Congenitally Corrected Transposition of the Great Arteries and Situs Inversus in an Octogenarian With Systemic Right Ventricular Failure.
Systemic right ventricular (RV) failure in patients with congenitally corrected transposition of the great arteries (ccTGA), a major cause of mortality in the long-term follow-up, is usually induced by concomitant severe morphologically tricuspid regurgitation (TR) with/without Ebstein's anomaly or progressive conduction tissue disturbances. However, whether or not myocardial fibrosis is a common cause of systemic RV failure in patients with ccTGA remains unclear. Here, we describe an 82-year-old man who had been diagnosed previously as having uncomplicated ccTGA and situs inversus and recently developed systemic RV failure, which was neither associated with severe TR nor advanced conduction tissue abnormalities. Cardiovascular magnetic resonance (CMR) with delayed-enhancement imaging clearly detected extensive myocardial scars (presumably fibrosis) in the RV wall as well as prominent dilatation, hypertrophy, and systolic dysfunction of the systemic RV. These findings suggest that myocardial fibrosis can cause systemic RV failure in elderly patients with uncomplicated ccTGA despite the absence of severe TR or advanced conduction tissue abnormalities and that CMR may be a useful examination to accurately detect systemic RV failure associated with myocardial fibrosis and to subsequently clarify the prognosis in these patients.
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center.
Mikio
M
Shiba
Shigefumi
S
Fukui
Hideo
H
Ohuchi
Jin
J
Ueda
Akihiro
A
Tsuji
Yoshiaki
Y
Morita
Aya
A
Miyazaki
Takeshi
T
Ogo
Satoshi
S
Yasuda
Isao
I
Shiraishi
Norifumi
N
Nakanishi
D000369:Aged, 80 and over; D000080041:Congenitally Corrected Transposition of the Great Arteries; D006333:Heart Failure / Q000209:etiology*; D006801:Humans; D008297:Male; D012857:Situs Inversus / Q000150:complications*; D014188:Transposition of Great Vessels / Q000150:complications*