Ann Thorac Surg 2008;85:e1-e2. doi:10.1016/j.athoracsur.2007.09.009
© 2008 The Society of Thoracic Surgeons
Case Reports
Successful Neonatal Double Switch in Symptomatic Patients With Congenitally Corrected Transposition of the Great Arteries
Victor Bautista-Hernandez, MDa,*,
Felix Serrano, MD, PhDa,
Jose M. Palacios, MDb,
Jose M. Caffarena, MD, PhDa
a Department of Cardiovascular Surgery, University Hospital La Fe, Valencia, Spain
b Department of Pediatric Cardiology, University Hospital La Fe, Valencia, Spain
Accepted for publication September 6, 2007.
* Address correspondence to Dr Bautista-Hernandez, Department of Cardiovascular Surgery, University Hospital La Fe, Av Campanar 21, Valencia, 46009, Spain (Email: vbautista_hernandez{at}hotmail.com).
 |
Abstract
|
|---|
Anatomic repair is the standard surgical approach to congenitally corrected transposition of the great arteries. However, timing to perform the procedure remains controversial. We present 2 cases of congenitally corrected transposition of the great arteries and Ebstein’s-like anomaly of the tricuspid valve presenting with heart failure. Both cases had successful anatomic repair during the neonatal period.
|
Introduction
|
|---|
Congenitally corrected transposition of the great arteries (ccTGA) is a rare and complex cardiac defect characterized by discordant connections between the atria and ventricles and between the ventricles and the great arteries. Surgical approaches for ccTGA include physiologic and anatomic repair. However, in patients undergoing a physiologic procedure, the morphologic RV remains supporting the systemic circulation with disappointing late results [1]. Anatomic repair or double switch represents the standard surgical management for ccTGA. Advantages in terms of improvement of right ventricle (RV) and tricuspid function combined with low operative morbidity and mortality, and the fact that the left ventricle (LV) becomes the systemic ventricle, have resulted in the application of this procedure to almost all patients with ccTGA, despite its complexity [2]. Nevertheless, adequate timing for repair is not well established. Reports of neonatal repair of ccTGA are extremely rare. We describe 2 patients with ccTGA presenting with heart failure and undergoing a double-switch procedure during the neonatal period.
Two full-term males presented with symptoms of heart failure. Echocardiography in patient 1 demonstrated ccTGA (S, L, L) with associated aortic coarctation, an inlet ventricular septal defect. Patient 2 had a ccTGA (S,L,L) with no ventricular septal defect. Both patients had an Ebstein’s-like anomaly of the tricuspid valve (Fig 1A) with significant tricuspid regurgitation (TR [Fig 1B]) and RV dysfunction. Patient 1 had coarctectomy with end-to-end anastomosis. However, no improvement in ventricular function was noted postoperatively. Furthermore, TR and RV function worsened. In both cases, we proceeded with a double-switch operation at the 21st and 15th day of life, respectively. A Mustard procedure was done at the atrial level, while an arterial switch was used to restore the ventricle-arterial concordance (Fig 2A). Patient 1 required closure of the ventricular septal defect. The postoperative course was uneventful, and both patients were discharged home in good condition. At a follow-up of 12 and 10 months, respectively, our patients are asymptomatic with no significant TR or RV dysfunction (Fig 2B). No interventricular conduction delay or LV dysfunction has been noted.
View larger version (42K):
[in this window]
[in a new window]
|
Fig 1. Preoperative echocardiographic assessment of congenitally corrected transposition of the great arteries in patient 1. (A) Note the existence of an Ebstein’s-like anomaly of the tricuspid valve, producing (B) significant tricuspid regurgitation.
|
|
View larger version (43K):
[in this window]
[in a new window]
|
Fig 2. Postoperative echocardiographic assessment of congenitally corrected transposition of the great arteries in patient 1. (A) Note the Mustard procedure utilized to restore the atrioventricular concordance, and (B) the absence of tricuspid regurgitation.
|
|
|
Comment
|
|---|
The double-switch operation is the preferred procedure for patients with ccTGA following the biventricular pathway. However, no standard timing for operation is widely accepted. The most common surgical approach entails performance of surgery at some point around the first year of life. At this point, the anatomic structures are not as delicate as during the neonatal period, and significant ventricular hypertrophy has not yet developed. In fact, patients with ccTGA rarely manifest symptoms requiring correction during infancy, so repair may frequently be delayed beyond the first few months, and occasionally years, of age. Even in cases presenting with cyanosis or congestive heart failure during the neonatal period, a palliative systemic-to-pulmonary shunt or pulmonary artery banding may be performed and the double switch delayed. However, palliative procedures are not innocuous, and significant morbidity and mortality has been reported. In fact, the performance of pulmonary artery banding in patients with pulmonary overcirculation has been related to aortic root dilation and neoaortic regurgitation late after the arterial switch procedure [3]. Moreover, TR and RV dysfunction can appear spontaneously anytime during the natural history of ccTGA, and TR is a potent risk factor for death [4]. Furthermore, TR develops a vicious circle with increasing volume load and further dilation of the annulus, damaging the valvar and subvalvar apparatus. This situation is of special concern in the setting of ccTGA as repair of the regurgitant tricuspid valve is rarely successful, and valve replacement is most often required.
The pathophysiologic mechanism for our patients was an Ebstein’s-like anomaly of the tricuspid valve producing significant (moderate-severe) TR and subsequently, RV dysfunction. In addition, patient 1 had an aortic coarctation exacerbating the symptoms. Despite neonatal age, the double switch was the procedure of choice as it consistently improves TR and RV function in patients with ccTGA.
Development of LV dysfunction, arrythmias, and aortic insufficiency has been reported late after anatomic repair of ccTGA. Recent studies relate worsening in LV function to electrocardiogram abnormalities and standard dual-chamber pacemaker implantation [5]. In addition, retraining of the LV is frequently indicated in patients when a double switch for ccTGA is undertaken late in infancy [5]. Interestingly, no high-grade heart block, LV dysfunction, or other complications have developed in either of our patients.
In conclusion, anatomic repair of ccTGA can be accomplished with excellent results during the neonatal period. The double switch is advocated for symptomatic patients, especially those with significant TR or RV dysfunction, or both. With increasing surgical experience, the neonatal approach could be expanded to all patients with ccTGA undergoing an anatomic repair.
|
References
|
|---|
- 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;81:1529-1535.[Abstract/Free Full Text]
- Bautista-Hernandez V, del Nido PJ. Congenitally corrected transposition of the great arteriesIn: Kaiser LR, Krong IL, Spray TL, editors. Mastery of cardiothoracic surgery. 2nd ed.. Philadelphia, PA: Lippincott Williams and Wilkins; 2006. pp. 873-880.
- Schwartz ML, Gauvreau K, del Nido P, et al. Long-term predictors of aortic root dilatation and aortic regurgitation after arterial switch operation Circulation 2004;110(Suppl 1):128-132.[Abstract/Free Full Text]
- Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries Circulation 1998;98:997-1005.[Abstract/Free Full Text]
- Bautista-Hernandez V, Marx GR, Gauvreau K, Mayer JE, Cecchin F, del Nido PJ. Determinants of Left Ventricular Dysfunction After Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries Ann Thorac Surg 2006;82:2059-2065.[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
M. Ly, E. Belli, B. Leobon, C. Kortas, O. E. Grollmuss, D. Piot, C. Planche, and A. Serraf
Results of the double switch operation for congenitally corrected transposition of the great arteries
Eur. J. Cardiothorac. Surg.,
May 1, 2009;
35(5):
879 - 884.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
