Successful heart transplantation in patients with inherited myopathies associated with end-stage cardiomyopathy.
Inherited myopathies in patients with secondary end-stage cardiomyopathies have always been considered a relative contraindication for cardiac transplantation. High operative risk related to muscle impairment and potential graft involvement secondary to the underlying myopathy have been the two main reasons implicated in the poor prognosis.


OBJECTIVE
The aim of this study was to evaluate the outcome in patients who underwent cardiac transplantation in our hospital due to end-stage cardiomyopathy secondary to inherited myopathies.


METHODS
Among 311 patients who underwent heart transplantation in our hospital, five (2%) had end-stage cardiomyopathies related to inherited myopathies. Four patients had muscular dystrophy (three Becker's muscular dystrophy and one hips-dystrophy) and the fifth desminopathy. In one patient cardiomyopathy was the initial manifestation of the disease. Mean age at the time of transplantation was 38.6 years (range from 24 to 55). The mean follow-up after transplantation was 57.4 months (range from 13 to 128). The intraoperative and postoperative course of these individuals did not show higher complication rates than other patients. All recipients experienced successful rehabilitation; no evidence of graft dysfunction has been detected during follow-up. All of them are alive with a good performance status.


CONCLUSIONS
In our experience, patients who underwent heart transplantation due to end-stage cardiomyopathy secondary to inherited myopathy with only a mild degree of muscle impairment did not display higher postoperative nor long-term complications compared to other recipients.
Ruiz-Cano
M J
MJ
Heart Failure and Transplant Unit, Cardiology Department, Hospital Doce de Octubre, Madrid, Spain.
Delgado
J F
JF
Jiménez
C
C
Jiménez
S
S
Cea-Calvo
L
L
Sánchez
V
V
Escribano
P
P
Gómez
M A
MA
Gil-Fraguas
L
L
Sáenz de la Calzada
C
C
D000293:Adolescent; D000328:Adult; D009202:Cardiomyopathies / Q000209:etiology / Q000601:surgery*; D005500:Follow-Up Studies; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D009136:Muscular Dystrophies / Q000150:complications*; D020388:Muscular Dystrophy, Duchenne / Q000601:surgery; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome