Ann Thorac Surg 2009;87:1599-1601. doi:10.1016/j.athoracsur.2008.09.025
© 2009 The Society of Thoracic Surgeons
Case Reports
Total Anomalous Systemic Venous Drainage to the Left Atrium
Ze-Wei Zhang, MD*,
Qun-Jun Duan, MD,
Zhan Gao, MD,
Wei Ru, MD,
Li-Yang Ying, MD
Department of Thoracic and Cardiovascular Surgery, Children's Hospital of Zhejiang University, School of Medicine, Hangzhou, China
Accepted for publication September 1, 2008.
* Address correspondence to Dr Zhang, Department of Thoracic and Cardiovascular Surgery, Children's Hospital of Zhejiang University, School of Medicine, No. 57 Zhugan Xiang, Hangzhou, 310003, China (Email: zeweiz{at}zju.edu.cn).
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Abstract
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We present an unusual case of total anomalous systemic venous drainage in which the right superior vena cava, persistent left superior vena cava, inferior vena cava, and coronary sinus were present and unusually connected to the left atrium. Successful surgical correction was achieved, and the patient's recovery was uneventful. Various types of total anomalous systemic venous drainage are discussed, and classification of total anomalous systemic venous drainage is made under the consideration of creation of cardiopulmonary bypass.
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Introduction
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Total anomalous systemic venous drainage is an exceptional form of congenital heart disease. All systemic venous flow, including the right superior vena cava (RSVC), persistent left superior vena cava (LSVC), inferior vena cava (IVC), and coronary sinus, drains abnormally into the heart. This disorder is usually associated with atrioventricular canal, common atrium, atrial septal defect (ASD), ventricular septal defect (VSD), and heterotaxia. Surgical correction is often difficult and complicated. Here, we describe a patient with unique total anomalous systemic venous drainage that was successfully corrected surgically.
A 33-month-old girl was admitted to our hospital for evaluation of mild cyanosis with a room air oxygen saturation of 86%. There was no evidence of dyspnea or heart failure. Her height and weight both ranked in the 90th percentile. The physical examination found a grade III/VI systolic murmur in her left sternal border. Echocardiography revealed that the RSVC, persistent LSVC, and IVC drained abnormally into the left atrium (Fig 1A). ASD and VSD were also evident. There was no evidence of heterotaxy syndrome. Although her parents prohibited further cardiac catheterization examination, surgical intervention was performed after cautious and detailed communication with her parents.
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Fig 1. (A) Inferior vena cava (IVC) and right superior vena cava (RSVC) were seen connected to the left atrium (LA) on the subcostal biatrial view in the echocardiography. (B) The attachment of the reconstructed atrial septum is shown by the deep dotted line. The intracardiac channel is displayed by the gray dotted and diagonal lines. (ASD = atrial septal defect; CS = coronary sinus; HV = hepatic vein; LSVC = left superior vena cava; MV = mitral valves; PV = pulmonary veins; RA = right atrium; TV = tricuspid valves.)
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We did not have an appropriately sized cannula for the direct cannulation of the RSVC or LSVC at the time of the operation. Fortunately, the opening of the RSVC was near the ASD in the left atrium, as evidenced on echocardiography. We therefore placed the RSVC cannula through the ASD and guided it into the RSVC instead of using direct cannulation. The IVC was directly cannulated and full bypass was achieved.
After right atriotomy and excision of the atrial septum, anomalous drainage of the great vessels was observed and was in accordance with echocardiographic outcome. The LSVC orifice was adjacent to the coronary sinus instead of draining into the coronary sinus, suggesting an unroofed LSVC. The coronary sinus was also connected to the left atrium. An intracardiac extractor was introduced into the LSVC to manage blood flow from the LSVC. Flow from the coronary sinus was extracted incidentally by another intracardiac extractor.
The LSVC could not be ligated due to high blood pressure. Subsequently, an intracardiac channel was formed by a piece of longitudinal pericardial patch so that the LSVC and coronary sinus communicated with the right atrium (Fig 1B). A repositioned atrial septum, using an autologous pericardium, was reconstructed such that all pulmonary veins drained through the mitral valves to the left ventricle and all systemic veins and the coronary sinus drained through the tricuspid valves to the right ventricle (Fig 1B).
The patient made an uneventful recovery. At the 1-year follow-up, she was healthy on physical examination, and a normal result was seen on the echocardiographic assessment.
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Comment
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Although one exceptional case was reported where all systemic venous blood returned to the coronary sinus [1], the openings of total anomalous systemic veins are much more commonly connected to the left atrium [2–8]. Upon reviewing the literature, we have found only seven reports of incidences of total anomalous systemic venous drainage to the left atrium that were successfully corrected surgically [2–8]. None of these patients presented with the RSVC, persistent LSVC, and IVC all affected. Our patient presented with total anomalous systemic venous drainage in which all three vena cavae and coronary sinus were present and anomalously entered the left atrium.
During surgical intervention, the real difficulty lies in the creation of cardiopulmonary bypass. If the IVC is not interrupted, both the IVC and SVC can be directly cannulated and conventional cardiopulmonary bypass is achieved. We did not cannulate the RSVC directly owing to lack of a cannula of appropriate size. If the IVC is interrupted, azygos or hemiazygos continuation of the IVC to a SVC that entered the left atrium is usually found [2–5]. This SVC could be cannulated solely to drain all the systemic venous flow. However, two hepatic veins are also usually seen connected to the left atrium directly in these patients. Venous blood flow from these hepatic veins would obstruct the surgical view. Therefore, these hepatic veins could be cannulated with a small cannula after excision of the atrial septum, or the use of an intracardiac extractor would help with the blood flow.
Thus, we tend to classify total anomalous systemic venous drainage into two types, according to the type of vena cava cannulation. In type I, the IVC is not interrupted and conventional cardiopulmonary bypass can be created. In type II, the IVC is interrupted, and single cannulation of the SVC and conventional cardiopulmonary bypass can be achieved. In this case, hepatic veins can be cannulated with a small cannula after excision of the atrial septum or an intracardiac extractor is used to manage the blood flow. Our patient is classified as type I category (Table 1).
We considered the LSVC–RSVC and LSVC–right atrial appendage anastomosis before the intervention. After intracardiac inspection of the LSVC and left atrium, however, we found that the LSVC and left atrium shared a long part of the wall between them. Therefore, in the situation of transecting the LSVC at the connection with the left atrium, the transected LSVC would be too short to reach the RSVC or the right atrial appendage. Neither of these two approaches was thus feasible in this specific case. We decided that intracardiac baffling was the most feasible method, and it was successfully accomplished.
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Acknowledgments
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This study was supported by a grant from the Science and Technology Department of Zhejiang Province (No. 2004C23016).
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References
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- Kadletz M, Black, MD, Smallhorn J, Freedom RM, Van Praagh S. Total anomalous systemic venous drainage to the coronary sinus in association with hypoplastic left heart disease: more than a mere coincidence J Thorac Cardiovasc Surg 1997;114:282-284.[Free Full Text]
- Krayenbuhl CU, Lincoln JC. Total anomalous systemic venous connection, common atrium, and partial atrioventricular canal. A case report of successful surgical correction. J Thorac Cardiovasc Surg 1977;73:686-689.[Medline]
- Danielson GK, McMullan MH, Kinsley RH, DuShane JW. Successful repair of complete atrioventricular canal associated with dextroversion, common atrium, and total anomalous systemic venous return J Thorac Cardiovasc Surg 1973;66:817-822.[Medline]
- Johnson TR, Schamberger MS, Brown JW, Girod DA. Resolution of acquired pulmonary arteriovenous malformations in a patient with total anomalous systemic venous return Pediatr Cardiol 2002;23:210-212.[Medline]
- Roberts KD, Edwards JM, Astley R. Surgical correction of total anomalous systemic venous drainage J Thorac Cardiovasc Surg 1972;64:803-810.[Medline]
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- Viart P, Le Clerc JL, Primo G, Polis O. Total anomalous systemic venous drainage Am J Dis Child 1977;131:195-198.[Abstract/Free Full Text]
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Abstract
Introduction
