Abstract
There is a large group of young adults who survived atrial baffle repair of transposition of the great arteries. Most survivors are asymptomatic, although nearly all have decreased exercise capacity. Loss of sinus rhythm and atrial arrhythmias are common and increase with age. There is concern about the ability of the right ventricle to function long term as a systemic pump, and recent publications have highlighted right ventricular dysfunction in this patient population. Sudden death and congestive heart failure are the main causes of death, and outcomes beyond 30 years are unknown. Pulmonary artery banding, late arterial switch, and cardiac transplantation are employed when intractable arrhythmias or right ventricular failure threaten survival or quality of life.
Similar content being viewed by others
References and Recommended Reading
Senning A: Surgical correction of transposition of the great vessels. Surgery 1959, 45:966–980.
Mustard WT: Successful two-stage correction of transposition of the great vessels. Surgery 1964, 55:469–472.
Wilson NJ, Clarkson PM, Barratt-Boyes BG, et al.: Long-term outcome after the Mustard repair for simple transposition of the great arteries. J Am Coll Cardiol 1998, 32:758–765.
Puley G, Siu S, Connelly M, et al.: 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.
Moons P, Gewillig M, Sluysmans T, et al.: Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart 2004, 90:307–313.
Roos-Hesselink J, Meijboom FJ, Spitaels SEC, et al.: Decline in ventricular function and clinical condition after Mustard repair for transposition of the great arteries (a prospective study of 22–29 years). Eur Heart J 2004, 25:1264–1270. The authors evaluated 54 patients in 1990 and again in 2001. They demonstrated an increase in QRS duration, a decline in New York Heart Association functional class, echocardiographic decrease in RV in 65% of patients. This study suggests that RV dysfunction may appear late after Mustard operation and may be progressive.
Birnie D, Tometzki A, Curzio J, et al.: Outcomes of transposition of the great arteries in the era of atrial inflow correction. Heart 1998, 80:170–173.
Sun ZH, Happonen JM, Bennhagen R, et al.: 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. This study of 22 TGA patients with sudden death were compared with 24 TGA controls. Loss of sinus rhythm was accompanied by an OR for sudden death of 9.5 (3.13–28.8). Increased QT dispersion also associated with an increased risk of sudden death as it is for Tetralogy of Fallot.
Kammeraad JAE, van Deurzen CHM, Sreeram N, et al.: Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. J Am Coll Cardiol 2004, 44:1095–1102.
Culbert EL, Ashburn DA, Cullen-Dean G, et al.: Quality of life of children after repair of transposition of the great arteries. Circulation 2003, 108:857–862.
Ebenroth ES, Hurwitz RA: Functional outcome of patients operated for d-Transposition of the great arteries with the Mustard procedure. Am J Cardiol 2002, 89:353–356.
Moons P, De Blesser L, Budts W, et al.: Health status, functional abilities, and quality of life after Mustard or Senning operation. Ann Thorac Surg 2004, 77:1359–1365.
Paul MH, Wessel HU: Exercise studies in patients with transposition of the great arteries after atrial repair operations (Mustard/Senning): a review. Pediatr Cardiol 1999, 20:49–55.
Reybrouck T, Mertens L, Brown S, et al.: Long-term assessment and serial evaluation of cardiorespiratory exercise performance and cardiac function in patients with atrial switch operation for complete transposition. Cardiol Young 2001, 11:17–24.
Hechter SJ, Webb G, Fredriksen PM, et al.: Cardiopulmonary exercise performance in adult survivors of the Mustard procedure. Cardiol Young 2001, 11:407–414.
Bolger AP, Gatzoulis MA: Towards defining heart failure in adults with congenital heart disease. Int J Cardiol 2004, 97:15–23.
Clarkson PM, Wilson NJ, Neutze JM, et al.: Outcome of pregnancy after the Mustard operation for transposition of the great arteries with intact ventricular septum. J Am Coll Cardiol 1994, 24:190–193.
Guedes A, Mercier LA, Leduc L, et al.: Impact of pregnancy on the systemic right ventricle after a Mustard operation for transposition of the great arteries. J Am Coll Cardiol 2004, 44:433–437. This is a retrospective study of 28 pregnancies in 16 women with Mustard operation. It adds to the series reported by Clarkson et al. [17] and provides information on pre-and postpregnancy RV function and tricuspid regurgitation. The study raises the possibility that pregnancy produces irreversible RV dysfunction in this population.
Siu SC, Sermer M, Colman JM, et al.: Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation 2001, 104:515–521. This is an epic study of 562 consecutive pregnant women with heart disease. It defines the range of maternal and fetal risks and provides valuable information for risk stratification.
Gewillig M, Cullen S, Mertens B, et al.: Risk factors for arrhythmia and death after Mustard operation for simple transposition of the great arteries. Circulation 1991, 84:III–187-III–192.
Myridakis DJ, Ehlers KH, Engle MA: 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.
Agnetti A, Carano N, Cavalli C, et al.: Long-term outcome after Senning operation for transposition of the great arteries. Clin Cardiol 2004, 27:611–614.
Gelatt M, Hamilton RM, McCrindle BW, et al.: Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol 1997, 29:194–201.
Van Hare GF, Lesh M, Ross BA, et al.: Mapping and radiofrequency ablation of intra-atrial reentrant tachycardia after the Senning or Mustard procedure for transposition of the great arteries. Am J Cardiol 1996, 77:985–991.
Perry JC, Boramanand NK, Ing FF: “Transseptal” technique through atrial baffles for 3-dimensional mapping and ablation of atrial tachycardia in patients with d-transposition of the great arteries. J Interv Card Electrophysiol 2003, 9:365–369.
Fogel MA, Hubbard A, Weinberg PM: A simplified approach for assessment of intracardiac baffles and extracardiac conduits in congenital heart surgery with two- and three-dimensional magnetic resonance imaging. Am Heart J 2001, 142:1028–1103.
Kaulitz R, Stumper OF, Geuskens R, et al.: Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after atrial correction procedure. J Am Coll Cardiol 1990, 16:686–694.
Kedia A, Hsu PYF, Holmes J, et al.: Use of intracardiac echocardiography in guiding radiofrequency catheter ablation of atrial tachycardia in a patient after Senning operation. PACE 2003, 26:2178–2180.
Kanter RJ, Papagiannis J, Carboni MP, et al.: Radiofrequency catheter ablation of supraventricular tachycardia substrates after Mustard and Senning operations for d-transposition of the great arteries. J Am Coll Cardiol 2000, 35:428–441.
Zrenner B, Dong J, Schreieck J, et al.: Delineation of intra-atrial reentrant tachycardia circuits after Mustard operation for transposition of the great arteries using bilateral electroanatomic mapping and entrainment mapping. J Cardiovasc Electrophysiol 2003, 14:1302–1310.
Gatzoulis MA, Walters J, McLaughlin PR, et al.: Late arrhythmia in adults with the Mustard procedure for transposition of great arteries: a surrogate marker for right ventricular dysfunction? Heart 2000, 84:409–415.
Brown SC, Eyskens B, Mertens L, et al.: Self expandable stents for relief of venous baffle obstruction after the Mustard operation. Heart 1998, 79:230–233.
Bu'Lock FA, Tometzki AJP, Kitchiner DJ, et al.: Balloon expandable stents for systemic venous pathway stenosis late after Mustard's operation. Heart 1998, 79:225–229.
Ward CJ, Mullins CE, Nihill MR, et al.: Use of intravascular stents in systemic venous and systemic venous baffle obstructions. Circulation 1995, 91:2948–2954.
Mohsen AE, Rosenthal E, Qureshi SA, et al.: Stent implantation for superior vena cava occlusion after the Mustard operation. Catheter Cardiovasc Interv 2001, 52:351–354.
Hornung TS, Derrick GP, Deanfield JE, et al.: Transposition complexes in the adult: a changing perspective. Clin Cardiol 2002, 20:405–420. This is an excellent review article that addresses the entire spectrum of transposition of the great arteries. It is an good overview of the anatomy, surgical approaches, and late complications, and is a valuable reference for trainees and practitioners less familiar with congenital heart disease.
Williams WG, McCrindle BW, Ashburn DA, et al.: Outcomes of 829 neonates with complete transposition of the great arteries 12–17 years after repair. Eur J Cardiothorac Surg 2003, 24:1–10.
Balzer DT, Johnson M, Sharkey AM, et al.: Transcatheter occlusion of baffle leaks following atrial switch procedures for transposition of the great vessels (d-TGV). Catheter Cardiovasc Interv 2004, 61:259–263.
Schneider DJ, Moore JW: Transcatheter treatment of IVC channel obstruction and baffle leak after Mustard procedure for d-transposition of the great arteries using Amplatzer ASD device and multiple stents. J Invasive Cardiol 2001, 13:306–309.
Kirjavainen M, Happonen J, Louhimo I: Late results of Senning operation. J Thorac Cardiovasc Surg 1999, 117:448–4995.
Burkhart HM, Dearani JA, Williams WG, et al.: Late results of palliative atrial switch for transposition, ventricular septal defect, and pulmonary vascular obstructive disease. Ann Thorac Surg 2004, 77:464–469.
Vogel M, Derrick G, White PA, et al.: Systemic ventricular function in patients with transposition of the great arteries after atrial repair: a tissue Dopplet and conductance catheter study. J Am Coll Cardiol 2004, 43:100–106.
Li W, Hornung TS, Francis DP, et al.: Relation of biventricular function quantified by stress echocardiography to cardiopulmonary exercise capacity in adults with Mustard (atrial switch) procedure for transposition of the great arteries. Circulation 2004, 110:1380–1386.
Laffon E, Jimenez M, Latrabe V, et al.: Quantitative MRI comparison of systemic hemodynamics in Mustard/Senning repaired patients and healthy volunteers at rest. Eur Radiol 2004, 14:875–880.
Tulevski II, van der Wall EE, Groenink M, et al.: Usefulness of magnetic resonance imaging dobutamine stress in asymptomatic and minimally symptomatic patients with decreased cardiac reserve from congenital heart disease(complete and corrected transposition of the great arteries and subpulmonic obstruction). Am J Cardiol 2002, 89:1077–1081.
Lissin LW, Li W, Murphy DJ, et al.: Comparison of transthoracic echocardiography versus cardiovascular magnetic resonance imaging for the assessment of ventricular function in adults after atrial switch procedures for complete transposition of the great arteries. Am J Cardiol 2004, 93:654–657.
RoestLW, Lamb HJ, van der Wall EE, et al.: Cardiovascular response to physical exercise in adult patients after atrial correction for transposition of the great arteries assessed with magnetic resonance imaging. Heart 2004, 90:678–684. The authors performed rest and exercise MRI in 27 patients and 14 controls. There was an abnormal response to exercise of both ventricles. This study will provide valuable comparison data for other studies, interventions, and populations.
Piran S, Veldtman G, Siu S, et al.: Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation 2002, 105:1189–1194.
Hornung TS, Anagnostopoulos C, Bhardwaj P, et al.: Comparison of equilibrium radionuclide ventriculography with cardiovascular magnetic resonance for assessing the systemic right ventricle after Mustard or Senning procedures for complete transposition of the great arteries. Am J Cardiol 2003, 92:640–643.
Markle BM, Cross RR: Cross-sectional imaging in congenital anomalies of the heart and great vessels: magnetic resonance imaging and computed tomography. Semin Roentgenol 2004, 39:234–262.
Oechslin EN, Harrison DA, Connelly MS, et al.: Mode of death in adults with congenital heart disease. Am J Cardiol 2000, 86:1111–1116.
Carrel T, Pfammatter JP: Complete transposition of the great arteries: Surgical concepts for patients with systemic right ventricular failure following intra-atrial repair. Thorac Cardiovasc Surg 2000, 48:224–227.
Millane T, Bernard EJ, Jaeggi E, et al.: Role of ischemia and infarction in late right ventricular dysfunction after atrial repair of transposition of the great arteries. J Am Coll Cardiol 2000, 36:1661–1668.
Singh TP, Humes RA, Muzik O, et al.: Myocardial flow reserve in patients with a systemic right ventricle after atrial switch repair. J Am Coll Cardiol 2001, 37:2120–2125.
Hornung TS, Kilner PJ, Davlouros PA, et al.: Excessive right ventricular hypertrophic response in adults with the Mustard procedure for transposition of the great arteries. Am J Cardiol 2002, 90:800–803.
Hechter SJ, Fredriksen PM, Liu P, et al.: Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure. Am J Cardiol 2001, 87:660–663.
Robinson BH, Heise CT, Moore JW, et al.: Afterload reduction therapy in patients following intraatrial baffle operation for transposition of the great arteries. Pediatr Cardiol 2002, 23:618–623.
Lester SJ, McElhenney DB, Viloria E, et al.: Effects of Losartan in patients with systemically functioning morphologic right ventricle after atrial repair of transposition of the great arteries. Am J Cardiol 2001, 88:1314–1316.
Janousek J, Tomek V, Chaloupecky V, et al.: Cardiac resynchronization therapy: a novel adjunct to the treatment and prevention of systemic right ventricular failure. J Am Coll Cardiol 2004, 44:1927–1931.
Mee RBB: Severe right ventricular failure after Mustard or Senning procedure. Two stage repair: pulmonary artery banding and switch. J Thorac Cardiovasc Surg 1986, 92:385–390.
Poirier NC, Yu JH, Brizard CP, et al.: 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. This large clinical series describes the results of late arterial switch in 39 children and adults over 20 years. The authors' considerable experience should guide any future treatment plans for the transposition population.
Cetta F, Bonilla JJ, Lichtenberg RC, et al.: Anatomic correction of dextrotransposition of the great arteries in a 36-year-old patient. Mayo Clin Proc 1997, 72:245–247.
Inoue M, Oba O, Arai S, et al.: Successful arterial switch operation for post-Mustard pulmonary venous obstruction and secondary pulmonary hypertension. Ann Thorac Surg 2002, 73:975–977.
Van Son JAM, Reddy VM, Silverman NH, et al.: Regression of tricuspid regurgitation after two-stage arterial switch operation for failing systemic ventricle after atrial inversion operation. J Thorac Cardiovasc Surg 1996, 111:342–347.
Benzaquen BS, Webb GD, Colman JM, et al.: 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.
Mavroundis C, Backer C: Arterial switch after failed atrial baffle procedures for transposition of the great arteries. Ann Thorac Surg 2000, 69:851–857.
Daebritz SH, Tiete AR, Sachweh JS, et al.: Systemic right ventricular failure after atrial switch operation: midterm results of conversion into a arterial switch. Ann Thorac Surg 2001, 71:1255–1259.
Sarkar D, Bull C, Yates R, et al.: Comparison of long-term outcomes of atrial repair of simple transposition with implications for a late arterial switch strategy. Circulation 1999, 100:II–176-II–181.
Landzberg MJ, Murphy DJ, Davidson WR, et al.: Task Force 4: organization of delivery systems for adults with congenital heart disease. J Am Coll Cardiol 2001, 37:1187–1193.
Deanfield J, Thaulow E, Warnes C, et al.: Management of grown up congenital heart disease. Eur Soc Cardiol 2003, 24:1035–1084.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Murphy, D.J. Transposition of the great arteries: long-term outcome and current management. Curr Cardiol Rep 7, 299–304 (2005). https://doi.org/10.1007/s11886-005-0052-0
Issue Date:
DOI: https://doi.org/10.1007/s11886-005-0052-0