title: Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study.
abstract: OBJECTIVE
  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.
authors.affiliation: Vanderbilt University Medical Center, Nashville, Tennessee 37232-2572, USA. tom.graham@mc.mail.vanderbilt.edu
authors.forename: T P
authors.initials: TP
authors.lastname: Graham
authors.forename: Y D
authors.initials: YD
authors.lastname: Bernard
authors.forename: B G
authors.initials: BG
authors.lastname: Mellen
authors.forename: D
authors.initials: D
authors.lastname: Celermajer
authors.forename: H
authors.initials: H
authors.lastname: Baumgartner
authors.forename: F
authors.initials: F
authors.lastname: Cetta
authors.forename: H M
authors.initials: HM
authors.lastname: Connolly
authors.forename: W R
authors.initials: WR
authors.lastname: Davidson
authors.forename: M
authors.initials: M
authors.lastname: Dellborg
authors.forename: E
authors.initials: E
authors.lastname: Foster
authors.forename: W M
authors.initials: WM
authors.lastname: Gersony
authors.forename: I H
authors.initials: IH
authors.lastname: Gessner
authors.forename: R A
authors.initials: RA
authors.lastname: Hurwitz
authors.forename: H
authors.initials: H
authors.lastname: Kaemmerer
authors.forename: J D
authors.initials: JD
authors.lastname: Kugler
authors.forename: D J
authors.initials: DJ
authors.lastname: Murphy
authors.forename: J A
authors.initials: JA
authors.lastname: Noonan
authors.forename: C
authors.initials: C
authors.lastname: Morris
authors.forename: J K
authors.initials: JK
authors.lastname: Perloff
authors.forename: S P
authors.initials: SP
authors.lastname: Sanders
authors.forename: J L
authors.initials: JL
authors.lastname: Sutherland
mesh_terms: D000328:Adult; D000367:Age Factors; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D015994:Incidence; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012737:Sex Factors; D014188:Transposition of Great Vessels / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D018754:Ventricular Dysfunction / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology