Cardiomyopathy, familial dilated

摘要: Dilated cardiomyopathy (DCM) is a heart muscle disease characterized by ventricular dilatation and impaired systolic function. Patients with DCM suffer from failure, arrhythmia, are at risk of premature death. has prevalence one case out 2500 individuals an incidence 7/100,000/year (but may be under diagnosed). In many cases the inherited termed familial (FDC). FDC account for 20–48% DCM. principally caused genetic mutations in genes that encode cytoskeletal sarcomeric proteins cardiac myocyte. Family history analysis important tool identifying families affected FDC. Standard criteria evaluating have been published use such increasing. Clinical testing developed some will increasingly utilized families. Through family screening pedigree and/or testing, it possible to identify patients earlier, or even presymptomatic stages their disease. This presents opportunity invoke lifestyle changes provide pharmacological therapy earlier course Genetic counseling used additional asymptomatic members who developing symptoms, allowing regular these individuals. The management focuses on limiting progression failure controlling based currently accepted treatment guidelines It includes general measures (salt fluid restriction, hypertension, limitation alcohol intake, control body weight, moderate exercise) pharmacotherapy. Cardiac resynchronization, implantable cardioverter defibrillators left assist devices progressively expanding usage. severe reduction functional capacity depressed ejection fraction low survival rate require transplant.