Outcome after Surgical Repair/Palliation of Congenitally Corrected Transposition of the Great Arteries

Abstract

Background: The surgical concepts for patients with congenitally corrected transposition of the great arteries (CCTGA) address discordant connections and associated lesions. The outcomes after biventricular repair without correction of discordant connections (“classic repair”, or with its correction “anatomic repair”) and after “univentricular palliation” were investigated. Methods: All patients with CCTGA who underwent “classic repair” (n = 39), “anatomic repair” (n = 6), or “univentricular palliation” (n = 11) between 1978 and 2006 were analyzed. The most frequently associated lesions were ventricular septal defect (n = 48), tricuspid insufficiency (TI) (n = 20) and functionally single ventricle (n = 11). Results: Thirty-day mortality was 4 % (2/56). Mean follow-up for early survivors was 7.2 ± 7.1 years. Eight patients died late, two after heart transplantation. Survival was not significantly different between patients who underwent “anatomic” or “classic repair”, or “univentricular palliation”: 83.3 ± 15.2 %, 79.7 ± 6.9 %, 90.9 ± 8.7 % at 10 years, respectively. In multivariate analysis, the presence of TI emerged as the only risk factor for late death (p = 0.004). Twenty patients required reoperation, mainly for TI (n = 10) and conduit failure (n = 6). Freedom from reoperation was lower after “anatomic repair”, but ventricular function was better and atrioventricular valves were more competent than after “classic repair”. Conclusions: Biventricular “anatomic” or “classic repair” and “univentricular palliation” yield equivalent survival rates in the mid-term. Biventricular “anatomic repair”, when feasible, should be promoted because of its better long-term outcome.

Key words

cardiovascular surgery - congenital heart disease - great vessel anomalies

References

• 1 Allwork S P, Bentall H H, Becker A E, Cameron H, Gerlis L M, Wilkinson J L, Anderson R H. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases.  Am J Cardiol. 1976;  38 910-923
• 2 Anderson R H, Becker A E, Arnold R, Wilkinson J L. The conducting tissues in congenitally corrected transposition.  Circulation. 1974;  50 911-923
• 3 Sano T, Riesenfeld T, Karl T R, Wilkinson J L. Intermediate-term outcome after intracardiac repair of associated cardiac defects in patients with atrioventricular and ventriculoarterial discordance.  Circulation. 1995;  92 II272-II278
• 4 Termignon J L, Leca F, Vouhe P R, Vernant F, Bical O M, Lecompte Y, Neveux J Y. «Classic» repair of congenitally corrected transposition and ventricular septal defect.  Ann Thorac Surg. 1996;  62 199-206
• 5 Yeh Jr T, Connelly M S, Coles J G, Webb G D, McLaughlin P R, Freedom R M, Cerrito P B, Williams W G. Atrioventricular discordance: results of repair in 127 patients.  J Thorac Cardiovasc Surg. 1999;  117 1190-1203
• 6 Prieto L R, Hordof A J, Secic M, Rosenbaum M S, Gersony W M. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries.  Circulation. 1998;  98 997-1005
• 7 Acar P, Sidi D, Bonnet D, Aggoun Y, Bonhoeffer P, Kachaner J. Maintaining tricuspid valve competence in double discordance: a challenge for the paediatric cardiologist.  Heart. 1998;  80 479-483
• 8 Ilbawi M N, DeLeon S Y, Backer C L, Duffy C E, Muster A J, Zales V R, Paul M H, Idriss F S. An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia.  J Thorac Cardiovasc Surg. 1990;  100 410-415
• 9 Yamagishi M, Imai Y, Hoshino S, Ishihara K, Koh Y, Nagatsu M, Shinoka T, Koide M. Anatomic correction of atrioventricular discordance.  J Thorac Cardiovasc Surg. 1993;  105 1067-1076
• 10 Koh M, Yagihara T, Uemura H, Kagisaki K, Hagino I, Ishizaka T, Kitamura S. Intermediate results of the double-switch operations for atrioventricular discordance.  Ann Thorac Surg. 2006;  81 671-677 677
• 11 Ilbawi M N, Ocampo C B, Allen B S, Barth M J, Roberson D A, Chiemmongkoltip P, Arcilla R A. Intermediate results of the anatomic repair for congenitally corrected transposition.  Ann Thorac Surg. 2002;  73 594-599 599-600
• 12 Devaney E J, Charpie J R, Ohye R G, Bove E L. Combined arterial switch and Senning operation for congenitally corrected transposition of the great arteries: patient selection and intermediate results.  J Thorac Cardiovasc Surg. 2003;  125 500-507
• 13 Langley S M, Winlaw D S, Stumper O, Dhillon R, De Giovanni J V, Wright J G, Miller P, Sethia B, Barron D J, Brawn W J. Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great arteries.  J Thorac Cardiovasc Surg. 2003;  125 1229-1241
• 14 Graham Jr T P, Bernard Y D, Mellen B G, Celermajer D, Baumgartner H, Cetta F, Connolly H M, Davidson W R, Dellborg M, Foster E, Gersony W M, Gessner I H, Hurwitz R A, Kaemmerer H, Kugler J D, Murphy D J, Noonan J A, Morris C, Perloff J K, Sanders S P, Sutherland J L. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study.  J Am Coll Cardiol. 2000;  36 255-261
• 15 Lange R, Horer J, Kostolny M, Cleuziou J, Vogt M, Busch R, Holper K, Meisner H, Hess J, Schreiber C. Presence of a ventricular septal defect and the Mustard operation are risk factors for late mortality after the atrial switch operation: thirty years of follow-up in 417 patients at a single center.  Circulation. 2006;  114 1905-1913
• 16 Horer J, Haas F, Cleuziou J, Schreiber C, Kostolny M, Vogt M, Holper K, Lange R. Intermediate-term results of the Senning or Mustard procedures combined with the Rastelli operation for patients with discordant atrioventricular connections associated with discordant ventriculoarterial connections or double outlet right ventricle.  Cardiol Young. 2007;  17 158-165
• 17 Alghamdi A A, McCrindle B W, Van Arsdell G S. Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries: meta-analysis of individual patient data.  Ann Thorac Surg. 2006;  81 1529-1535
• 18 Hraska V, Duncan B W, Mayer Jr J E, Freed M, del Nido P J, Jonas R A. Long-term outcome of surgically treated patients with corrected transposition of the great arteries.  J Thorac Cardiovasc Surg. 2005;  129 182-191
• 19 Jahangiri M, Redington A N, Elliott M J, Stark J, Tsang V T, de Leval M R. A case for anatomic correction in atrioventricular discordance? Effects of surgery on tricuspid valve function.  J Thorac Cardiovasc Surg. 2001;  121 1040-1045
• 20 Bautista-Hernandez V, Marx G R, Gauvreau K, Mayer Jr J E, Cecchin F, del Nido P J. Determinants of left ventricular dysfunction after anatomic repair of congenitally corrected transposition of the great arteries.  Ann Thorac Surg. 2006;  82 2059-2065 2065-2066
• 21 Delius R E, Rademecker M A, de Leval M R, Elliott M J, Stark J. Is a high-risk biventricular repair always preferable to conversion to a single ventricle repair?.  J Thorac Cardiovasc Surg. 1996;  112 1561-1568 1568-1569

1 Both authors contributed equally to the study.