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© 2004 European Association of Cardio-Thoracic Surgery
Extracardiac reconstruction of anomalous superior systemic venous return
a Department of Pediatric Cardiovascular Surgery, The Chaim Sheba Medical Center, Tel Hashomer, The Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
* Corresponding author. Address: 804 Ames Av, Palo Alto, CA 94303, USA. Tel.: +1-650-8529-694 Received October 1, 2003; received in revised form January 7, 2004; accepted January 12, 2004
A heretofore undescribed surgical approach for correcting a rare congenital cardiac malformation consisting of an absent right superior vena cava with persistent left superior vena cava associated with situs solitus draining into the left atrium is presented. The intra-atrial septum was intact. Normal anatomy was achieved and recovery was uneventful.
Key Words: Absent right superior vena cava; Persistent left superior vena cava; Situs solitus; Intact atrial septum; Surgical correction Left superior vena cava (LSVC) is a relatively common cardiac malformation, occurring in 0.3% of the general population and in 3–34% of patients with all congenital cardiac defects. In contrast, an absent right superior vena cava (RSVC) in combination with an LSVC is a much more rare cardiac malformation and one that is usually associated with additional heart defects and arrhythmias [1]. We present a heretofore undescribed surgical correction of an absent RSVC and an LSVC associated with situs solitus that was draining into the left atrium. The intra-atrial septum was intact.
An 18-month-old girl presented with profound cyanosis (O2 room air saturation of 79%) and digital clubbing. Echocardiography and cardiac catheterization demonstrated small right heart chambers and enlarged left heart chambers, an absent RSVC, an LSVC draining directly into the left atrium, a normal inferior vena cava (IVC) and pulmonary venous drainage and normal coronary sinus. There were no other intra-cardiac anomalies, the intra-atrial septum was intact and the pulmonary and abdominal situs were normal. Surgical correction was performed through a median sternotomy, and intra-operative inspection confirmed the preoperative diagnosis (Fig. 1a). The ascending aorta and the LSVC and IVC were cannulated and snared. The patient was connected to cardiopulmonary bypass and most of the operation was performed on a beating heart. The LSVC was transected from the left atrium proximally and the left side of the innominate vein distally and translocated as a free graft to form the neo-right SVC by anastomosing its proximal end to the right atrium and its distal end to the right side of the innominate vein (Fig. 1b). Approximately 2 min of ventricular fibrillation were applied in order to create a small atrial septal defect that could function as a decompression vent for the mildly hypoplastic right heart chambers. The child was easily weaned from cardiopulmonary bypass, no gradients were measured across the neo-right SVC, and a small right-to-left shunt was visualized through the atrioseptal defect (ASD) yielding perioperative room air saturations of 92%.
The postoperative course was uneventful and the patient was discharged home on the fifth day following surgery. At the 1-month follow-up visit, the patient was well with an O2 room air saturation of 95%; echocardiographic evaluation demonstrated an increase in right-sided chamber volume with good ventricular function, no hepatic venous congestion, and disappearance of the right-to-left atrial shunt.
Absent RSVC with persistence of LSVC associated with situs solitus is a rare cardiac anomaly that occurs in approximately 0.1% of patients with congenital heart disease and is usually accompanied by additional cardiovascular malformations (46% of patients) or rhythm disturbances (36% of patients) [1]. In most cases, the left SVC drains into the coronary sinus and the right atrium causing coronary sinus enlargement but no right-to-left shunt, while in some cases a coronary sinus ASD is present with bidirectional shunting. Rarely, the LSVC can drain directly into the left atrium without an ASD, causing significant right-to-left shunting, cyanosis, polycythemia and even brain abscesses [2–4]. Surgical correction of this anomaly has been previously performed by creating and intra-atrial baffle directing the venous blood through the left atrium into the right atrium through a newly formed ASD [2,3], by creating a baffle for directing the arterial blood from the pulmonary veins to the mitral valve [2] or by disconnecting the left SVC and attaching it end-to-side to the right atrial appendage [5,6]. The creation of an intra-atrial baffle, however, can over time cause stenosis [3], intra-atrial shunts [2,3] and even thrombosis and obstruction [7]. Moreover, disconnecting the proximal LSVC and re-anastomosing it to the right atrial appendage can cause stretching or compression of the newly formed conduit since it courses between the great arteries and the sternum, and the filling pressures are low. We reasoned that the translocation and re-implantation of the LSVC into the ‘anatomically’ right-sided position would avoid the baffle-associated complications and conduit compression, thereby creating a normally appearing anatomy. Our patient had a mildly hypoplastic right atrium and ventricle due to preoperative limited venous drainage to the right atrium. In order to facilitate immediate postoperative recovery, we created a small ASD to serve as a decompression vent in the event of diastolic dysfunction of the right ventricle. Our patient was mildly cyanotic perioperatively but she had an uneventful and rapid recovery, and the right-to-left shunt disappeared after 1 month, once right ventricular chamber size and compliance had improved.
ICVTS on-line discussion Author: Dr. Antonio Corno, CHUV, Department of Cardiovascular Surgery, 46 rue du Bugnon, Lausanne, CH-1011 Switzerland Date: 23-Feb-2003 Message: The Authors are to be congratulated for their astute solution of translocation and re-implantation of the persistent left superior vena cava in the position of the absent right superior vena cava. Also the idea of creating a small interatrial communication, to decompress the hypoplastic right ventricle in the immediate post-operative period has been a winning one. Probably other readers involved in the surgical treatment of children with congenital heart defect will be interested to have more details of the surgical technique, in particular, to know what kind of sutures were utilized to reimplant the left superior vena cava. Since there are two circular anastomosis in a child of 18 months of age, it would be interesting to know what kind of precautions the Authors used to reduce the risk of later thromboembolism. Did they use interrupted sutures? Absorbable sutures? Did they treat the patient with prophylactic antiplatelets medications? How long? Another useful piece of information is to know where they performed the incision on the right auricular appendage to avoid the sinus node; and of course if the patient remained in sinus rhythm. This information must be compared with the potential risks of alternative surgical techniques, like the intra-atrial baffling of the orifice of the persistent left superior vena cava into the right atrium through a created inter-atrial communication. Once again, congratulations for the clever solution and the clear case report. Response Author: Dr. Gabriel Amir, Sheba Medical Center, Tel Hashomer, Department of Pediatric Heart Surgery, Ramat Gan, 804 Ames Avenue, Palo Alto, CA 94303, USA Date: 01-Apr-2004 Message: Thank you for your comment. The proximal anastomosis was created by performing a wide incision in the right atrial appendage, thus sparing the SA node and its blood supply. Absorbable continuous sutures were used. The patient remained in sinus rhythm through the operation, hospital stay, and was in sinus rhythm at follow up. We did not use anti platelet medication.
We thank Esther Eshkol for editorial assistance. doi:10.1016/j.icvts.2004.01.011
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Abstract
1. Case presentation