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Ann Thorac Surg 2002;73:1317-1320
© 2002 The Society of Thoracic Surgeons
a Division of Cardiovascular Surgery The Montréal Children’s Hospital, McGill University Health Center, Montréal, Canada
b Division of Pediatric Cardiology The Montréal Children’s Hospital, McGill University Health Center, Montréal, Canada
Accepted for publication July 30, 2001.
* Address reprint requests to Dr Tchervenkov, Division of Cardiovascular Surgery, Rm C-829, The Montreal Children’s Hospital, McGill University Health Center, 2300 Tupper St, Montreal, PQ H3H-1P3, Canada
e-mail: christo.tchervenkov{at}muhc.mcgill.ca
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Abstract |
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Introduction |
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A 17-month-old girl was referred with mild cyanosis and the diagnosis of CAVC, DORV, PS, cor triatriatum, and left SVC to unroofed coronary sinus. At cardiac catheterization, the pulmonary artery pressures were 25/15 mm Hg with a mean of 20, and the mean left atrial pressure below the cor triatriatum was 7. The systemic oxygen saturation was 78% and the ratio of pulmonary blood flow to systemic blood flow (QP/QS) was 1. The degree of pulmonary outflow obstruction masked the obstructive nature of her cor triatriatum, allowing her to remain relatively asymptomatic until such a late age. She was taken to the operating room for correction of this complex lesion.
The operation was performed with cardiopulmonary bypass (CPB) and hypothermia to 20.5°C. After exposure of the heart and great vessels through a median sternotomy, the aorta was cannulated, as were both SVCs and inferior vena cava. The aorta was cross-clamped and the heart was arrested with cold crystalloid cardioplegia. The intracardiac anatomy was then examined. We identified a DORV with both aorta and pulmonary artery (PA) arising 100% from the right ventricle (RV) coexisting with CAVC (Fig 1A). The VSD component of the CAVC was confined only to the inlet portion of the interventricular septum without any extension towards the great vessels. The complete origin of the aorta from the right ventricle (COARV) resulted in a significant distance between the inlet VSD and the aorta, precluding the use of simple comma-shaped patch for the intraventricular repair. Valvar PS with a hypoplastic pulmonary valve annulus was present. There was also a cor triatriatum membrane with a 4- to 5-mm opening between an upper and lower chamber in the left atrium. The upper chamber received four pulmonary veins and the lower chamber received the left atrial appendage and left SVC with no coronary sinus (Fig 1B). We proceeded to repair the cor triatriatum by resecting the left atrial membrane, rendering the left atrium a single chamber. The coronary sinus was then reconstructed with left atrial wall tissue sutured around a 6-mm Hagar dilator to drain the left SVC into the upper portion of the right atrium. The CAVC was repaired by closing the ventricular component with a patch of autologous pericardium and implanting the right and left components of the atrioventricular (AV) valves into their respective sides of the patch. A small 4-mm opening at the most superior portion of the VSD was left open to guide the creation of a new VSD. Then, via a vertical right ventriculotomy incision, the new subaortic VSD was created leftwards and superiorly by resecting a portion of the interventricular septum (Fig 2A). This effectively translocated the VSD from an inlet to a subaortic position of the interventricular septum. This allowed the creation of a straight tunnel from the left ventricle to the aorta by sewing a Gore-Tex patch (W. L. Gore and Associates, Flagstaff, AZ) around the edges of the newly created VSD with pledgeted sutures (Fig 2B). Because of the absence of the conal septum, the patch also encompassed the hypoplastic pulmonary valve annulus. The main PA was transected and proximally oversewn, and RV to PA continuity was reestablished with a 13-mm valved pulmonary homograft. The proximal end was augmented with a patch of autologous pericardium (Fig 2C). The atrial septal defect was then repaired through the right atrium using autologous pericardium, allowing the pulmonary veins to drain into the left atrium and the reconstructed coronary sinus and systemic veins to drain into the right atrium. The patient was rewarmed and weaned from CPB in normal sinus rhythm. The aortic cross-clamp time was 159 minutes and the CPB time was 241 minutes.
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The patient remained asymptomatic for 9 years but recently, on echocardiogram, she was found to have an RV to PA pressure gradient of 94 mm Hg. There was only trivial mitral regurgitation and well-preserved biventricular function with mild RV dilatation. Cardiac catheterization revealed a RV pressure of 76/10 mm Hg (66% systemic) and the RV to PA pressure gradient was 42 mm Hg. There was no pressure gradient across the left ventricular outflow tract (LVOT) that had been reconstructed 9 years earlier using the VSD translocation technique. Left ventriculograms are shown demonstrating the unobstructed LVOT (Fig 3).
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Despite reports of these combined lesions in pathological specimens, reports of successful surgical repair are much less frequent [5–10]. When the VSD component of the CAVC extends cephalad to be committed to the aorta, it can be closed with a comma-shaped patch to direct blood from the left ventricle to the aorta [5–7], accompanied by CAVC repair. Similarly, if it extends to the subpulmonic area, a tunnel can be constructed from the left ventricle (LV) to the pulmonary artery and an arterial switch can be performed [8]. However, in the presence of COARV and when the VSD does not have subaortic extension, anatomical repair becomes more challenging. Pacifico and associates [5], describe 2 patients in which the VSD did not extend cephalad into the subaortic area. Repair in these patients consisted of VSD closure, atrioventricular valve reconstruction, closure of the main pulmonary artery, left ventricle to pulmonary artery conduit, and atrial switch. A modified Fontan procedure has been described by Russo and associates as a viable alternative for this difficult group of patients [10].
We have used a technique of translocation of the VSD to a subaortic position to achieve an anatomical two-ventricle repair. An intraventricular tunnel was then created between the left ventricle and the aorta using the newly created VSD.
The association of cor triatriatum with DORV has been reported in a patient who underwent palliative repair [11] but, to our knowledge, the complete repair of DORV and CAVC associated with cor triatriatum has not been described. This component of the defect was successfully dealt with at the time of initial operation, as was reconstruction of the coronary sinus.
In summary, we present the rare case of CAVC, DORV with COARV and no subarterial VSD extension, PS, cor triatriatum, and left SVC to unroofed coronary sinus with long-term follow-up after successful single-stage anatomical repair. This technique of VSD translocation has been associated with adequate growth of the left ventricular outflow tract as evidenced by our long-term angiographic and hemodynamic data showing no obstruction between the LV and the aorta 9 years after initial repair.
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This article has been cited by other articles:
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  J. H. Shuhaiber, S. Y. Ho, M. Rigby, and B. Sethia Current options and outcomes for the management of atrioventricular septal defect Eur. J. Cardiothorac. Surg., May 1, 2009; 35(5): 891 - 900. [Abstract] [Full Text] [PDF]
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 J. M. Caffarena and J. M. Gomez-Ullate Biventricular repair of complete atrioventricular canal, double-outlet right ventricle and common atrium using a modified double switch technique. A valid alternative to univentricular procedure Interactive CardioVascular and Thoracic Surgery, June 1, 2005; 4(3): 200 - 202. [Abstract] [Full Text] [PDF]
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 E. A. Bacha and J. P. Starr One-stage repair of complete atrioventricular canal, double-outlet right ventricle, left superior vena cava, cor triatriatum and pulmonary stenosis Ann. Thorac. Surg., January 1, 2003; 75(1): 323 - 323. [Full Text] [PDF]
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