Ann Thorac Surg 1998;65:250
© 1998 The Society of Thoracic Surgeons
Case Reports
Two-Stage Correction of Transposition of Great Arteries With Complete Atrioventricular Canal
Kisaburo Sakamoto, MD,
Lorenzo Galletti, MD,
Anita Touchot, MD,
Jean Dominique Piot, MD,
Jacqueline Bruniaux, MD,
Claude Planche, MD
Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, Le Plessis-Robinson, France
Accepted for publication September 2, 1997.
Dr Planche, Service de Chirurgie des Cardiopathies Congenitales, Hopital Marie Lannelongue, Universitaire Paris-Sud, 133 Ave de la Resistance, 92350 Le Plessis-Robinson, France.
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Abstract
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We report a successful two-stage anatomic correction of D-tra nsposition of the great arteries associated with complete atrioventricular canal without pulmonary obstruction, combining an arterial switch procedure and a two-patch repair for complete atrioventricular canal, after pulmonary artery banding in the neonatal period.
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Introduction
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Transposition of the great arteries (TGA) associated with complete atrioventricular canal (CAVC) is a rare congenital cardiac anomaly that usually coexists with pulmonary stenosis or atresia [1][2][3][4][5][6]. Therefore, TGA with CAVC without pulmonary obstruction appears to be exceptional [7][8]. We report our experience of a two-stage anatomic repair for this anomaly.
A female neonate (age, 19 days; weight, 2 kg) was referred to Marie-Lannelongue Hospital because of severe congestive heart failure. Echocardiography revealed D-TGA, CAVC, and patent ductus arteriosus with severe regurgitation through the common atrioventricular valve. At the age of 21 days, she underwent patent ductus arteriosus ligation and pulmonary artery banding to control heart failure and pulmonary hypertension. She was discharged on the 12th postoperative day in good hemodynamic condition with a pulse oximeter saturation of 82%. The echocardiographic pressure gradient through the band was 56 mm Hg and the atrioventricular valve regurgitation was trivial. Progressive cyanosis developed, and at 14 months of age she underwent a complete cardiac evaluation including detailed echocardiography and cardiac catheterization. Echocardiography confirmed the diagnosis of situs solitus, atrioventricular concordance, and CAVC with two balanced ventricles and D-TGA (Fig 1).
At catheterization systolic blood pressure was 90 mm Hg in both ventricles and in the aorta; the distal mean pulmonary artery pressure was 15 mm Hg. Atrioventricular valve regurgitation was moderate on the angiography, but normal pressure was recorded in both atria.
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Great arteries are transposed and common atrioventricular valve is opening. Arrows represent an ostium of the atrioventricular valve. (Ao = aorta; LV = left ventricle; PA = pulmonary artery; RV = right ventricle.)
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When the child was 17 months of age, anatomic repair of the malformation was performed, combining an arterial switch operation with a two-patch repair of the CAVC. Hypothermic (22°C) continuous cardiopulmonary bypass was employed, and myocardial protection was ensured by intermittent anterograde crystalloid cardioplegia administration. At the inspection of the heart the aorta was anterior and slightly to the right of the pulmonary artery, and there was a single right coronary artery originating from the right facing sinus (type 3 of the Marie-Lannelongue Hospital classification [9]). A double-orifice mitral valve was discovered intraoperatively, and the mitral cleft was left open to avoid mitral stenosis (Fig 2).
The ventricular component of CAVC was closed by a Dacron patch, whereas fresh autologous pericardium was employed for closure of the ostium primum defect. The coronary sinus drainage was directed to the right atrium. Both great vessels were transected, a Lecompte maneuver was performed, and the right coronary artery button was detached from the aortic wall. At that time a small, isolated right infundibular branch originating from the left-facing sinus was discovered and ligated. The right coronary artery was relocated using a trap-door incision on the neo-aortic wall. A piece of fresh autologous pericardium was employed to repair the defect in the neo-pulmonary root. Cardiopulmonary bypass and aortic cross-clamp time were respectively 165 and 119 minutes. A low-dose dopamine infusion was employed to wean the patient from the cardiopulmonary bypass. The chest was left open because of heart size and closed on the second postoperative day. The hospital stay was uneventful, and the child was discharged on the 14th postoperative day.
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The left atrioventricular valve was a little smaller than normal and had double orifices. (A = aortic valve; LAD = left anterior descending artery; LCX = left circumflex artery; P = pulmonary valve; RCA = right coronary artery.)
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Comment
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Transposition of the great arteries and CAVC is a rare association, almost always associated with pulmonary stenosis. In a previously reported anatomic review of TGA/CAVC, all 17 cases had restricted pulmonary blood flow [2]. Successful repair of TGA/CAVC with pulmonary stenosis or atresia (anatomic left ventricular outflow tract obstruction) with a Rastelli-type operation [3], Fontan procedure [4], and Senning or Mustard procedure [5][6] has been reported. One case of surgical repair of TGA/CAVC without pulmonary obstruction was reported by Kumar and associates [8]. They described anatomic repair in a 9-year-old child with TGA and an intermediate form of CAVC with two separate atrioventricular valve orifices. In their patient, despite the presence of a large ventricular component, pulmonary vascular obstructive disease did not develop; at the same time, elevated pulmonary artery and left ventricular pressure maintained left ventricular competence, avoiding involution of its mass.
Our patient was referred to the hospital at the age of 19 days in a very poor condition. Although neonatal arterial switch operation is a very well established strategy for repair of simple and complex forms of TGA, and the results of neonatal repair of CAVC have improved, the operative risk for a primary repair in a 2-kg sick newborn was considered too high. A palliative procedure was perferred, with the aim of controlling cardiac failure and allowing the patient to grow, postponing definitive repair to between 12 and 18 months of age. The pulmonary artery band should be placed to avoid coronary artery compression and late development of aortic regurgitation after the arterial switch operation. Postoperative cardiac failure was moderate after complete repair, and the chest was left open for 36 hours. Nevertheless, recovery was substantially fast and the child was discharged on the 14th postoperative day. Up to now, 4 months after repair, the child’s clinical condition is very good.
In conclusion, we think that in the presence of TGA/CAVC with balanced ventricles and unrestricted pulmonary blood flow, anatomic correction combining an arterial switch operation with CAVC repair is the best strategy, especially in terms of late functional result. It avoids the long-term consequences of "systemic" right ventricle and Fontan operation. Primary or two-stage repair should be decided on only after a full appreciation of anatomy and pathophysiology.
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References
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- Shaher RM Complete transposition of great vessels. New York: Academic Press, 1973:162.
- Bharati S, Kirklin JW, McAllister HA, Lev M Surgical anatomy of common atrioventricular orifice associated with tetralogy of Fallot, double outlet right ventricle and complete regular transposition. Circulation 1980;61:1142-1149.[Free Full Text]
- McGoon DC, Wallace RB, Danielson GK The Rastelli operation. Its indication and results. J Thorac Cardiovasc Surg 1973;65:65-75.[Medline]
- Gonzalez-Lavin L, Blair TC, Chi S, Sparrow AW Orthoterminal correction of coexisting d-transposition of great arteries, subpulmonary stenosis and a complete form of atrioventricular canal. J Thorac Cardiovasc Surg 1977;73:694-698.[Abstract]
- Alfieri O, Plokker M Repair of common atrioventricular canal associated with transposition of the great arteries and left ventricular outflow obstruction. J Thorac Cardiovasc Surg 1982;84:872-875.[Abstract]
- Pacifico AD, Ricchi A, Bargeron LM, Jr, Colvin EC, Kirklin JW, Kirklin JK Corrective repair of complete atrioventricular canal defects and major associated cardiac anomalies. Ann Thorac Surg 1988;46:645-651.[Abstract/Free Full Text]
- Benson IB Complete transposition of the great vessels. Importance of septal defects and patent ductus arteriosus. Circulation 1963;28:885-887.[Abstract/Free Full Text]
- Kumar N, Prabhaker G, Wilson N, Al Halees Z, Duran CMG Total correction of transposition of great arteries with atrioventricular septal defect. Ann Thorac Surg 1992;54:989-990.[Abstract/Free Full Text]
- Serraf A, Lacour-Gayet F, Bruniaux J, et al. Anatomic correction of transposition of the great arteries in neonates. J Am Coll Cardiol 1993;22:193-200.[Abstract]
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Abstract
Introduction
