Congenitally Corrected Transposition of the Great Arteries plus Dextrocardia Operated with an Unusual Operative Technique

Introduction

Congenitally corrected transposition of the great arteries (CCTGA) described by Schiebler and colleagues [1] is a rare form of congenital heart disease for which significant tricuspid insufficiency (TI) has been reported in 20 % to 50 % of patients [2]. Dysfunction, mostly regurgitation of the morphologically tricuspid systemic atrioventricular valve, is one of the strongest risk factors for clinical congestive heart failure (CHF) and right ventricular dysfunction. The main underlying disorder in CCTGA is atrioventricular and ventriculoarterial discordance. The morphologic right ventricle functions as the systemic ventricle, and the morphologic left ventricle functions as the pulmonary ventricle. The atrioventricular valve connected to the systemic ventricle is morphologically tricuspid, and the valve connected to the pulmonary ventricle is morphologically mitral. Although in some patients the morphologic right ventricle retains its normal function even into late adulthood, some dysfunction of the systemic ventricle occurs progressively with age [3]. Various surgical approaches have been proposed to treat this anomaly. We report a patient with CCTGA who underwent a left (tricuspid) atrioventricular (AV) valve replacement through a left atriotomy anterior to the left pulmonary veins with an excellent postoperative outcome.

References

• 1 Schiebler G L, Edwards J E, Burchell H B, DuShane J W, Ongley P A, Wood E H. Congenitally corrected transposition of the great vessels. A study of 33 cases.  Pediatrics. 1961;  27 851-888
• 2 McGrath L B, Kirklin J W, Blackstone E H, Pacifico A D, Kirklin J K, Bargeron Jr L M. Death and other events after cardiac repair in discordant atrioventricular connection.  J Thorac Cardiovasc Surg. 1989;  90 711-723
• 3 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
• 4 Anderson K R, Danielson G K, McGoon D C, Lie J T. Ebstein's anomaly of the left-sided tricuspid valve: pathological anatomy of the valvular malformation.  Circulation. 1978;  58 (Suppl) I87-I91
• 5 Fogel M A, Weinberg P M, Fellows K E, Hoffman E A. A study in ventricular-ventricular interaction: single right ventricles compared with systemic right ventricles in a dual-chamber circulation.  Circulation. 1995;  92 219-230
• 6 Van Son J AM, Danielson G K, Huhta J C, Warnes C A, Edwards W D, Schaff H V, Puga F J, Ilstrup D M. Late results of systemic atrioventricular valve replacement in corrected transposition.  Thorac Cardiovasc Surg. 1995;  109 642-653
• 7 Beauchesne L M, Warnes C A, Connoly H M, Ammash N M, Tajik A J, Danielson G K. Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries.  J Am Coll Cardiol. 2002;  40 285-290
• 8 Akar H, Iriz E, Kolbakir F, Sarac A, Sagkan O. Bacterial endocarditis of systemic valve (tricuspid valve) in corrected transposition and its surgical treatment.  Ann Thorac Cardiovasc Surg. 2005;  11 201-203
• 9 Kambara A, Michler R E. Dextrocardia: technical aspects of reoperative aortic and tricuspid valve replacement.  J Card Surg. 2002;  17 163-165
• 10 Rammos St K, Bakas A J, Panaqopoulas F G. Mitral valve replacement in a Jehovah's Witness with dextrocardia and situs solitus.  J Heart Valve Dis. 1996;  5 673-674