Ann Thorac Surg 1997;63:1781-1783
© 1997 The Society of Thoracic Surgeons
Case Report
One-Stage Repair of Truncus Arteriosus, CAVC, and TAPVC
Stefano Conte, MD,
Tim Jensen, MD,
Joes Ramsøe Jacobsen, MD,
Frederic S. Joyce, MD,
Poul Lauridsen, MD,
Gösta Pettersson, MD, PhD
Departments of Cardiothoracic Surgery and Pediatrics, The National University Hospital "Rigshospitalet," Copenhagen, Denmark
Accepted for publication January 23, 1997.
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Abstract
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An infant with truncus arteriosus, complete atrioventricular canal, and total anomalous pulmonary venous connection successfully underwent one-stage complete repair. Residual mitral valve regurgitation required reoperation after 12 days. The patient is doing well at 6 months' follow-up. Echocardiography demonstrates no residual defects, competent atrioventricular valves, and normal pulmonary pressure. This case illustrates the potential for successful one-stage repair even of associated complex heart defects involving venous, intracardiac, and arterial pathways.
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Introduction
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The association of truncus arteriosus (TA), complete atrioventricular canal (CAVC), and total anomalous pulmonary venous connection (TAPVC) is exceedingly rare; our literature search found only 1 postmortem case [1]. Primary surgical repair of TA in association with CAVC [2] or TAPVC [3] has been recently reported. We report successful complete repair of TA with both CAVC and TAPVC.
After an uncomplicated pregnancy, a 2,460-g boy was delivered by cesarean section at the 38th gestational week. On the first day, he successfully underwent surgical treatment of myelomeningocele. An arterial oxygen saturation of 87% motivated further cardiac evaluation. Echocardiography demonstrated situs ambiguus with right isomerism, common atrium, type C CAVC with a grade II atrioventricular valve regurgitation and a large ventricular septal defect (VSD) involving both inlet and infundibular areas, type II truncus arteriosus with a well-functioning tricuspid truncal valve, right-sided aortic arch, and TAPVC of the cardiac type, with all four pulmonary veins draining to the coronary sinus through a common confluence, a vertical vein, and a left superior vena cava with a 20 mm Hg gradient. No innominate artery was present. Abdominal echography showed a transverse liver with absent spleen. At that time, the patient was not considered operable and was discharged from the hospital without therapy.
During the following period he was surprisingly well and developed normally. At 6 months, however, he presented with cyanosis and dyspnea and furosemide treatment was started. The initial diagnosis was confirmed by cardiac catheterization, and angiography showed normal-sized pulmonary artery (PA) branches. The arterial oxygen saturation was 77%. As the suitability of the patient for surgical correction was not clear, it took a few months before a decision in favor of surgical correction could be taken. After long discussions, we assumed that irreversible pulmonary vascular disease was not yet present.
At the time the patient eventually underwent operation he was 11 months old and weighed 8 kg. The operation was performed with bicaval cannulation and continuous cardiopulmonary bypass at 20°C. A single dose of crystalloid cardioplegia was administered through the truncal root. The TAPVC was repaired by means of a large side-to-side anastomosis between the pulmonary venous confluence and the posterior wall of the left portion of the common atrium. The vertical vein was ligated.
The intracardiac repair was accomplished with a three-patch technique. The ventricular septation was obtained with two Dacron patches, the first being used to close the inlet part of the VSD through a right atrial approach and the second to close the outlet part of the VSD through the infundibulum. This patch was sutured to the first one to form an intraventricular tunnel from the posterior left ventricle to the aorta anteriorly (Fig 1A
). To avoid left ventricular outflow tract obstruction, we enlarged the outlet VSD before the reconstruction. The mitral cleft was only partially closed to avoid narrowing the mitral anulus. Atrial septation was achieved by means of a large autologous pericardial patch. Finally, after the truncus arteriosus was transected and the PA confluence detached, the PA confluence was connected to the right ventricle by means of a 14-mm cryopreserved pulmonary homograft and a Dacron patch as a hood. Due to the posterior position of the PA confluence and to reduced length of the homograft (15 mm), this was placed to the right of the aorta (Fig 1B). After rewarming, weaning from cardiopulmonary bypass was possible with moderate inotropic support. The right ventricular/left ventricular pressure ratio was 0.4. Modified ultrafiltration was performed. Total perfusion time was 350 minutes with an aortic cross-clamp time of 190 minutes.
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Fig 1. . Drawings illustrating part of the surgical repair. (A) The ventricular septation is obtained with two Dacron patches; the outlet ventricular septal defect patch is sutured to the inlet ventricular septal defect patch to form an intraventricular tunnel from the posterior left ventricle to the anterior aorta. (B) Right ventricular outflow reconstruction by means of a pulmonary homograft placed to the right of the aorta.
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The sternum was left open 4 days. An early echocardiographic study demonstrated satisfactory repair (Fig 2), except for a residual grade II-III mitral insufficiency. The postoperative course was complicated by renal insufficiency, treated by peritoneal dialysis, and sepsis, caused by an infected groin catheter and requiring prolonged antibiotic therapy. When we were trying to wean the patient from the ventilator, the left atrial pressure rose to 25 mm Hg. On postoperative day 12, the patient successfully underwent a mitral valvuloplasty with complete closure of the mitral cleft and two commissural stitches. The patient was extubated on postoperative day 21 and discharged from the hospital 48 days after the initial operation. Six months after the operation he is in good condition on a regimen of low doses of diuretics, digoxin, and penicillin as prophylaxis because of the asplenia syndrome. On recent Doppler echocardiography, the PA pressure was 25 mm Hg.
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Fig 2. . Postoperative two-dimensional echocardiograms demonstrating (A) unobstructed pulmonary venous return to the left atrium and (B) satisfactory repair at the outflow tract level of both ventricles.
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Comment
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The surgical management of truncus arteriosus has dramatically progressed over the last 10 years. Nowadays, uncomplicated TA can be repaired in the neonatal period with excellent results [4]. However, patients with severe truncal valve incompetence or additional complex heart defects represent still a surgical challenge [5–7]. As regards associated cardiac anomalies, interrupted aortic arch is one of the most common [8]. In contrast, CAVC and TAPVC are very rarely associated with TA. Twelve cases of TA and CAVC have been described, with only 1 successful repair [2]. Similarly, the literature reports only 3 cases of TA and TAPVC, 1 of these successfully corrected [3]. The association of TA and both CAVC and TAPVC has been reported in a patient who died on the sixth day after progressive ventilatory insufficiency and metabolic acidosis [1]. Autopsy confirmed a few other features in common with our case such as the presence of common atrium, asplenia syndrome, and anteroposterior ventricular relationship. Characteristic of our case was the presence of partial pulmonary venous obstruction (20 mm Hg gradient). Patients with TA and arterial oxygen saturation less than 85% usually have pulmonary vascular resistance greater than 12.0 U/m2 and are probably inoperable [5]. In our case, however, the preoperative arterial oxygen saturation of 77% was partly related to the moderate pulmonary venous obstruction.
The decision for surgical repair was delayed mainly because it was very difficult to obtain a complete preoperative evaluation of the patient. However, it seems clear now that an earlier repair would have been possible and perhaps safer.
The operation was technically difficult and time consuming, particularly as regards the understanding of the anatomy and how to connect the posterior left ventricle to the anterior aorta (see Fig 1A
). This was accomplished by a Rastelli-like intracardiac tunnel after resection of the left lateral brim of the interventricular septum to avoid systemic obstruction. This concern led us to use two patches to close the large VSD. For the right ventricle-to-PA connection we used a pulmonary homograft [6], according to our general policy for TA repair. As the available homograft was rather short, it was placed to the right of the aorta (see Fig 1B) to avoid compression of the right PA and the left coronary artery. The anterior pulmonary translocation used by others [2, 8] in similar cases was not considered because of the anterior position of the large truncal root. This case demonstrates that complete repair of the very rare association of TA, CAVC, and TAPVC is possible and that customized technical solutions are often necessary to achieve complete repair of complex cardiac anomalies.
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Footnotes
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Address reprint requests to Dr Conte, Department of Cardiothoracic Surgery, The National University Hospital "Rigshospitalet," 9 Blegdamsvej, 2100 Copenhagen, Denmark.
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References
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- Gumbiner CH, McManus BM, Latson LA. Associated occurrence of persistent truncus arteriosus and asplenia. Pediatr Cardiol 1991;12:192–5.[Medline]
- Sousa-Uva M, Serraf A, Cloez JL, et al. Repair of truncus arteriosus and complete atrioventricular canal defect. J Thorac Cardiovasc Surg 1994;108:385–7.[Free Full Text]
- Berdjis F, Wells WJ, Starnes VA. Truncus arteriosus with total anomalous pulmonary venous return and interrupted aortic arch. Ann Thorac Surg 1996;61:220–2.[Abstract/Free Full Text]
- Hanley FL, Heinemann MK, Jonas RA, et al. Repair of truncus arteriosus in the neonate. J Thorac Cardiovasc Surg 1993;105:1047–56.[Abstract]
- Mair DD, Ritter DG, Davis GD, Wallace RB, Danielson GK, McGoon DC. Selection of patients with truncus arteriosus for surgical correction. Anatomic and hemodynamic considerations. Circulation 1974;49:144–51.[Abstract/Free Full Text]
- Heinemann MK, Hanley FL, Fenton KN, Jonas RA, Mayer JE, Castañeda AR. Fate of small homograft conduits after repair of truncus arteriosus. Ann Thorac Surg 1993;55:1409–12.[Abstract]
- Kirklin JW, Barratt-Boyes BG, eds. Cardiac surgery. 2nd ed. New York: Churchill Livingstone, 1993:1131-51.
- Rao IM, Swanson JS, Hovaguimian H, McIrvin DM, King DH, Starr A. Anterior pulmonary translocation for repair of truncus arteriosus with interrupted arch. Ann Thorac Surg 1995;59:216–8.[Abstract/Free Full Text]
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Abstract
Introduction
