Case Study

Total anomalous systemic and pulmonary venous connection

Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(1) 61–63 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313495859 aan.sagepub.com

Amit Mishra, Pranav Sharma, Ritesh Shah, Nilesh Oswal and Yashpal Rana

Abstract Total anomalous systemic and pulmonary venous connection is an extremely rare congenital cardiac anomaly. We present our unique experience of managing this complex partially diagnosed cardiac anomaly in a 16-month-old boy. The systemic venous anomaly was not detected during the initial preoperative evaluation. He was doing well on followup, with normal pulmonary artery pressure.

Keywords Congenital heart defect, Atrial septal defect, Pulmonary veins, Superior vena cava, Inferior vena cava

Introduction Total anomalous pulmonary venous connection and total anomalous systemic venous connection account for approximately 2% of congenital cardiac anomalies.1,2 Total anomalous systemic and pulmonary venous connection is an extremely rare cardiac anomaly. Only one such case of common atrium with pulmonary stenosis and anomalous systemic and pulmonary venous connection (cardiac total anomalous pulmonary venous connection) with visceral heterotaxy has been reported in the English literature.3

Case report A 16-month-old boy weighing 7 kg was admitted to our institute with a history of recurrent respiratory infections since birth. Physical examination revealed cyanosis with systemic oxygen saturation of 78% on room air. Two-dimensional and Doppler echocardiography revealed situs solitus, atrioventricular and ventriculoarterial concordance. All 4 pulmonary veins formed a common chamber that drained via the vertical vein into the left innominate vein, which in turn drained into a right-sided superior vena cava (SVC). A nonrestrictive secundum atrial septal defect (ASD) shunting blood from the right atrium to the left atrium, with severe pulmonary arterial hypertension. An elective surgical repair was planned under cardiopulmonary bypass and cardioplegic arrest. The heart was deflected

in the right pleural cavity, and the left atrium was opened. The secundum ASD was closed directly, only to be reopened when the central venous catheter was seen to be to the left atrial side of the interatrial septum, its margins were reconfirmed with the decision to close it through the right atrium. The common chamber was anastomosed to the left atrium. On opening the right atrium, a large high secundum ASD was seen along with a central venous pressure catheter in the SVC which was noted to drain below the margin of the secundum ASD (Figure 1). The ASD was closed using a pericardial patch to divert the SVC into the right atrium. The patient was weaned from cardiopulmonary bypass, and the vertical vein was ligated. Post-bypass, he was noted to have a systemic oxygen saturation of 90% and PO2 of 60 mm Hg. As blood-stained frothy secretions were noted from the endotracheal tube, the low oxygen saturation was accepted. A postoperative echocardiogram showed an adequate anastomosis between the common pulmonary venous chamber and the left atrium, with an intact interatrial septum. U.N. Mehta Institute of Cardiology and Research Center, Asarwa, Ahmedabad, India Corresponding author: Amit Mishra, MCh, FPCS, Department of Cardiovascular and Thoracic Surgery, U.N. Mehta Institute of Cardiology and Research Center, New Civil Hospital Campus, Asarwa, Ahmedabad 380016, Gujarat, India. Email: [email protected]

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Asian Cardiovascular & Thoracic Annals 23(1)

Figure 1. Operative photograph showing a central venous pressure (CVP) catheter lying below the margin of a high secundum atrial septal defect (ASD). IVC: inferior vena cava; SVC: superior vena cava; TAPVC: total anomalous pulmonary venous connection.

Contrast echocardiography via the upper limb revealed that the SVC drained into the right atrium with no residual right-to-left shunt. The pulmonary arterial hypertension was now categorized as mild. Although the patient had stable hemodynamics, extubation was deferred owing to low PO2 and desaturation. A repeat contrast echocardiogram via lower limb revealed that the IVC drained into the left atrium. A contrastenhanced computed tomography scan ruled out other associated anomalies and also reconfirmed the diagnosis (Figure 2). On reoperation, although there was no external evidence of anomalous drainage of the SVC or IVC to the left atrium, after opening the right atrium, the anomalous drainage of the IVC to the left atrium was confirmed when the ASD patch and native septum were excised. The entire defect was closed using a pericardial patch, diverting the SVC and IVC into the right atrium. Postoperative echocardiography showed the pulmonary veins draining normally to the left atrium, and the SVC and IVC to the right atrium. The further postoperative course was uneventful, and the child was soon discharged.

Figure 2. Computed tomography coronal section reconstruction of a maximum intensity projection image showing the inferior vena cava draining to the left atrium, and the ligated vertical vein. LPV: left pulmonary veins; RLL PV: right lower lobe pulmonary vein; RUL PV: right upper lobe pulmonary vein.

wise to confirm systemic and pulmonary venous drainage in such a situation. A pulmonary arteriovenous fistula, an undiagnosed coronary sinus type of ASD, accessory IVC, and iatrogenic diversion of IVC to the left atrium, are a few of the common causes of postoperative desaturation. In such situations, contrast-enhanced echocardiograms via the upper and lower limbs are pivotal, even as contrast-enhanced computed tomography and magnetic resonance imaging are the gold standard noninvasive imaging modalities. On the other hand, angiography delineates the exact nature of such a complex anomaly.4 This article reports a rare presentation of unusual drainage of the SVC and IVC in a patient initially diagnosed with a routine supracardiac total anomalous pulmonary venous connection, without visceral heterotaxy. A careful and timely diagnostic reevaluation clinched the cause of desaturation, and thus a rare variety of anomalous systemic and pulmonary venous drainage was successfully dealt with. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.

Discussion In the presence of anomalous SVC drainage, there is always a possibility of anomalous drainage of the hepatic veins, coronary sinus, and IVC. On total bypass with low IVC cannulation, it is very difficult to suspect or identify anomalous IVC drainage. In retrospect, the pulmonary edema in our patient after coming off bypass was possibly due to the sudden volume overload of the left heart, but it gradually improved. The most vital learning point for the author is that it is always

Conflict of interest statement None declared.

Acknowledgement The authors acknowledge Ms. Foram Buch and Ms. Hiral Surti, Mr. Sanjay Patel for their contribution in manuscript preparation and Mr.Piyush Solanki for preparing operative photographs.

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References 1. Bharati S and Lev M. Congenital anomalies of the pulmonary veins. Cardiovasc Clin 1973; 5: 23–41. 2. De Leval MR, Ritter DG, Mcgoon DC and Danielson GK. Anomalous systemic venous connection. Surgical considerations. Mayo Clin Proc 1975; 50: 599–610.

3. Ghosh PK, Donnelly RJ, Hamilton DI and Wilkinson JL. Surgical correction of a case of common atrium with anomalous systemic and pulmonary venous drainage. J Thorac Cardiovasc Surg 1977; 74: 604–606. 4. Catton K, Blurton D, Vorobiof G and Setty SP. Bilateral superior vena cavae with anomalous criss-cross atrial drainage. Pediatr Cardiol 2013; 34: 769–770.

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Total anomalous systemic and pulmonary venous connection.

Total anomalous systemic and pulmonary venous connection is an extremely rare congenital cardiac anomaly. We present our unique experience of managing...
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