RESUMEN
PURPOSE OF REVIEW: Duchenne muscular dystrophy is one of many neuromuscular disorders, but it frequently causes severe disability early in life and early death. Cardiac involvement is an important cause of morbidity and mortality. RECENT FINDINGS: Heart disease in Duchenne muscular dystrophy can include a cardiomyopathy leading to end-stage heart failure along with associated supraventricular and ventricular arrhythmias. This article reviews the diagnosis and treatment of heart disease in Duchenne muscular dystrophy as well as emerging therapies.
Asunto(s)
Cardiomiopatía Dilatada/etiología , Manejo de la Enfermedad , Ecocardiografía/métodos , Electrocardiografía , Imagen por Resonancia Cinemagnética/métodos , Distrofia Muscular de Duchenne/diagnóstico , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/epidemiología , Salud Global , Humanos , Masculino , Morbilidad/tendencias , Distrofia Muscular de Duchenne/complicaciones , Distrofia Muscular de Duchenne/epidemiología , Tasa de Supervivencia/tendencias , Adulto JovenRESUMEN
Idiopathic giant cell myocarditis (IGCM) is a rare disease causing progressive myocarditis characterized by myocardial necrosis and giant cells. Patients often present with rapidly progressive heart failure, ventricular arrhythmias, and heart block. Without treatment, the disease often results in progressive pump failure requiring urgent cardiac transplantation or the need for mechanical circulatory support. The underlying pathophysiologic mechanisms are not yet defined but appear to involve genetics, autoimmune disorders, and possibly environmental factors such as viruses. Combined immunosuppressive regimens appear to prolong survival from death or cardiac transplant. Nevertheless, cardiac transplant is an effective treatment. The disease can recur in the transplanted heart resulting in death or the need for retransplant.
Asunto(s)
Células Gigantes/patología , Miocarditis/diagnóstico , Fármacos Cardiovasculares/uso terapéutico , Trasplante de Corazón , Corazón Auxiliar , Humanos , Miocarditis/epidemiología , Miocarditis/fisiopatología , Miocarditis/terapia , Enfermedades Raras/diagnóstico , Enfermedades Raras/epidemiología , Enfermedades Raras/fisiopatología , Enfermedades Raras/terapiaRESUMEN
Anthracycline-based chemotherapeutics have long been recognized as effective agents for treating a wide range of malignancies. However, their use is not without significant adverse cardiotoxic side effects. Strategies for prevention involve limiting free-radical production and subsequent cardiac myocyte damage. Dexrazoxane remains the most widely studied cardioprotective medication. Alternative agents may reduce cardiotoxicity but may still cause significant cardiovascular problems. The role of ß-blockers and angiotensin-converting enzyme inhibitors in the treatment of heart failure is well proven. The role of these medications in the prevention and treatment of chemotherapy-induced cardiotoxicity is not well established.