How Well Are Patients Doing Up to 30 Years after a Mustard Operation?

Abstract

Background: Right heart failure and baffle complications may affect the health status of patients with transposition of the great arteries after an atrial switch operation. Methods: This study aims to identify risk factors for late death, the incidence of reoperations, and the functional status of 88 patients who underwent a Mustard operation with a mean follow-up of 20.9 ± 10.0 years. Results: There were 7 early and 19 late deaths. Follow-up was complete for 97 % of the hospital survivors. Survival and freedom from reoperation of the hospital survivors at 20 years was 83.7 ± 4.2 %, and 70.6 ± 5.4 %, respectively. Seven reoperations were performed for systemic ventricular failure, and 24 for baffle complications, with no operative mortality. Presence of a ventricular septal defect at the time of the Mustard operation was predictive for late death in multivariate analysis (p = 0.040). At follow-up, 82 % of the patients were able to work full-time, 11 % part-time, and 7 % experienced noticeable limitations of their activities. Conclusions: Presence of a VSD at the time of the Mustard operation defines a distinct subgroup with an increased risk for late death. Long-term survivors were in a good functional status but had to be reoperated frequently due to baffle complications that seemed to increase in adulthood.

adult - heart defects - congenital - follow‐up studies - surgery - transposition of great arteries - risk factors

References

• 1 Blalock A, Hanlon C R. The surgical treatment of complete transposition of the aorta and the pulmonary artery.  Surg Gynecol Obstet. 1950;  90 1-15
• 2 Rashkind W J, Miller W W. Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries.  JAMA. 1966;  196 991-992
• 3 Senning A. Surgical correction of transposition of the great vessels.  Surgery. 1959;  45 966-980
• 4 Mustard W T. Successful two-stage correction of the transposition of the great vessels.  Surgery. 1963;  55 469-472
• 5 Jatene A D, Fontes V F, Paulista P P, Souza L C, Neger F, Galantier M, Sousa J E. Anatomic correction of transposition of the great vessels.  J Thorac Cardiovasc Surg. 1976;  72 364-370
• 6 Birnie D, Tometzki A, Curzio J, Houston A, Hood S, Swan L, Doig W, Wilson N, Jamieson M, Pollock J, Hillis W S. Outcomes of transposition of the great arteries in the era of atrial inflow correction.  Heart. 1998;  80 170-173
• 7 Moons P, Gewillig M, Sluysmans T, Verhaaren H, Viart P, Massin M, Suys B, Budts W, Pasquet A, De Wolf D, Vliers A. Long-term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium.  Heart. 2004;  90 307-313
• 8 Kirjavainen M, Happonen J M, Louhimo I. Late results of Senning operation.  J Thorac Cardiovasc Surg. 1999;  117 488-495
• 9 Agnetti A, Carano N, Cavalli C, Tchana B, Bini M, Squarcia U, Frigiola A. Long-term outcome after Senning operation for transposition of the great arteries.  Clin Cardiol. 2004;  27 611-614
• 10 Oechslin E, Jenni R. 40 years after the first atrial switch procedure in patients with transposition of the great arteries: long-term results in Toronto and Zurich.  Thorac Cardiovasc Surg. 2000;  48 233-237
• 11 Sarkar D, Bull C, Yates R, Wright D, Cullen S, Gewillig M, Clayton R, Tunstill A, Deanfield J. Comparison of long-term outcomes of atrial repair of simple transposition with implications for a late arterial switch strategy.  Circulation. 1999;  100 II176-II181
• 12 Warnes C A, Somerville J. Tricuspid atresia in adolescents and adults: current state and late complications.  Br Heart J. 1986;  56 535-543
• 13 Peto R, Peto J. Asymptotically efficient rank invariant test procedures.  J R Stat Soc. 1972;  135 185-206
• 14 Gelatt M, Hamilton R M, McCrindle B W, Connelly M, Davis A, Harris L, Gow R M, Williams W G, Trusler G A, Freedom R M. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience.  J Am Coll Cardiol. 1997;  29 194-201
• 15 Meijboom F, Szatmari A, Deckers J W, Utens E M, Roelandt J R, Bos E, Hess J. Long-term follow-up (10 to 17 years) after Mustard repair for transposition of the great arteries.  J Thorac Cardiovasc Surg. 1996;  111 1158-1168
• 16 Wells W J, Blackstone E. Intermediate outcome after Mustard and Senning procedures: a study by the Congenital Heart Surgeons Society.  Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2000;  3 186-197
• 17 Williams W G, McCrindle B W, Ashburn D A, Jonas R A, Mavroudis C, Blackstone E H. Outcomes of 829 neonates with complete transposition of the great arteries 12 - 17 years after repair.  Eur J Cardiothorac Surg. 2003;  24 1-9 9-10
• 18 Myridakis D J, Ehlers K H, Engle M A. Late follow-up after venous switch operation (Mustard procedure) for simple and complex transposition of the great arteries.  Am J Cardiol. 1994;  74 1030-1036
• 19 Helbing W A, Hansen B, Ottenkamp J, Rohmer J, Chin J G, Brom A G, Quaegebeur J M. Long-term results of atrial correction for transposition of the great arteries. Comparison of Mustard and Senning operations.  J Thorac Cardiovasc Surg. 1994;  108 363-372
• 20 Clarkson P M, Neutze J M, Barratt-Boyles B G, Brandt P W. Late postoperative hemodynamic results and cineangiocardiographic findings after Mustard atrial baffle repair for transposition of the great arteries.  Circulation. 1976;  53 525-532
• 21 Arciniegas E, Farooki Z Q, Hakimi M, Perry B L, Green E W. Results of the Mustard operation for dextro-transposition of the great arteries.  J Thorac Cardiovasc Surg. 1981;  81 580-587
• 22 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
• 23 Carrel T, Pfammatter J P. Complete transposition of the great arteries: surgical concepts for patients with systemic right ventricular failure following intraatrial repair.  Thorac Cardiovasc Surg. 2000;  48 224-227
• 24 Winlaw D S, McGuirk S P, Balmer C, Langley S M, Griselli M, Stumper O, De Giovanni J V, Wright J G, Thorne S, Barron D J, Brawn W J. Intention-to-treat analysis of pulmonary artery banding in conditions with a morphological right ventricle in the systemic circulation with a view to anatomic biventricular repair.  Circulation. 2005;  111 405-411
• 25 Poirier N C, Yu J H, Brizard C P, Mee R B. Long-term results of left ventricular reconditioning and anatomic correction for systemic right ventricular dysfunction after atrial switch procedures.  J Thorac Cardiovasc Surg. 2004;  127 975-981
• 26 Boutin C, Jonas R A, Sanders S P, Wernovsky G, Mone S M, Colan S D. Rapid two-stage arterial switch operation. Acquisition of left ventricular mass after pulmonary artery banding in infants with transposition of the great arteries.  Circulation. 1994;  90 1304-1309
• 27 Benzaquen B S, Webb G D, Colman J M, Therrien J. Arterial switch operation after Mustard procedures in adult patients with transposition of the great arteries: is it time to revise our strategy?.  Am Heart J. 2004;  147 E8
• 28 Puley G, Siu S, Connelly M, Harrison D, Webb G, Williams W G, Harris L. Arrhythmia and survival in patients > 18 years of age after the Mustard procedure for complete transposition of the great arteries.  Am J Cardiol. 1999;  83 1080-1084
• 29 Hosseinpour A R, Cullen S, Tsang V T. Transplantation for adults with congenital heart disease.  Eur J Cardiothorac Surg. 2006;  30 508-514
• 30 Sun Z H, Happonen J M, Bennhagen R, Sairanen H, Pesonen E, Toivonen L, Jokinen E. Increased QT dispersion and loss of sinus rhythm as risk factors for late sudden death after Mustard or Senning procedures for transposition of the great arteries.  Am J Cardiol. 2004;  94 138-141
• 31 Kammeraad J A, van Deurzen C H, Sreeram N, Bink-Boelkens M T, Ottenkamp J, Helbing W A, Lam J, Sobotka-Plojhar M A, Daniels O, Balaji S. Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries.  J Am Coll Cardiol. 2004;  44 1095-1102