RESUMO
La distrofia muscular de Emery-Dreifuss está asociada a anomalías cardiacas, pudiendo ser el trasplante cardiaco el tratamiento de elección en ciertos casos raros. En este caso, un paciente con distrofia muscular de Emery-Dreifuss desarrolló insuficiencia cardiaca de clase IV de NYHA a los 33 años de edad, y fue sometido a trasplante cardiaco. Se adaptó la anestesia para prevenir el desarrollo de hipertermia maligna y rabdomiólisis. La cirugía fue exitosa, y el paciente progresó de manera excelente con mejora sintomática. En estos casos, es esencial ajustar, no solo el posicionamiento del paciente, sino también la terapia administrada, a fin de reducir la iatrogenia y acelerar la recuperación. (AU)
Emery-Dreifuss muscular dystrophy is associated with cardiac abnormalities and rarely heart transplantation may be the treatment of choice. In this case, a male patient with Emery- Dreifuss muscular dystrophy developed NYHA class IV heart failure at 33 years of age and was submitted to heart transplantation. Anesthesia was adapted to prevent the development of malignant hyperthermia and rhabdomyolysis. The surgery was a success and the patient's progress was extremely positive with symptomatic improvement. In these patients, is critical to adjust not only his positioning but also the therapy administered in order to reduce iatrogeny and promote a faster recovery. (AU)
Assuntos
Humanos , Adulto Jovem , Transplante de Coração , Anestesia , Cardiomiopatias/terapia , Distrofia Muscular de Emery-Dreifuss/complicações , Distrofia Muscular de Emery-Dreifuss/cirurgiaRESUMO
Emery-Dreifuss muscular dystrophy is associated with cardiac abnormalities and rarely heart transplantation may be the treatment of choice. In this case, a male patient with Emery- Dreifuss muscular dystrophy developed NYHA class IV heart failure at 33 years of age and was submitted to heart transplantation. Anesthesia was adapted to prevent the development of malignant hyperthermia and rhabdomyolysis. The surgery was a success and the patient's progress was extremely positive with symptomatic improvement. In these patients, is critical to adjust not only his positioning but also the therapy administered in order to reduce iatrogeny and promote a faster recovery.
