Ann Thorac Surg 2007;83:672-674
© 2007 The Society of Thoracic Surgeons
Case Reports
Congenitally Corrected Transposition of the Great Arteries: Surgical Repair in Adulthood
Fotios A. Mitropoulos, MD, PhDa,b,*,
Meletios Kanakis, MDa,
Antonios P. Vlachos, MDb,
Paraskevi Lathridou, MDb,
George Tsaoussis, MDc,
George Georgiou, MDc,
John A. Goudevenos, MD, PhDb
a National and Kapodistrian University of Athens School of Medicine, Athens, Greece
b Department of Cardiology, University of Ioannina School of Medicine, Athens, Greece
c Department of Pediatric Cardiology, Medical Center of Athens, Athens, Greece
Accepted for publication July 10, 2006.
* Address correspondence to Dr Mitropoulos, National and Kapodistrian University of Athens School of Medicine, Monastiriou & Thracomacedones, Athens 13676 Greece. (Email: fotiosmitropoulos{at}yahoo.com).
Presented at the Congenital Cardiac Case Report Breakfast Session of the Forty-second Annual Meeting of The Society of Thoracic Surgeons, Chicago, IL, Jan 31, 2006.
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Abstract
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We report a patient with congenitally corrected transposition of the great arteries, dextrocardia in congestive heart failure, with severe tricuspid regurgitation; a large ventricular septal defect, and giant left atrium. The patient underwent a two-ventricle repair with physiologic correction and had an excellent postoperative outcome.
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Introduction
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Congenitally corrected transposition of the great arteries (CCTGA) is a rare complex cardiac anomaly with a wide range of morphologic characteristics [1]. The main underlying disorder is atrioventricular and ventriculoarterial discordance. The morphologic right ventricle functions as the systemic ventricle, and the morphologic left ventricle functions as the pulmonary ventricle. The atrioventricular valve connected to the systemic ventricle is morphologically tricuspid, and the valve connected to the pulmonary ventricle is morphologically mitral. Although in some patients the morphologic right ventricle retains normal function even in the late adulthood, some dysfunction of the systemic ventricle occurs progressively with age [2]. Various surgical approaches have been proposed to treat this anomaly. We report a patient with CCTGA who underwent a two-ventricle repair with physiologic correction with an excellent postoperative outcome.
A 36-year-old man with dextrocardia, CCTGA situs solitus (Van Praagh, S, L, L), with ventricular septal defect (VSD), and complete heart block, and who was in New York Heart Association class II congestive heart failure, developed severe (4+) systemic atrioventricular tricuspid regurgitation (TR), mild (2+) pulmonary atrioventricular mitral regurgitation (MR), moderate pulmonary hypertension (55/25/37 mm Hg), and cardiomegaly. His medical history included palpitations and paroxysmal atrial fibrillation.
On physical examination, there was a visible heart impulse giving the picture of a dancing thorax. The apex of the heart was at the right mid-axillary line. A chest roentgenogram showed massive cardiomegaly (Fig 1). Transthoracic echocardiography revealed severe TR (4+), mild MR (2+), a large VSD and severe dilation of the systemic ventricle and left atrium (107 mm), with an ejection fraction (EF) of 0.66. Cardiac magnetic resonance imaging confirmed these findings (Fig 2).
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Fig 2. Preoperative cardiac magnetic resonance image (sagittal view) shows the giant left atrium (LA), the enlarged systemic ventricle (SV), and the ascending aorta (AAo).
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The operation was performed with hypothermic cardiopulmonary bypass. A large VSD and a dysplastic Ebstein-like tricuspid valve with a giant left atrium were identified. The VSD was closed with Gore-Tex patch (W. L. Gore & Associates, Flagstaff, Ariz), and the valve was replaced with an ATS 33-mm mechanical valve (ATS Medical, Minneapolis, Minn), with preservation of the subvalvular apparatus (cords and pappilary muscle attachments). The left atrial appendage was ligated, and the left atrium was reduced by about 5.5 cm. A modified left-sided Maze procedure (box lesion around the pulmonary veins with radiofrequency ablation) was performed owing to his history of paroxysmal atrial fibrillation. Permanent epicardial pacing wires were also implanted. After weaning from the cardiopulmonary bypass, the pressure of the left atrium was 15 mm Hg.
The patient was extubated in 24 hours and transferred to the floor, where his postoperative course was uneventful. He was discharged in 7 days. His immediate postoperative echocardiogram revealed no TR, no VSD leak, and (1+) mild MR. A chest roentgenogram showed a significant decrease in the cardiothoracic index (Fig 3). An echocardiogram at 6 months showed a significant reduction of the systemic ventricle size and almost no MR. The patient remains in sinus rhythm.
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Fig 3. Postoperative chest roentgenogram shows a significant decrease of the cardiothoracic index (ratio: 0.56). Epicardial pacing wires are also present.
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Comment
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CCTGA can be categorized in two main groups: CCTGA with visceroatrial situs solitus (most common), and CCTGA with situs inversus [1]. This congenital heart disease may be first diagnosed in adulthood or even in the elderly patient. It can also be misdiagnosed as simple dextrocardia [3]. The clinical presentation is usually secondary to TR, ventricular failure, or conduction abnormalities. The most common hemodynamic abnormality is TR in the setting of impaired systemic ventricle function [3].
The tricuspid valve is morphologically abnormal, with an Ebstein-like anatomy and with short, thickened chordae tendinae and cusps [1, 3–5]. Prieto and colleagues [5] reported that patients with a morphologically abnormal tricuspid valve identified early in life are at increased risk for developing TR. They also stated that severe TR was the only independent factor of long-term survival in CCTGA patients both with and without surgical correction [5].
Previous cardiac operation, age, and preoperative heart block have been implicated as risk factors for the development of TR [4]. TR after spontaneous complete heart block has been observed more frequently in situs solitus than in situs inversus [4]. The usual abnormal course of the conduction system predisposes it to fibrosis with resulting complete heart block [1, 4]. It has been assumed that the right ventricle is not suited to function as a long-term systemic pump, although occasional cases of elderly patients in their 70s and 80s with essentially normal function have been reported. This has been the case with CCTGA without any associated lesions [3].
Interestingly, Hornung and colleagues [6] described a correlation between the extent of perfusion defects and degree of ventricular dysfunction. Myocardial perfusion defects suggest that abnormal perfusion plays a major role in the development of progressive systemic ventricle dysfunction [6]. A possible interpretation is that a right coronary artery system has limited abilities to provide adequate flow to a hypertrophied systemic right ventricle [2]. It is unclear if TR results in systemic ventricle dysfunction or vice versa [2]; however, evidence suggests that in the absence of associated cardiac deformities, primary systemic ventricle failure is uncommon and is primarily a consequence of TR [5].
When to replace or not replace the tricuspid valve in the setting of systemic ventricle dysfunction is a key question. Van Son and colleagues [4] showed that replacement of the tricuspid valve when the EF of the systemic ventricle is lower than 0.44 was associated with poor early and late outcome. Current guidelines have suggested tricuspid valve replacement when TR is moderately severe or severe, especially if signs or symptoms of ventricular dysfunction are present. Limitation in exercise capacity has been suggested as another factor to determine the optimal timing of tricuspid valve replacement [3].
Operation should be considered at the earliest signs of progression of symptoms or evidence of progressive systemic ventricle deterioration, such as an enlarging ventricle and left atrium, development of pulmonary hypertension, and appearance of atrial arrhythmias. The atypical morphologic features of tricuspid valve make its repair not a long-lasting solution (4).
For these reasons and the available clinical data, we proceeded with a physiologic correction using a mechanical valve for the TR. Although the "double" switch operation has been proposed as a more anatomic correction, it is a more technically challenging procedure and is associated with a significant mortality rate, even in experienced hands (17%), and with significant long-term problems such as baffle obstruction, atrial arrhythmias, and sick sinus syndrome [2, 5, 7]. A modified Fontan procedure has also been proposed as an alternative surgical option in patients not suitable for biventricular repair, but the experience with this approach is limited [8]. Heart transplantation remains the final destination for patients with intractable TR and severe systemic ventricle dysfunction [5].
The type and the timing for the surgical intervention in the adult patient with CCTGA remains a challenging problem. Routine follow-up of patients with CCTGA who have not had a surgical correction is warranted. A patient in whom significant TR develops should be considered for tricuspid valve replacement, especially in the setting of systemic ventricle dysfunction.
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References
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- 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;35:772-785.
- Graham Jr TP, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries J Am Coll Cardiol 2000;36:255-261.[Abstract/Free Full Text]
- Beauchesne LM, Warnes CA, Connoly HM, Ammash NM, Tajik AJ, Danielson GK. Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries J Am Coll Cardiol 2002;40:285-290.[Abstract/Free Full Text]
- Van Son JA, Danielson GK, Huhta JC, et al. Late results of systemic atrioventricular valve replacement in corrected transposition J Thorac Cardiovasc Surg 1995;109:642-653.[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]
- Hornung TS, Bernard EJ, Celermajer DS, et al. Right ventricular dysfunction in congenitally corrected transposition of the great arteries Am J Cardiol 1999;84:1116-1119.[Medline]
- Poirier NC, Yu JH, Brizard CP, Mee RB. 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.[Abstract/Free Full Text]
- Rutledge JM, Nihill MR, Fraser CD, Smith OE, McMahon CJ, Bezold LI. Outcome of 121 patients with congenitally corrected transposition of the great arteries Pediatr Cardiol 2002;23:137-145.[Medline]
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Abstract
Introduction
