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From the Division of Pediatric Cardiology, Department of Pediatrics,
College of Physicians and Surgeons of Columbia University, New York, NY
(L.R.P., A.J.H., M.S.R., W.M.G.), and the Department of Biostatistics and
Epidemiology, Cleveland Clinic Foundation, Cleveland, Ohio (M.S.).
Correspondence to Lourdes R. Prieto, MD, The Cleveland Clinic Foundation, Department of Pediatric Cardiology/M41, 9500 Euclid Ave, Cleveland, OH 44195. E-mail prietol{at}cesmtp.ccf.org
Background—The outcome of patients
with corrected transposition of the great arteries (CTGA) is variably
affected by associated intracardiac defects, tricuspid valve
competence, and systemic right ventricular (RV) function.
The relative importance of these factors in long-term outcome has not
been evaluated.
Methods and Results—Since 1958, 40 patients with CTGA were
studied to determine risk factors for poor outcome, including age, open
heart surgery, tricuspid insufficiency (TI), cardiac rhythm,
pulmonary overcirculation, and RV dysfunction. Follow-up ranged
from 7 to 36 years (mean 20 years). Intracardiac repair was performed
in 21 patients; 19 were unoperated or had closed heart procedures. For
the purposes of this study the designation TIs refers to at
least moderately severe TI as delineated by echocardiogram and/or
angiography. TIs was the only independently significant
factor for death (P=0.01), and in turn, only the
presence of a morphologically abnormal TV predicted TIs
(P=0.03). Twenty-year survival without TIs
was 93%, but only 49% with TIs. Poor long-term
postoperative outcome was due to TIs in all but 1 patient;
20-year survival rates for operated patients with and without
TIs were 34% and 90%, respectively
(P=0.002). Similarly, 20-year survival rates for
unoperated patients with and without TIs were 60% and
100%, respectively, whether or not attempts to repair the TI were made
(P=0.08).
Conclusions—TIs represents the major risk
factor for CTGA patients; RV dysfunction appears to be almost always
secondary to long-standing TI. Decisions related to surgical
interventions with or without associated lesions must be strongly
influenced by the status of the tricuspid valve.
© 1998 American Heart Association, Inc.
Clinical Investigation and Reports
Progressive Tricuspid Valve Disease in Patients With Congenitally Corrected Transposition of the Great Arteries
Key Words: transposition of great vessels • valves • survival • pediatrics
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