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J Thorac Cardiovasc Surg 1997;114:848-850
© 1997 Mosby, Inc.
BRIEF COMMUNICATIONS |
Saitama, Japan
Received for publication Jan. 30, 1997 accepted for publication March 7, 1997. Address for reprints: Masaaki Yamagishi, MD, Department of Surgery II, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi, Hirokoji, Kamikyo-ku, Kyoto 602, Japan.
Anatomic correction of congenitally corrected transposition of the great arteries (TGA), the double switch operation, has been applied in children with associated cardiac anomalies and risk factors for systemic right ventricular function.
1-3 The association of total anomalous pulmonary venous return (TAPVR) with congenitally corrected TGA, which is rare in the nonisomerism heart, may increase the difficulty of constructing competent intraatrial venous channels during the double switch operation. Only one case of congenitally corrected TGA in which all pulmonary veins returned to the coronary sinus has been reported previously. 4 Here, we describe an infant with situs solitus, congenitally corrected TGA, and TAPVR who underwent a double switch operation consisting of an arterial switch and a modified atrial switch procedure. Morphologic nomenclature is used in this report.
A 6-month-old male infant weighing 7.5 kg with situs solitus, congenitally corrected TGA, atrial septal defect, ventricular septal defect, and TAPVR underwent surgical repair. The spatial position of the heart was mesocardia. The systemic right ventricular end-diastolic volume was 81% of normal, and the right ventricular ejection fraction was decreased to 54%. The pulmonic left ventricular end-diastolic volume was 119% of normal, and the left ventricular ejection fraction was 72%. A preoperative angiogram revealed that the confluence of the left pulmonary veins traversed the midline and connected with the right inferior pulmonary vein. Each branch of the right superior pulmonary vein drained directly into the vertical vein. The vertical vein ascended in the right pleural cavity and drained via a short stenotic segment into the superior vena cava.
Through a median sternotomy, the ascending aorta and the right and left pulmonary arteries were dissected free. After hypothermic (27° C) cardiopulmonary bypass had been established with aortic and bicaval cannulation, the vertical vein was also mobilized. The right atrium was opened longitudinally 15 mm anterior to the crista terminalis to preserve more posterior right atrial wall (Fig. 1, 1). After the aorta was crossclamped, the ventricular septal defect was closed by de Leval's maneuver through the mitral valve. 4 The atrial septum inferior to the atrial septal defect was resected, and the roof of the coronary sinus was cut down. The posterior right atrial wall was fashioned by making a small incision (Fig. 1, 2). The posterior right atrial wall suture was placed along the cut edge of the atrial septum anteriorly, running around the superior edge of the atrial septal defect to provide the systemic venous pathway from the inferior vena cava to the left (functionally right) atrium. The coronary sinus was left to drain within the systemic venous channel (Fig. 1, 3). The superior vena cava was transected just proximal to the junction with the azygos vein, which was ligated and transected. The vertical vein was transected at its junction with the superior vena cava, and the stenotic segment was resected. The orifice of the vertical vein was enlarged by an incision of a few millimeters. The proximal stump of the superior vena cava was also enlarged by incising toward the right atrium (Fig. 1, 2). Special care was taken to avoid damage to the sinus node and the sinus nodal artery. A pulmonary venous channel was created by an anastomosis between the vertical vein and the proximal stump of the superior vena cava (Fig. 1, 3). An incision was made on the roof of the left atrium, and the cephalic end of the superior vena cava was anastomosed to this opening (Fig. 1, 3 and 4). The arterial switch procedure was performed according to a standard technique. 1 No difficulty was encountered in transferring the coronary arteries with a mirror-image branching pattern relative to normal coronary arteries. 1,2 A postoperative two-dimensional echocardiogram demonstrated competent systemic and pulmonary venous channels without stenosis and sufficient systemic left ventricular performance. The systemic left ventricular ejection fraction was 68%. The electrocardiogram revealed normal sinus rhythm without supraventricular arrhythmias.
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1-3; secondary heart failure resulting from tricuspid regurgitation or systemic right ventricular dysfunction is inevitable in patients who have undergone the conventional repair, in which the morphologically right ventricle supports the systemic circulation. However, a small volume of the right atrium in congenitally corrected TGA lying behind the overhanging left ventricle may be a potential drawback of the double switch operation. 1,3 The small atria become more of a conduit than a reservoir after an atrial switch procedure and appear to play a major role in the subsequent development of suboptimal cardiac function. In this infant, the left atrial volume was also smaller than that of the common type of congenitally corrected TGA because of the association with TAPVR. The confluence of the left pulmonary veins traversed the midline relatively low, not in close apposition with the atria, and the vertical vein ascended in the right pleural cavity at a distance from the atria. Therefore, usual techniques of TAPVR repair were not suitable for creating a large tension-free anastomosis. When the double switch operation was performed, a substitute atrial switch technique for the Senning or the Mustard procedure was required to construct efficient venous channels in the small atria. Our modified atrial switch technique allowed both atria to be able to function effectively as reservoirs. The pulmonary venous blood and the systemic venous blood from the superior vena cava were directly drained into each functional atrium. The inferior vena caval pathway was able to be kept to a minimum volume in the right (functionally left) atrium by cutting back the roof of the coronary sinus and roofing over with an autologous right atrial wall. This simplified atrial switch technique with minimized incision and suture of the atria can obviate the possible dangers of caval obstruction 3 and supraventricular arrhythmias. Although the intraatrial flap suture ran across the triangle of Koch and the coronary sinus was positioned on the systemic venous side of the flap, no atrioventricular conduction disturbances occurred. Because the atrioventricular node and conduction bundle in patients with congenitally corrected TGA with situs solitus are located anteriorly, away from the coronary sinus. Care should be taken in transferring the coronary arteries, because congenitally corrected TGA may have a short left main trunk, as well as various kinds of branching patterns of coronary arteries. 5 In conclusion, concomitant anomalies of systemic and pulmonary venous return are not always a contraindication to a double switch operation. The double switch operation should be applied to patients with congenitally corrected TGA when each of the ventricles has an adequate volume and both atrioventricular valves are functionally competent.
References
This article has been cited by other articles:
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  G. W. Raff, D. M. Geiss, J. J. Shah, L. M. Bond, and J. A. Carroll Repair of transposition of the great arteries with total anomalous pulmonary venous return Ann. Thorac. Surg., February 1, 2002; 73(2): 655 - 657. [Abstract] [Full Text] [PDF]
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