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Heart transplantation in X-linked dilated cardiomyopathy
Antonino M Grande1, Mauro Rinaldi, Stefano Pasquino
1Cardiac Surgery Department, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy. amgrande@libero.it
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.