial AVSD. A patch diversion was successfully performed using two patches of glutaraldehyde-treated pericardium to create a nonobstructive pulmonary and systemic venous drainage.

Keywords: Atrioventricular septal defect, congenital disease, surgery

How to cite this article:
Talwar S, Yadav M, Bhoje A, Siddharth B, Das S, Balasubramaniam U, Choudhary SK. Atrioventricular septal defect with anomalous systemic venous and pulmonary venous connection. J Pract Cardiovasc Sci 2018;4:250-3

How to cite this URL:
Talwar S, Yadav M, Bhoje A, Siddharth B, Das S, Balasubramaniam U, Choudhary SK. Atrioventricular septal defect with anomalous systemic venous and pulmonary venous connection. J Pract Cardiovasc Sci [serial online] 2018 [cited 2022 Jan 23];4:250-3. Available from: https://www.j-pcs.org/text.asp?2018/4/3/250/249945

  Introduction Top

Atrioventricular septal defect (AVSD) with anomalous pulmonary venous drainage and abnormal systemic venous drainage into the left atrium (LA) is an extremely rare combination. Surgical correction of such a complex association is demanding and requires careful planning. In this report, we present a patient with partial AVSD with abnormal systemic and pulmonary venous drainage who underwent complete repair using a combination of patches.

  Case Report Top

The parents of this patient provided written informed consent to publish this report provided his identity was not disclosed. The Institutional Ethics Committee waived the need for a formal approval.

A 23-year-old male presented with a history of progressive cyanosis since birth. On examination, there was central cyanosis with room air saturation of 74%. On auscultation, he had a fixed and loud second heart sound. A Grade III/VI pansystolic murmur was present at the apex, and a Grade III ejection systolic murmur was present in the left 2nd intercostal space at the left sternal border. A chest X-ray revealed cardiomegaly with dextrocardia [Figure 1]: I] and electrocardiogram showed the left axis deviation with a prolonged PR interval with evidence of incomplete right bundle branch block. Echocardiography revealed a common atrium with partial AVSD with moderate regurgitation of the left atrioventricular valve (AVV) and mild right AVV regurgitation. Echocardiographically estimated the systolic pulmonary artery pressure was 80 mmHg, and the predicted right ventricular systolic pressure was 90 mmHg. The systemic and pulmonary venous drainage was not clearly elucidated on echocardiography. Hence, a computed tomography (CT) angiogram [Figure 1]: II] was performed. It showed evidence of a partial AVSD defect with a small ventricular septal defect (VSD). There was a complete cleft of the left AVV. There was a large primum type of atrial septal defect (ASD) with an additional ASD of the secundum type, the two defects resulting in a nearly common atrium. The atrioventricular and ventriculoarterial connections were concordant. There was mesocardia with a midline liver and polysplenia. Both the atrial appendages were of the left atrial morphology, indicating a left atrial isomerism. The aortic arch was left sided. Both the left-sided pulmonary veins drained into the LA. Both right-sided pulmonary veins drained into the right atrium (RA) anterior to the plane of the interatrial septum, thus signifying the partial anomalous pulmonary venous drainage (PAPVC). The right superior caval vein (SVC) drained into the RA, whereas, the left SVC drained into the roof of the LA. The inferior caval vein (IVC) was located in the midline and drained into the RA. The hepatic veins formed a common confluence that drained into the LA on the left atrial side of the remnants of the interatrial septum on the left of the IVC. Intraoperative transesophageal echocardiography (TEE) was used for confirmation of the diagnosis.
Figure 1: I: A chest X-ray posteroanterior view showing cardiomegaly with dextrocardia, left bronchial isomerism, and midline liver. II: (a) Coronal maximum intensity projection section showing right superior vena cava draining into right atrium, (b) left superior vena cava draining into left atrium, (c) axial thin section showing atrioventricular canal defect, (d) axial thin section showing drainage of right superior pulmonary vein into right atrium and left superior pulmonary vein ostium into left atrium, (e) axial thin section showing drainage of right inferior pulmonary vein and left inferior pulmonary veins into right atrium and left atrium respectively, (f) coronal maximum intensity projection section showing separate drainage of inferior caval vein and hepatic vein into right atrium and left atrium, respectively.

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Surgical technique

The surgical approach was through a standard median sternotomy. After opening the pericardium, a dilated and tense main pulmonary artery was encountered. The pulmonary artery pressure was measured to be 66/34 mmHg against the systemic pressure of 100/61 mmHg. Cardiopulmonary bypass (CPB) was established by cannulation of the aorta, the right and left SVC, and IVC using angled cannulae with core cooling to 28°C. Antegrade cold blood cardioplegia (del Nido cardioplegia) was used to arrest the heart. After the RA was opened, the intracardiac anatomy was confirmed and found to be consistent with a diagnosis of a transitional AVSD with the VSD measuring only about 5 mm. There was a large ASD of the primum type and an additional secundum type of ASD, giving the appearance of a common atrium. There was a complete cleft of the anterior leaflet in the left AVV with almost a common atrial cavity. Gentle saline insufflation into the left ventricle revealed moderate left AVV regurgitation, whereas the right AVV was found to be nonregurgitant after saline insufflation into the right ventricle. The IVC drained into the RA, and the hepatic venous confluence drained into the LA. The left SVC drained into the roof of the LA. The right-sided pulmonary veins opened into the RA and the left side ones drained into the LA.

As afirst step, the cleft in the left AVV was closed completely using interrupted nonpledged polypropylene sutures. The valve was found to be competent on saline insufflation. The attention was now directed to route, the left SVC toward the RA. Due to the abnormal position of the RA and considerable depth of the opening of the left SVC, it was practically impossible to accomplish this through the RA. Therefore, the LA was opened by an incision starting at the base of the left atrial appendage and extending obliquely downward for about 5 cm. A patch of glutaraldehyde-treated autologous pericardium was chosen, and it was sutured in around the orifice of the left SVC to direct it toward the RA. This suturing then continued behind the mitral valve annulus toward the RA. The patch was then passed to the right atrial side, and the remainder of this suture line was completed from the RA in such a manner that the left SVC opening was left above the pericardial patch along with the left atrial appendage. The partition of both the atria continued, and the right AVV, hepatic vein, IVC, and SVC were kept above the pericardial patch in the RA, whereas the left AVV and the pulmonary veins were rerouted to LA, below the patch.

It was not possible to construct this partition using a single patch, hence, another patch of homograft pericardial patch (as the native pericardium was enough) was used to direct the hepatic veins toward the RA following which both patches were sutured together [Figure 2]. The AVSD was now repaired by using the standard modified single-patch technique as described by Nunn.[1] Both the right and the left atria were now closed, and the patient was uneventfully weaned off CPB on elective inotropic support of dopamine 5 μg/kg/min, dobutamine 5 μg/kg/min, and nitroglycerine 0.5 μg/kg/min. The CPB time was 226 min and the aortic cross-clamp time was 191 min. TEE after termination of CPB [Figure 3]: I] revealed no residual surgical defects with unobstructed drainage of the left SVC into the RA.
Figure 2: Schematic diagram showing atrioventricular septal defect repair with rerouting of pulmonary and systemic veins using pericardial patch. (a) Intraoperative anatomy showing transitional atrioventricular septal defect with common atrium, cleft left atrioventricular valve, and anomalous systemic and pulmonary venous drainage as described in text, (b) Proposed line of baffle reconstruction, and (c) After cleft atrioventricular valve repair, ventricular septal defect closure and composite two patch repair.

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Figure 3: I: (a and b) Transesophageal echocardiography mid-esophageal aortic valve short axis view showing the right-and left-sided pulmonary veins draining into right atrium and left atrium, respectively, (c) transesophageal echocardiography mid-esophageal four chamber view large atrial septal defect with the right superior caval vein draining into the right atrium, (d) transesophageal echocardiography mid-esophageal long axis view showing atrial septal defect patch with left superior vena cava draining into right atrium, (e) transesophageal echocardiography mid-esophageal right ventricle inflow outflow view showing atrial septal defect patch. II (a): Coronal maximum intensity projection section showing left superior vena cava, inferior vena cava, and hepatic vein draining in the right atrium. The demarcation between the right and left atrium suggesting the contour of pericardial patch, (b) coronal maximum intensity projection section showing both right and left pulmonary vein draining in the left atrium while inferior vena cava and hepatic vein draining in the right atrium, and (c) sagittal section showing right superior vena cava draining in the right atrium. AVSD: Atrioventricular septal defect, AVV: Atrioventricular valve, RSVC: Right superior vena cava, LSVC: Left superior vena cava, RA: Right atrium, LA: Left atrium, RSPV: Right superior pulmonary veins, LSPV: Left superior pulmonary veins, RIPV: Right inferior pulmonary veins, LIPV: Left inferior pulmonary veins, IVC: Inferior vena cava.

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Postoperative recovery was uneventful, and the patient was discharged after 5 days. Predischarge echocardiography demonstrated adequate repair with normal biventricular functioning. A follow-up CT angiogram [Figure 3]: II] revealed unobstructed systemic venous baffle.

  Discussion Top

Rarity of diagnosis

AVSD with anomalous pulmonary and systemic venous drainage is one of the rare forms of congenital heart defects and is even rarer when the hepatic veins and IVC drain separately into the LA. Partial AVSD accounts for approximately 1% of patients who undergo cardiac surgery for congenital heart disease per year in the United States.[2] However, there are few reports of PAPVC and anomalous systemic venous drainage with a partial AVSD. Studer et al.[3] reported that the condition was associated with PAPVC in only one patient (the right pulmonary vein into the RA) out of the 154 patients who underwent surgical repair for partial AVSD. King et al.[4] reported one patient with this anomalous combination among 199 patients who underwent a surgical correction of partial AVSD. Digilio et al.[5] reported that no association was observed with PAPVC in the 91 partial AVSD patients without Down syndrome. Bharati and Lev[6] also reported the absence of PAPVC in 57 pathological specimens of partial AVSD. Therefore, the anomalous systemic and pulmonary venous connections are rarely associated with partial AVSD.

Surgical approach and its advantage

Seike et al.[7] described rerouting of the anomalous pulmonary venous connection with partial AVSD by creating an ASD. In our patient, there was hardly any atrial septum and because it resembled a common atrium, rerouting of the left SVC that was draining into the LA was very difficult both due to its far and peculiar location and difficult exposure in view of dextrocardia. Hence, we decided to accomplish repair using a biatrial approach; initially, the LA was opened to reroute the left SVC into the RA and later the patch was passed into the RA where rest of the rerouting was performed. This approach was rendered simple because of the good exposure achieved by accomplishing repair through both the atria.

Patch material

Two patches

One autologous and another glutaraldehyde-treated homologous pericardium were used as one patch was insufficient. These patches were inexpensive, easy to handle, and probably eliminated the risk of thromboembolism that could have potentially occurred from a large prosthetic patch.


Informed consent was obtained from all individual participants included in the study.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Nunn GR. Atrioventricular canal: Modified single patch technique. Pediatr Card Surg Annu 2007;10:28-31.  Back to cited text no. 1
Jacobs JP, Lacour-Gayet FG, Jacobs ML, Clarke DR, Tchervenkov CI, Gaynor JW, et al. Initial application in the STS congenital database of complexity adjustment to evaluate surgical case mix and results. Ann Thorac Surg 2005;79:1635-49.  Back to cited text no. 2
Studer M, Blackstone EH, Kirklin JW, Pacifico AD, Soto B, Chung GK, et al. Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg 1982;84:523-42.  Back to cited text no. 3
King RM, Puga FJ, Danielson GK, Schaff HV, Julsrud PR, Feldt RH, et al. Prognostic factors and surgical treatment of partial atrioventricular canal. Circulation 1986;74:I42-6.  Back to cited text no. 4
Digilio MC, Marino B, Toscano A, Giannotti A, Dallapiccola B. Atrioventricular canal defect without down syndrome: A heterogeneous malformation. Am J Med Genet 1999;85:140-6.  Back to cited text no. 5
Bharati S, Lev M. The pathology of congenital heart disease. A personal experience with more than 6300 congenitally malformed hearts. Vol. 1. Armonk, New York: Futura; 1996. p. 467-78.  Back to cited text no. 6
Seike Y, Nakamura Y, Tagusari O, Domoto S, Nakano K, Nagashima M. PAPVC and partial AVSD in adult. Surg Today 2011;41:1421-3.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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