Ann Thorac Surg 2006;82:1535-1537
© 2006 The Society of Thoracic Surgeons
Case Reports
Transplantation in Truncus Arteriosus Combined with Interrupted Aortic Arch
Hakan Akintuerk, MD,
Gerold Goerlach, MD, PhD*,
Klaus Valeske, MD,
Matthias Mueller, MD,
Juergen Bauer, MD,
Stefan Scholz, MD,
Dietmar Schranz, MD, PhD
Children Heart Center, Justus-Liebig-University, Giessen, Germany
Accepted for publication February 2, 2006.
* Address correspondence to Dr Goerlach, Children Heart Center, Rudolf-Buchheim-Strasse 7, Giessen, 35385 Germany. (Email: gerold.goerlach{at}chiru.med.uni-giessen.de).
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Abstract
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We present the case of an infant with truncus arteriosus combined with a dysplastic truncal valve not amenable to repair and interrupted aortic arch. Due to the disappointing results of truncal valve replacement we decided to perform cardiac transplantation. The infant survived transplantation and was discharged 4 weeks after surgery.
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Introduction
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In the current era, complete one-stage repair is the method of choice in infants with truncus arteriosus associated with interrupted aortic arch. In case of truncal valve dysfunction, repair is a preferable option [1]. If valve replacement is mandatory the results are disappointing [2, 3].
A female neonate whose birth weight was 2,690 g developed tachypnea, which was accompanied by a decrease of arterial saturation. Echocardiography showed a truncus arteriosus associated with interrupted aortic arch type B (Fig 1). The truncal valve was moderately insufficient and a severe stenosis was found. At cardiac catheterization the systolic gradient was 60 mm Hg. Due to dysplasia the valve was not suitable for repair.
Considering the inferior results of truncal valve replacement, we decided to perform cardiac transplantation. On the 7th day of age, bilateral banding of the pulmonary arteries using polytetrafluoroethylene prostheses (diameter, 3.5 mm) was performed by surgery. The size was chosen according to the body weight. Twenty days later a stent (length, 20 mm; diameter, 9 mm) was placed into the ductus arteriosus (Fig 2). Prostaglandin was administered until the placement of the stent. Treated with diuretics and digoxin the infant remained in a stable condition until transplantation.
Seven weeks after the birth we got an organ. The age of the donor was 8 days and his weight was 4,200 g. Cardiopulmonary bypass was established by bi-caval cannulation, an 8-French cannula was placed in the innominate artery, and a 6-French cannula was placed in the ductus arteriosus proximal to the stent. The body temperature was decreased to 20°C. During cooling we clamped the truncus arteriosus below the origin of the innominate artery and ligated the ductus arteriosus at its origin. Proximal to the pulmonary bifurcation we detached the truncus arteriosus. After performing the atrial anastomoses, we removed the cannula placed into the ductus arteriosus, resected the ductus including the stent, and divided the truncus arteriosus distal to the pulmonary bifurcation and closed it with a running suture (Fig 3). We continued the procedure during low flow perfusion. We anastomosed the distal aortic arch end-to-end to the descending aorta of the donor and the proximal aortic arch of the recipient was anastomosed end-to-side to the ascending aorta of the donor (Fig 4). After cannulation of the new aortic arch the aortic clamp was removed. Thereafter the pulmonary banding was removed and a patch plasty of both pulmonary arteries was performed. The donor pulmonary artery was anastomosed end-to-end to the pulmonary bifurcation of the previous truncus arteriosus (Fig 4). The ischemic time of the donor organ was 180 minutes.
Pulmonary hypertension was managed by prostaglandin and nitrous oxide therapy. The infant was extubated on postoperative day 5. After an uneventful recovery during the following course the infant was discharged 4 weeks after transplantation. Echocardiography showed a normal cardiac function.
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Comment
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In a Congenital Heart Surgeons Society study, concerning interrupted aortic arch, the mortality in patients with associated truncus arteriosus was 67% [4]. Miyamoto and colleagues [5] reported a 1-year survival rate of 37.5%. Only patients without moderate or severe truncal valve regurgitation survived. Tlaskal and associates [6] observed a mortality of 13% in neonates. Jahangiri and colleagues [1] concluded that interrupted aortic arch associated to truncus arteriosus is no longer a risk factor for repair. In both publications none of the patients underwent truncus valve repair or replacement. After truncal valve replacement, Elkins and associates [2] reported a mortality of 50%, and McElhinney and colleagues [3] observed a 30% survival rate after 7 months. In a recent article of the Congenital Heart Surgeons Society the mortality in patients with truncal valve stenosis was 81% [7].
The 5-year survival rate in our pediatric cardiac transplantation program is 84% in all patients and 69% in those with hypoplastic left heart syndrome [8]. Considering this experience and the previously mentioned results we believe that transplantation is the method of choice in patients with truncal valve lesions not amenable to repair. Reviewing literature databases we found one similar case treated by transplantation [7].
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References
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- Jahangiri M, Zurakowski D, Mayer JE, del Nido PJ, Jonas RA. Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus J Thorac Cardiovasc Surg 2000;119:508-514.[Abstract/Free Full Text]
- Elkins RC, Steinberg JB, Razook JD, Ward KE, Overholt ED, Thompson WM. Correction of truncus arteriosus with truncal valve stenosis or insufficiency using two homografts Ann Thorac Surg 1990;50:728-733.[Abstract]
- McElhinney DB, Reddy MV, Rajasinghe HA, Mora BN, Silverman NH, Hanley FL. Trends in the management of truncal valve insufficiency Ann Thorac Surg 1998;65:517-524.[Abstract/Free Full Text]
- McCrindle BW, Tchervenkov CI, Konstatinov IE, et al. Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a Congenital Heart Surgeons Society study J Thorac Cardiovasc Surg 2005;129:343-350.[Abstract/Free Full Text]
- Miyamoto T, Sinzobahamvya N, Kumpikaite D, et al. Repair of truncus arteriosus and aortic arch interruption: outcome analysis Ann Thorac Surg 2005;79:2077-2082.[Abstract/Free Full Text]
- Tlaskal T, Hucin B, Kucera V, et al. Repair of persistent truncus arteriosus with interrupted aortic arch Eur J Cardiothorac Surg 2005;28:736-741.[Abstract/Free Full Text]
- Konstantinov IE, Karamlou T, Blackstone EH, et al. Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study Ann Thorac Surg 2006;81:214-222.[Abstract/Free Full Text]
- Bauer J, Thul J, Kraemer U, et al. Heart transplantation in children and infants: short-term outcome and long-term follow-up Pediatr Transplantation 2001;5:457-462.
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Abstract
Introduction
