Asian Cardiovasc Thorac Ann 2007;15:e1-e2
© 2007 Asia Publishing EXchange Ltd
Primary Repair of d-Transposition With Complete Atrioventricular Canal Defect
Akshaykumar Bisoi, MCh,
Smartin Abraham, MCh,
C Sai Krishna, MS,
Sandeep Chauhan, MD1,
Shyamsunder Kothari, DM2,
Panangipalli Venugopal, MCh
Department of Cardiothoracic and Vascular Surgery
1 Department of Cardiac Anaesthesia
2 Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
For reprint information contact: Smartin Abraham, MCh Tel: 91 11 2658 9026 Fax: 91 11 2658 8663 Email: smartin{at}rediffmail.com, All India Institute of Medical Sciences, New Delhi 110029, India.
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ABSTRACT
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A 4-month-old boy underwent successful single-stage anatomic repair of d-transposition of the great arteries with complete balanced atrioventricular canal defect, using the arterial switch procedure and two-patch repair of the atrioventricular canal defect. He had associated persistent left superior vena cava draining to the coronary sinus, and a patent ductus arteriosus.
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INTRODUCTION
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Complete atrioventricular canal (CAVC) associated with transposition of the great arteries (TGA) is rare.1,2 These anomalies usually co-exist with pulmonary atresia or pulmonary stenosis (valvar or subvalvular).2 When unrestricted pulmonary blood flow is present, the best option for treating this complex anomaly is complete repair of both defects by the arterial switch procedure and repair of the CAVC defect. This can be done in 2 stages or a single stage, depending on the time of presentation, status of the patient, and the anatomy of the defect.3–5 The incidence of left superior vena cava (SVC) associated with TGA and CAVC is even more rare.2
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CASE REPORT
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A 4-month-old boy weighing 5 kg presented with a history of recurrent lower respiratory tract infection as the only outstanding symptom. Physical examination revealed an otherwise normal infant except for mild tachypnea (respiratory rate 65 breaths·min–1). A loud second heart sound and a grade 4/6 systolic murmur along the left parasternal border were found on auscultation. The liver was palpably enlarged 3.5 cm below the right costal margin in the mid clavicular line. Chest radiography indicated cardiomegaly, pulmonary plethora, dilated right atrium, and widened superior mediastinum.
An electrocardiogram showed left axis deviation and biventricular hypertrophy. Echocardiography demonstrated atrioventricular concordance and ventriculoarterial discordance. The aorta was slightly anterior and to the right of the pulmonary artery. There was a CAVC defect with mild atrioventricular valve regurgitation (primum atrial septal defect, 6 mm; left-to-right shunt), and a moderate-sized inlet ventricular septal defect. The pulmonary valve showed mild thickening and doming, with a gradient of 20 mm Hg (flow related) across the valve, and there was mild pulmonary regurgitation. Both ventricles were normal in size and function. The coronary sinus was dilated, and a 2.5 mm patent ductus arteriosus (flow, left to right; peak gradient 25 mm Hg) was seen. The arch and arch vessels were normal and there was no co-arctation. The child was not catheterized. Single-stage correction of the defects was decided as the best option.
At surgery, bilateral superior venae cavae were observed, with a bridging vein in between. Hence, the left SVC could be safely occluded before a right atriotomy, without affecting venous drainage of the upper extremity. The patent ductus arteriosus was transfixed and divided. The CAVC defect was repaired using a standard two-patch technique (Dacron patch closure of the ventricular septal defect and pericardial patch closure of the atrial septal defect), directing the coronary sinus and connected left SVC to the right atrium. The cleft in the posterior bridging leaflet was repaired with a single stitch to achieve competency of the future mitral valve. This was followed by an arterial switch operation. The coronary anatomy was 1L 2CxR. The pulmonary valve (future aortic valve) was tricuspid but mildly thickened. The aortic cross clamp time was 105 min and the cardiopulmonary bypass time was 185 min.
Postoperative echocardiography revealed moderate aortic regurgitation, low to moderate mitral regurgitation, and mild tricuspid regurgitation. Left ventricular function was mildly impaired (ejection fraction, 40%), however the right ventricular size, function, and outflow tract were normal. The child remained hemodynamically stable and was extubated on the third postoperative day but had to be re-intubated due to respiratory failure. He was tracheostomized and later weaned off the ventilator without further problems.
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DISCUSSION
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Surgical correction of this anomaly in hearts with pulmonary obstruction is undertaken using the Rastelli procedure or the Fontan procedure or its modifications.6,7 When associated with unobstructed pulmonary blood flow, the repair can be accomplished in 1 or 2 stages. Pulmonary artery banding as the initial procedure, followed by complete anatomical repair is one option.3 Total correction of both these defects in a single stage is another option.4,5 This can be performed safely in infancy.5 Physiological correction of this complex anomaly using atrial redirection and CAVC repair is another option available.8
In this infant, the large ventricular component of the CAVC had prevented regression of the left ventricular mass, which aided in the subsequent successful correction of the anomaly. In spite of the presence of a left SVC and a dilated coronary sinus, pericardial patch closure of the primum atrial septal defect could be carried out safely, directing the left SVC to the right atrium without causing heart block. A successful single-stage 2-pump anatomic repair is possible for TGA with CAVC defect in infancy.
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REFERENCES
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ABSTRACT
INTRODUCTION
