Rare Association of Tetralogy of Fallot with Partially Unroofed Coronary Sinus and PLSVC: Case Report

Abstract

A 41-year-old man with tetralogy of Fallot and dextrocardia, coronary sinus atrial septal defect and partially unroofed coronary sinus with persistent left superior vena cava (PLSVC) underwent a total correction of these anomalies. An association between these specific anomalies has not been reported previously in the literature. These defects were successfully corrected by a) closure of the coronary sinus atrial septal defect with PLSVC entering the right atrium using a pericardial graft; and b) ventricular partition with a Dacron patch and conotruncal repair for tetralogy of Fallot. The postoperative course of this patient has been uneventful.

Key words

Tetralogy of Fallot - partially unroofed coronary sinus - persistent left superior vena cava

References

• 1 Barbaix E, Kerckaert I, D'Herde K, Depreter M, Espeel M. Simultaneous occurrence of a thyromediastinal muscle, a truncus bicaroticobrachialis and a left superior vena cava.  Clin Anat. 2003;  16 176-181
• 2 Quaegebeur J, Kirklin J W, Pacifico A D, Bargeron Jr L M. Surgical experience with unroofed coronary sinus.  Ann Thorac Surg. 1979;  27 418-425
• 3 Cherian K M, Rao S G. Surgical correction of the unroofed coronary sinus syndrome.  Indian Heart J. 1994;  46 91-95
• 4 Nomura K, Kurosawa H, Morita K, Miyamoto H, Tatara A, Hanai M. A surgical case report–rare association of tetralogy of Fallot with cor triatriatum, unroofed coronary sinus and persistent left superior vena cava.  Nippon Kyobu Geka Gakkai Zasshi. 1997;  45 92-95
• 5 Jacques A M V S, Rudiger A, Friedrich W M. Repair of cor triatriatum associated with partially unroofed coronary sinus.  Ann Thorac Surg. 1999;  68 1414-1416
• 6 Hahm J K, Park Y W, Lee J K et al. Magnetic resonance imaging of unroofed coronary sinus: three cases.  Pediatr Cardiol. 2000;  21 382-387
• 7 Ou P, Celermajer D S, Calcagni G, Brunelle F, Bonnet D, Sidi D. Three-dimensional CT scanning: a new diagnostic modality in congenital heart disease.  Heart. 2007;  93 908-913
• 8 Laks H. Invited commentary to: Teske DW, Davis T, Allen HG. Cavopulmonary anastomotic aneurysm: a complication in pulsatile pulmonary arteries.  Ann Thorac Surg. 1994;  57 1661-1664