Surgical Approach to Corrected Transposition of the Great Arteries Treatment & Management
- Author: Prema Ramaswamy, MD; Chief Editor: John Kupferschmid, MD more...
Treatment of congestive cardiac failure secondary to a ventricular septal defect (VSD) is along the standard lines of management and includes the use of afterload-reducing agents (eg, enalapril), as well as other agents (eg, digoxin, diuretics).
The guidelines from the American College of Cardiology and the American Heart Association state that permanent pacing for complete heart block is indicated for congenital heart block in infants with any type of congenital heart disease and a heart rate of less than 70 beats per minute.
In infants with corrected transposition of the great arteries with associated defects, pacemakers are implanted regardless of the heart rate or symptoms of heart failure. Permanent pacing is also indicated after surgically induced heart block. In other patients with corrected transposition of the great arteries who develop complete heart block spontaneously after infancy, the placement of a pacemaker is usually predicated by the presence of symptoms. Dual-chamber pacing is the preferred modality for almost all of these patients except perhaps in the smallest (< 15 kg), in whom placement of both leads through the superior vena cava may present a risk of thrombosis and obstruction.
Surgical therapy may be palliative, at least initially, which may be a good option in this condition (discussed above), or it may involve complete repair. Palliation includes pulmonary artery banding in infancy for moderate VSDs and modified Blalock Taussig shunts for infants with severe pulmonic stenosis that causes the oxygen saturation level to fall below 75-80%. Pulmonary artery banding has gained added importance because, in addition to palliation, it also serves as a tool by which to train the morphologic left ventricle in preparation for an anatomic repair, as discussed below.
The period of training required for the morphologic left ventricle to assume the position of a systemic ventricle varies with the age of the child, from 2 weeks in an infant to a few months or even longer in an older child. Winlaw et al found that patients older than 16 years were unlikely to achieve an anatomic repair and that the morphologic left ventricular function was a critical determinant of survival.
Complete repair may be either a physiologic or an anatomic repair. In the former, only the associated defects are addressed, but the right ventricle continues to function as the systemic ventricle. An anatomic repair is one in which the morphologic left ventricle is established as the systemic ventricle. This includes alternative surgical approaches, such as double-switch procedures.
A physiologic repair may involve any of the following procedures:
Relief of pulmonic stenosis
Tricuspid valve replacement
An anatomic repair may involve Senning or Mustard (see below) and arterial switch if no pulmonic stenosis is present and Senning or Mustard with Rastelli, which is a right ventricle–to–pulmonary artery (RV-to-PA) conduit, in the presence of pulmonic stenosis
These procedures are theoretically attractive because the morphologic left ventricle then assumes the position of the systemic ventricle and the late failure of the right ventricle, which is a prominent part of the natural history of this condition, may possibly be avoided.
Pulmonary artery banding
If performed as a precursor to an anatomic repair, this procedure has been noted by several groups to be more effective in younger children. Devaney et al stated that they tighten the pulmonary artery band until the interventricular septum moves to the midline and the left ventricular pressure is 80% of systemic.
Ventricular septal defect closure
Most of these defects are perimembranous in nature. The optimal approach to the VSD has been described to be through the right atrium and the morphologic mitral valve. Risk of possible permanent damage to the mitral valve and injury to the AV node is noted. In 1979, de Leval et al described how the VSD could be approached from the right atrium or through a careful low left ventriculotomy to avoid the AV bundle as it courses along the roof of the pulmonary outflow tract. The VSD is then closed, and the stitches are applied to the morphologic right side of the VSD in order to minimize the risk of damaging the conduction tissue. In older patients with larger aortas, the VSD can also be approached through the aorta, and, because this allows exposure of the right ventricular side of the defect, a reduced risk of injury to the AV node is noted.
Relief of pulmonic stenosis
Usually, many causes of pulmonic stenosis are present in this condition. Because the pulmonic valve is wedged between the tricuspid and the mitral valve, its annulus is usually hypoplastic. Also, obstruction may be caused by subpulmonary narrowing secondary to fibromuscular tissue. Because the conduction tissue passes anterior to the pulmonic valve, incisions in this area can cause complete heart block. Thus, for most patients, an extracardiac conduit is required from the left ventricle to the pulmonary artery. Cryopreserved aortic homografts are usually used for this because they are long enough to be able to reach the pulmonary artery. The conduit must also be positioned to the right so that compression from the sternum is not an issue.
Tricuspid valve replacement
Tricuspid regurgitation is common and is associated with a poor long-term prognosis. Exposure for repair is difficult; hence, replacement of the valve is required when tricuspid regurgitation is severe. A mechanical prosthesis is preferred over a bioprosthesis because of its longevity.
All of the above procedures leave the right ventricle as the systemic ventricle, the progressive failure of which has been noted in long-term follow-up studies. This was confirmed by a large study that demonstrated that the conventional physiologic biventricular repair is one of the risk factors for progressive right ventricular dysfunction. Hence, an alternative concept of an anatomic repair was first proposed by Ilbawi et al, who performed it in corrected transposition, VSD, and pulmonic stenosis. Since then, several other approaches using 2 surgical techniques have been described.
Senning with arterial switch
- The principle of both the Senning and the Mustard operations is that the venous return into the heart is rerouted to the other ventricle.Thus, blood returning in the superior and inferior vena cava is directed via a baffle to the left-sided right ventricle, and the pulmonary venous return is directed to the right-sided ventricle.
- An operation such as this in combination with an arterial switch operation essentially ensures that the left ventricle becomes the systemic ventricle and the right one becomes the pulmonary ventricle. However, the left ventricle pressure must be near systemic for an arterial switch to be possible. This may occur if a large VSD is present or if a pulmonary artery band is applied to prepare the left ventricle by causing left ventricular hypertrophy. For the atrial switch, the Senning procedure is preferred because it uses less prosthetic material. With this operation, obstruction to the vena cava or the pulmonary venous return is a potential long-term concern. Also, this combination of surgeries is suitable only in the absence of pulmonic stenosis.
Senning and Rastelli (RV-to-PA conduit with VSD closure)
- When pulmonic stenosis is associated, an arterial switch is not suitable because the pulmonic stenosis would then become neoaortic stenosis. Instead, the left ventricle can be routed to the aorta by a baffle, closing the VSD in such a way that blood is directed from the left ventricle to the aorta and the right ventricle is connected to the pulmonary artery with a homograft.
- The VSD is optimally closed through a right ventriculotomy, which is required for the conduit placement. This ensures that the sutures for the VSD closure are on the right ventricular side and, hence, reduces risk of injury to the AV node.
One-and-a-half ventricle repair: This operation involves establishing a Glenn shunt for the superior vena cava return, a hemi Senning or Mustard to direct the inferior vena cava blood into the right ventricle, and either an arterial switch or a Rastelli, depending on the presence of pulmonic stenosis. This operation is especially useful in the presence of a small pulmonary ventricle. [33, 34] The following are some of the other advantages of this procedure: (1) no risk of superior vena cava obstruction is noted; (2) the intra-atrial space available for the pulmonary venous return is increased, thus, the risk of obstruction to the pulmonary venous return is reduced; (3) the intra-atrial suture lines are reduced, as is the risk of arrhythmias.
Complete heart block is a likely complication during surgery for ventricular septal defect (VSD) closure or relief of the pulmonic stenosis.
Outcome and Prognosis
The prognosis depends on the associated anatomic malformations and the conduction system abnormalities.
Conduction system deterioration: Complete heart block can develop over time at a rate of 2% per year. [12, 14] Surgical intervention also increases the risk of this condition because the conduction tissue is fragile and in an abnormal position.
Right ventricular function
- Because the right ventricle is morphologically different from the left ventricle and, in a person with a healthy heart, only sustains pressures that are about one fourth to one fifth that of the systemic circuit, whether this ventricle is able to sustain systemic pressures over a lifetime is an area of concern.
- Numerous pathways to ventricular dysfunction are possible for the systemic right ventricle in corrected transposition of the great arteries, such as volume overload from tricuspid regurgitation or a ventricular septal defect (VSD) and conduction and rhythm disturbances.
- Studies using equilibrium-gated radionuclide angiocardiography have shown that, although the right ventricular ejection volume increases from rest to peak exercise, it is less than that observed in a normal left ventricle. However, patients with systemic right ventricles who have corrected transposition of the great arteries are able to augment their cardiac output with an increase in both heart rate and stroke volume. This is in contradistinction to patients with D-transposition who have undergone a Senning-type operation and, thus, have a systemic right ventricle and can increase their cardiac output only with an increase in the heart rate.
- Right ventricular failure remains a concern; progressive tricuspid regurgitation may be indicative of this failure, and surgical treatment should be considered early when it is noted. Although the timing of tricuspid valve surgery beyond adolescence is still a matter of debate, mortality is low after tricuspid surgery in adult patients with mild-to-moderate right ventricular dysfunction, tricuspid valve function and functional class significantly improve after surgery, and systemic right ventricular function is preserved.
- In one retrospective review of 46 patients with congenitally corrected transposition who underwent tricuspid valve replacement for tricuspid regurgitation, the preoperative systemic ventricle ejection fraction (SVEF) was the only independent predictor of the postoperative SVEF after more than a year. The late SVEF was preserved (defined as ≥40%) in 63% of patients who underwent surgery with an SVEF ≥40% compared with 10.5% of patients who underwent surgery with an SVEF < 40%. Hence, the authors of this study suggested considering tricuspid valve replacement earlier while the SVEF is still above 40% and the subpulmonary ventricular pressure below 50 mm Hg.
- To mitigate long-term effects with respect to right ventricular failure, some surgeons have advocated the double-switch operation.
Prognosis after surgical correction of associated defects: The operative mortality rate for repair of associated defects is higher than that in patients with normal hearts. In a 1996 publication, Szufladowicz et al reported a 14% hospital mortality rate and a 10-year actuarial survival rate of 70%.  Factors contributing to this include the presence of complete heart block and the mirror image coronary artery pattern, which makes placement of the left ventricle to the pulmonary artery conduit technically challenging.  Another factor is the development of significant tricuspid regurgitation after surgical repair of the VSD, even when it is mild preoperatively. 
Studies comparing outcomes after physiologic and anatomic repairs are limited by their small size. However, the following 2 important studies have addressed this issue:
- The first is a metaanalysis of 11 studies involving 124 patients. In this study, only the immediate outcome (ie, in-hospital mortality) was compared between 2 types of anatomic repair (ie, atrial switch plus Rastelli and atrial switch plus arterial switch) and physiologic repair. The study suggests that a Rastelli anatomic repair has the lowest in-hospital mortality, the lowest incidence of postoperative heart block, and a lower incidence of postoperative AV valve regurgitation compared with physiologic repair. In another recent study, anatomic repair for congenitally corrected transposition of great arteries associated with left ventricular outflow tract obstruction and a VSD were excellent and an overall survival rate of 70% was reported at 20 years in 21 consecutive patients.
- The other large study (189 patients) found no statistical differences between long-term survival rates (follow up over 30 y for some patients) of patients who underwent conventional surgical repair versus those of patients who underwent anatomic surgical repair. The study reported that results of conventional repair were satisfactory, except in patients with significant tricuspid regurgitation. The recommendation was that anatomic repair should be the procedure of choice for those patients.
Future and Controversies
Although the surgical management of this condition is still evolving, a shift towards anatomic repair is clearly noted. In his review of the recent surgical literature for this condition, Bove combined reports from 4 studies and obtained an operative mortality rate of 8% (4 deaths in 50 operations) for patients undergoing either combined Senning or Mustard with arterial switch or Rastelli. He noted that associated heart block, ventricular dysfunction, and tricuspid valve regurgitation are all reduced when compared with traditional techniques.
Several centers have demonstrated excellent hospital and midterm survival after double-switch procedures.[45, 46, 47, 29] Langley et al have noted new left ventricular dysfunction and new aortic regurgitation in a few patients late after the double-switch procedure. Continued surveillance of these abnormalities is required.
Whether or not atrial arrhythmias are commonly encountered after the atrial switch operations remains to be determined. The long-term mortality rate after the double-switch operations is still unknown because these have only been proposed in approximately the last decade.
Lochte EH. Ein fall von situs viscerum irregularis, nebst einum beitrag zur lehre von der transposition der arterieller grosser gefasstamme des herzens. Beitrage Pathol Anat. 1898. 24:187-222.
Van Praagh R. What is congenitally corrected transposition?. N Engl J Med. 1970 May 7. 282(19):1097-8. [Medline].
Lundstrom U, Bull C, Wyse RK, Somerville J. The natural and "unnatural" history of congenitally corrected transposition. Am J Cardiol. 1990 May 15. 65(18):1222-9. [Medline].
Van Praagh R, Weinberg PM, Smith SD, et al. Malpositions of the heart. Adams FH, Emmanoulides GC, Riemenschneider TA, eds. Moss’ Heart Disease in Infants, Children and Adolescents. Baltimore, Md: Lippincott Williams & Wilkins; 1989: 530-80.
Van Praagh R, Papagiannis J, Grünenfelder J, Bartram U, Martanovic P. Pathologic anatomy of corrected transposition of the great arteries: medical and surgical implications. Am Heart J. 1998 May. 135(5 Pt 1):772-85. [Medline].
Bjarke BB, Kidd BS. Congenitally corrected transposition of the great arteries. A clinical study of 101 cases. Acta Paediatr Scand. 1976 Mar. 65(2):153-60. [Medline].
Fontana RS, Edwards JE. Frequency of occurence of various anatomic types of malformations among patients with congenital heart disease. Congenital Cardiac Disease-A Review of 357 cases studied Pathologically. 1962. 28-65.
Losekoot TG, Anderson RH, Becker AE, et al. Congenitally Corrected Transposition. Edinburgh: Churchill Livingstone; 1983.
Ellis K, Morgan BC, Blumenthal S, Anderson DH. Congenitally corrected transposition of the great vessels. Radiology. 1962. 79:35-50.
Allwork SP, Bentall HH, Becker AE, et al. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol. 1976 Dec. 38(7):910-23. [Medline].
Piacentini G, Digilio MC, Capolino R, et al. Familial recurrence of heart defects in subjects with congenitally corrected transposition of the great arteries. Am J Med Genet A. 2005. 137(2):176-180. [Medline].
Huhta JC, Danielson GK, Ritter DG, Illstrup DM. Survival in atrioventricular discordance. Pediatr Cardiol. 1985. 6(2):57-60. [Medline].
Daliento L, Corrado D, Buja G, et al. Rhythm and conduction disturbances in isolated, congenitally corrected transposition of the great arteries. Am J Cardiol. 1986 Aug 1. 58(3):314-8. [Medline].
Connelly MS, Liu PP, Williams WG, et al. Congenitally corrected transposition of the great arteries in the adult: functional status and complications. J Am Coll Cardiol. 1996 Apr. 27(5):1238-43. [Medline].
Presbitero P, Somerville J, Rabajoli F, et al. Corrected transposition of the great arteries without associated defects in adult patients: clinical profile and follow up. Br Heart J. 1995 Jul. 74(1):57-9. [Medline].
Graham TP, Bernard YD, Mellen BG, et al. Long-Term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000. 36(1):255-261. [Medline].
Rutledge JM, Nihill MR, Fraser CD, et al. Outcome of 121 patients with congenitally corrected transposition ofthe great arteries. Pediatr Cardiol. 2002 Mar-Apr. 23(2):137-145. [Medline].
Dimas AP, Moodie DS, Sterba R, Gill CC. Long-term function of the morphologic right ventricle in adult patients with corrected transposition of the great arteries. Am Heart J. 1989 Sep. 118(3):526-30. [Medline].
Ikeda U, Furuse M, Suzuki O, et al. Long-term survival in aged patients with corrected transposition of the great arteries. Chest. 1992 May. 101(5):1382-5. [Medline].
Mullins CE. The Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, Md: Lippincott Williams & Wilkins; 1998.
Bove EL. Congenital heart surgery. Curr Opin Cardiol. 1990 Feb. 5(1):45-51. [Medline].
de Leval MR, Bastos P, Stark J, et al. Surgical technique to reduce the risks of heart block following closure of ventricular septal defect in atrioventricular discordance. J Thorac Cardiovasc Surg. 1979 Oct. 78(4):515-26. [Medline].
Thiene G, Nava A, Rossi L. The conduction system in corrected transposition with situs inversus. Eur J Cardiol. 1977 Sep-Oct. 6(1):57-70. [Medline].
Shin'oka T, Kurosawa H, Imai Y, et al. Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: risk analyses in 189 patients. J Thorac Cardiovasc Surg. 2007 May. 133(5):1318-28, 1328.e1-4. [Medline].
Anderson RH, Becker AE. Coronary arterial patterns: a guide to identification of congenital heart disease. Paediatric Cardiology. Edinburgh: Churchill Livingstone; 1981. 251-62.
Sharland G, Tingay R, Jones A, Simpson J. Atrioventricular and ventriculoarterial discordance (congenitally corrected transposition of the great arteries): echocardiographic features, associations, and outcome in 34 fetuses. Heart. 2005 Nov. 91(11):1453-8. [Medline].
[Guideline] Gregoratos G, Cheitlin MD, Conill A, et al. ACC/AHA guidelines for implantation of cardiac pacemakers and antiarrhythmia devices: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Pacemaker Implantation). J Am Coll Cardiol. 1998 Apr. 31(5):1175-209. [Medline].
Fischbach PS. Congenitally corrected L-transposition of the great arteries: abnormalities of atrioventricular conduction. Prog Ped Cardiol. 1999. 10:37-43.
Devaney EJ, Charpie JR, Ohye RG, Bove EL. Combined arterial switch and Senning operation for congenitally corrected transposition of the great arteries: patient selection and intermediate results. J Thorac Cardiovasc Surg. 2003 Mar. 125(3):500-7. [Medline].
Russo P, Danielson GK, Driscoll DJ. Transaortic closure of ventricular septal defect in patients with corrected transposition with pulmonary stenosis or atresia. Circulation. 1987 Sep. 76(3 Pt 2):III88-92. [Medline].
Ilbawi MN, DeLeon SY, Backer CL, et al. 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 Sep. 100(3):410-5. [Medline].
Yamagishi M, Imai Y, Hoshino S, et al. Anatomic correction of atrioventricular discordance. J Thorac Cardiovasc Surg. 1993 Jun. 105(6):1067-76. [Medline].
Sojak V, Kuipers I, Koolbergen D, Rijlaarsdam M, Hruda J, Blom N, et al. Mid-term results of bidirectional cavopulmonary anastomosis and hemi-Mustard procedure in anatomical correction of congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg. 2012 Oct. 42(4):680-4. [Medline].
Mavroudis C, Backer CL, Kohr LM, et al. Bidirectional Glenn shunt in association with congenital heart repairs: the 1(1/2) ventricular repair. Ann Thorac Surg. 1999 Sep. 68(3):976-81; discussion 982. [Medline].
Benson LN, Burns R, Schwaiger M, et al. Radionuclide angiographic evaluation of ventricular function in isolated congenitally corrected transposition of the great arteries. Am J Cardiol. 1986 Aug 1. 58(3):319-24. [Medline].
Winter MM, van der Plas MN, Bouma BJ, Groenink M, Bresser P, Mulder BJ. Mechanisms for cardiac output augmentation in patients with a systemic right ventricle. Int J Cardiol. 2009 Feb 22. [Medline].
Warnes CA. Transposition of the great arteries. Circulation. 2006 Dec 12. 114(24):2699-709. [Medline].
Scherptong RW, Vliegen HW, Winter MM, et al. Tricuspid valve surgery in adults with a dysfunctional systemic right ventricle: repair or replace?. Circulation. 2009 Mar 24. 119(11):1467-72. [Medline].
Mongeon FP, Connolly HM, Dearani JA, Li Z, Warnes CA. Congenitally corrected transposition of the great arteries ventricular function at the time of systemic atrioventricular valve replacement predicts long-term ventricular function. J Am Coll Cardiol. 2011 May 17. 57(20):2008-17. [Medline].
Szufladowicz M, Horvath P, de Leval M, et al. Intracardiac repair of lesions associated with atrioventricular discordance. Eur J Cardiothorac Surg. 1996. 10(6):443-8. [Medline].
McKay R, Anderson RH, Smith A. The coronary arteries in hearts with discordant atrioventricular connections. J Thorac Cardiovasc Surg. 1996 May. 111(5):988-97. [Medline].
Westerman GR, Lang P, Castaneda AR, Norwood WI. Corrected transposition and repair of associated intracardiac defects. Circulation. 1982 Aug. 66(2 Pt 2):I197-202. [Medline].
Alghamdi AA, McCrindle BW, Van Arsdell GS. Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries: meta-analysis of individual patient data. Ann Thorac Surg. 2006 Apr. 81(4):1529-35. [Medline].
Hoashi T, Kagisaki K, Miyazaki A, Kurosaki K, Shiraishi I, Yagihara T, et al. Anatomic repair for corrected transposition with left ventricular outflow tract obstruction. Ann Thorac Surg. 2013 Aug. 96(2):611-20. [Medline].
Duncan BW, Mee RB, Mesia CI, et al. Results of the double switch operation for congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg. 2003 Jul. 24(1):11-9; discussion 19-20. [Medline].
Langley SM, Winlaw DS, Stumper O, et al. 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 Jun. 125(6):1229-41. [Medline].
Imamura M, Drummond-Webb JJ, Murphy DJ, et al. Results of the double switch operation in the current era. Ann Thorac Surg. 2000 Jul. 70(1):100-5. [Medline].
Mandell AJ, Russo PV, Blomgren BW. Geometric universality in brain allosteric protein dynamics: complex hydrophobic transformation predicts mutual recognition by polypeptides and proteins. Ann N Y Acad Sci. 1987. 504:88-117. [Medline].