TY - JOUR
T1 - Long-term outcome in congenitally corrected transposition of the great arteries
AU - Graham, Thomas P.
AU - Bernard, Yvonne D.
AU - Mellen, Beverly G.
AU - Celermajer, David
AU - Baumgartner, Helmut
AU - Cetta, Frank
AU - Connolly, Heidi M.
AU - Davidson, William R.
AU - Dellborg, Mikael
AU - Foster, Elyse
AU - Gersony, Welton M.
AU - Gessner, Ira H.
AU - Hurwitz, Roger A.
AU - Kaemmerer, Harald
AU - Kugler, John D.
AU - Murphy, Daniel J.
AU - Noonan, Jacqueline A.
AU - Morris, Cynthia
AU - Perloff, Joseph K.
AU - Sanders, Stephen P.
AU - Sutherland, James L.
N1 - Funding Information:
Support for this study was provided by the Pediatric Cardiology Development Fund, Vanderbilt University Medical Center.
PY - 2000/7
Y1 - 2000/7
N2 - 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. (C) 2000 by the American College of Cardiology.
AB - 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. (C) 2000 by the American College of Cardiology.
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U2 - 10.1016/S0735-1097(00)00682-3
DO - 10.1016/S0735-1097(00)00682-3
M3 - Article
C2 - 10898443
AN - SCOPUS:17444452828
SN - 0735-1097
VL - 36
SP - 255
EP - 261
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 1
ER -