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.
Heart transplantation in X-linked dilated cardiomyopathy
RINALDI, MAURO;PASQUINO, STEFANO;D'ARMINI, ANDREA MARIA;VIGANO', MARIO
2002-01-01
Abstract
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
