Heart transplantation in X-linked dilated cardiomyopathy.
BACKGROUND
X-linked dilated cardiomyopathy (DCM) is a clinical phenotype of dystrophinopathy characterized by preferential myocardial involvement without overt signs of skeletal muscle disease. X-linked DCM is a familiar myocardial disease characterized by ventricular dilation resulting in progressive heart failure and/or sudden death, and it may be differentiated from other DCMs. The aim of this retrospective study was to assess that patients with end-stage X-linked DCM can safely undergo heart transplantation.


METHODS
Between August 1989 and January 2000, 7 patients presenting with X-linked DCM underwent heart transplantation for end-stage disease at our Institution. The patients' age ranged from 16 to 31 years (mean 24.4 years) and all were in NYHA functional class IV.


RESULTS
The mean follow-up was 44 months (range 22-66 months). Only one sudden death occurred at 66 months of follow-up; all the other patients are doing well and are in NYHA functional class I.


CONCLUSIONS
Our data suggest that heart transplantation can be considered as the treatment of choice for refractory cardiac failure in X-linked DCM.
Grande
Antonino M
AM
Cardiac Surgery Department, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy. amgrande@libero.it
Rinaldi
Mauro
M
Pasquino
Stefano
S
D'Armini
Andrea M
AM
Viganò
Mario
M
D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000235:genetics* / Q000601:surgery*; D016189:Dystrophin / Q000235:genetics; D040181:Genetic Diseases, X-Linked; D016027:Heart Transplantation; D006801:Humans; D007902:Length of Stay; D012720:Severity of Illness Index; D016896:Treatment Outcome
D016189:Dystrophin