CLINICAL STUDIES: PEDIATRIC CARDIOLOGY
Long-term outcome in congenitally corrected transposition of the great arteries
A multi-institutional study
Thomas P. Graham, Jr., MD, FACC*,
Yvonne D. Bernard, RN*,
Beverly G. Mellen, PhD*,
David Celermajer, MD ,
Helmut Baumgartner, MD ,
Frank Cetta, MD, FACC ,
Heidi M. Connolly, MD, FACC||,
William R. Davidson, MD, FACC¶,
Mikael Dellborg, MD#,
Elyse Foster, MD, FACC**,
Welton M. Gersony, MD, FACC,
Ira H. Gessner, MD, FACC,
Roger A. Hurwitz, MD, FACC,
Harald Kaemmerer, MD||,
John D. Kugler, MD, FACC¶¶,
Daniel J. Murphy, MD, FACC##,
Jacqueline A. Noonan, MD, FACC***,
Cynthia Morris, MD,
Joseph K. Perloff, MD, FACC,
Stephen P. Sanders, MD, FACC and
James L. Sutherland, MD, FACC||||
* Vanderbilt University Medical Center, Nashville, Tennessee, USA
Royal Prince Alfred Hospital, Sydney, Australia
Vienna General Hospital, Vienna, Austria
Loyola University, Chicago, Illinois, USA
|| Mayo Clinic, Rochester, Minnesota, USA
¶ Hershey Medical Center, Hershey, Pennsylvania, USA
# Sahlgrenska University/Ostra Hospital, Goteborg, Sweden
** University of California, San Francisco, California, USA
Babies Hospital, New York, New York, USA
University of Florida, Gainesville, Florida, USA
Indiana University, Indianapolis, Indiana, USA
|| Hannover Medical School, Hannover, Germany
¶¶ University of Nebraska, Omaha, Nebraska, USA
## Cleveland Clinic, Cleveland, Ohio, USA
*** University of Kentucky, Lexington, Kentucky, USA
University of Oregon, Portland, Oregon, USA
University of California, Los Angeles, California, USA
Duke University, Durham, North Carolina, USA
|||| Children’s Heart Center, Atlanta, Georgia, USA
Manuscript received July 6, 1999;
revised manuscript received January 17, 2000,
accepted March 2, 2000.
Reprint requests and correspondence: Dr. Thomas P. Graham, Jr., Pediatric Cardiology, Vanderbilt Medical Center, D2212 MCN, Nashville, Tennessee 37232-2572
tom.graham{at}mc.mail.vanderbilt.edu
OBJECTIVES
The purpose of this study was to determine long-term outcome in adults with congenitally corrected transposition of the great arteries (CCTGA), with particular emphasis on systemic ventricular dysfunction and congestive heart failure (CHF).
BACKGROUND
Patients with CCTGA have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and CHF being relatively common in older adults.
METHODS
Retrospective analysis of records of 182 patients from 19 institutions were reviewed to determine current status and possible risk factors for systemic ventricular dysfunction and CHF. Factors considered included age, gender, associated cardiac defects, operative history, heart block, arrhythmias and tricuspid (i.e., systemic atrioventricular) regurgitation (TR).
RESULTS
Both CHF and systemic ventricular dysfunction were common in groups with or without associated cardiac lesions. By age 45, 67% of patients with associated lesions had CHF, and 25% of patients without associated lesions had this complication. The rates of systemic ventricular dysfunction and CHF were higher with increasing age, the presence of significant associated cardiac lesions, history of arrhythmia, pacemaker implantation, prior surgery of any type, and particularly with tricuspid valvuloplasty or replacement. Aortic regurgitation (a previously unreported problem) was also relatively common in this patient population.
CONCLUSIONS
Patients with CCTGA are increasingly subject to CHF with advancing age; this complication is extremely common by the fourth and fifth decades. Tricuspid (systemic atrioventricular) valvular regurgitation is strongly associated with RV (anatomical right ventricle connected to aorta in CCTGA patients; systemic ventricle in CCTGA) dysfunction and CHF; whether it is causative or a secondary complication remains speculative.
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Abbreviations and Acronyms
| | AR | = aortic regurgitation | | CCTGA | = congenitally corrected transposition of the great arteries | | CHF | = congestive heart failure | | LA | = left atrium | | LV | = anatomical left ventricle connected to pulmonary artery in CCTGA patients | | PS | = pulmonary stenosis | | RV | = anatomical right ventricle connected to aorta in CCTGA patients systemic ventricle in CCTGA | | TR | = tricuspid regurgitation (right ventricular/atrioventricular regurgitation) | | VSD | = ventricular septal defect |
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|
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R. Sharma, S. Talwar, A. Marwah, S. Shah, S. Maheshwari, P. Suresh, R. Garg, B. S. Bali, R. Juneja, A. Saxena, et al.
Anatomic repair for congenitally corrected transposition of the great arteries.
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137(2):
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January 1, 2009;
2(1):
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52(23):
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[PDF]
|
|
|
|
|
|
|
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118(23):
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|
|
|
|
|
|
|
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A case of congenital cardiomyopathy with acute heart failure
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10(8):
811 - 813.
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|
|
|
|
|
|
|
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August 1, 2008;
59(2_suppl):
93S - 96S.
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[PDF]
|
|
|
|
|
|
|
A. M. Gaca, J. J. Jaggers, L. T. Dudley, and G. S. Bisset III
Repair of Congenital Heart Disease: A Primer-Part 1
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June 1, 2008;
247(3):
617 - 631.
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|
|
|
|
|
|
|
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J. Am. Coll. Cardiol.,
May 27, 2008;
51(21):
e1 - e62.
[Full Text]
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|
|
|
|
|
|
|
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May 27, 2008;
117(21):
e350 - e408.
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|
|
|
|
|
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Circulation,
March 11, 2008;
117(10):
1340 - 1350.
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|
|
|
|
|
|
|
E. Bedard, D. F. Shore, and M. A. Gatzoulis
Adult congenital heart disease: a 2008 overview
Br. Med. Bull.,
March 1, 2008;
85(1):
151 - 180.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Shin'oka, H. Kurosawa, Y. Imai, M. Aoki, M. Ishiyama, T. Sakamoto, S. Miyamoto, K. Hobo, and Y. Ichihara
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
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133(5):
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|
|
|
|
|
|
|
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May 1, 2007;
188(5):
W428 - W430.
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|
|
|
|
|
|
|
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|
|
|
|
|
|
|
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83(2):
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|
|
|
|
|
|
|
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December 12, 2006;
114(24):
2699 - 2709.
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|
|
|
|
|
|
|
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December 1, 2006;
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|
|
|
|
|
|
|
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J. Am. Coll. Cardiol.,
September 5, 2006;
48(5):
1064 - 1108.
[Full Text]
[PDF]
|
|
|
|
|
|
|
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J. Am. Coll. Cardiol.,
September 5, 2006;
48(5):
e247 - e346.
[Full Text]
[PDF]
|
|
|
|
|
|
|
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September 1, 2006;
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2099 - 2140.
[Full Text]
[PDF]
|
|
|
|
|
|
|
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September 1, 2006;
8(9):
746 - 837.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. A. Alghamdi, B. W. McCrindle, and G. S. Van Arsdell
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Ann. Thorac. Surg.,
April 1, 2006;
81(4):
1529 - 1535.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G B Bleeker, P Steendijk, E R Holman, C-M Yu, O A Breithardt, T A M Kaandorp, M J Schalij, E E van der Wall, P Nihoyannopoulos, and J J Bax
Assessing right ventricular function: the role of echocardiography and complementary technologies
Heart,
April 1, 2006;
92(suppl_1):
i19 - i26.
[Full Text]
[PDF]
|
|
|
|
|
|
|
P A Davlouros, K Niwa, G Webb, and M A Gatzoulis
The right ventricle in congenital heart disease
Heart,
April 1, 2006;
92(suppl_1):
i27 - i38.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. G. Williams, G. D. Pearson, R. J. Barst, J. S. Child, P. del Nido, W. M. Gersony, K. S. Kuehl, M. J. Landzberg, M. Myerson, S. R. Neish, et al.
Report of the National Heart, Lung, and Blood Institute Working Group on Research in Adult Congenital Heart Disease
J. Am. Coll. Cardiol.,
February 21, 2006;
47(4):
701 - 707.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G Sharland, R Tingay, A Jones, and J Simpson
Atrioventricular and ventriculoarterial discordance (congenitally corrected transposition of the great arteries): echocardiographic features, associations, and outcome in 34 fetuses
Heart,
November 1, 2005;
91(11):
1453 - 1458.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. A. Warnes
The Adult With Congenital Heart Disease: Born To Be Bad?
J. Am. Coll. Cardiol.,
July 5, 2005;
46(1):
1 - 8.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. S. Winlaw, S. P. McGuirk, C. Balmer, S. M. Langley, M. Griselli, O. Stumper, J. V. De Giovanni, J. G. Wright, S. Thorne, D. J. Barron, et al.
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,
February 1, 2005;
111(4):
405 - 411.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
O. Salehian, M. Schwerzmann, N. Merchant, G. D. Webb, S. C. Siu, and J. Therrien
Assessment of Systemic Right Ventricular Function in Patients With Transposition of the Great Arteries Using the Myocardial Performance Index: Comparison With Cardiac Magnetic Resonance Imaging
Circulation,
November 16, 2004;
110(20):
3229 - 3233.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Derrick and J. E. Deanfield
Decline in ventricular function and clinical condition after Mustard repair
Eur. Heart J.,
November 1, 2004;
25(21):
1863 - 1864.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Aeba, T. Katogi, K. Koizumi, Y. Iino, M. Mori, and R. Yozu
Apico-pulmonary artery conduit repair of congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary outflow tract obstruction: A 10-year follow-up
Ann. Thorac. Surg.,
November 1, 2003;
76(5):
1383 - 1388.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. W. Duncan, R. B.B. Mee, C. I. Mesia, A. Qureshi, G. L. Rosenthal, S. G. Seshadri, G. K. Lane, and L. A. Latson
Results of the double switch operation for congenitally corrected transposition of the great arteries
Eur. J. Cardiothorac. Surg.,
July 1, 2003;
24(1):
11 - 20.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. J. Devaney, J. R. Charpie, R. G. Ohye, and E. L. Bove
Combined arterial switch and Senning operation for congenitally corrected transposition of the great arteries: Patient selection and intermediate results
J. Thorac. Cardiovasc. Surg.,
March 1, 2003;
125(3):
500 - 507.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. M. Beauchesne, C. A. Warnes, H. M. Connolly, N. M. Ammash, A. J. Tajik, and G. K. Danielson
Outcome of the unoperated adult who presents with congenitallycorrected transposition of the great arteries
J. Am. Coll. Cardiol.,
July 17, 2002;
40(2):
285 - 290.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. N. Ilbawi, C. B. Ocampo, B. S. Allen, M. J. Barth, D. A. Roberson, P. Chiemmongkoltip, and R. A. Arcilla
Intermediate results of the anatomic repair for congenitally corrected transposition
Ann. Thorac. Surg.,
February 1, 2002;
73(2):
594 - 600.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
