A 19 year old man collapses during basketball practice and is successfully resuscitated. His father died suddenly at age 32. Electrocardiogram shows ventricular tachycardia with a left bundle branch block morphology. Cardiac MRI demonstrates thinning of the right ventricular free wall with regional akinesia. Which of the following is the most likely underlying defect?
- A)Autoantibodies against beta 1 adrenergic receptors
- B)Mutation in desmosomal proteinsGABARITO
- C)Post inflammatory scarring after rheumatic fever
- D)Defect in dystrophin associated glycoproteins
- E)Amyloid light chain deposition
Explicação
Mutation in desmosomal proteins is correct. Arrhythmogenic right ventricular cardiomyopathy classically presents in young athletes with ventricular arrhythmias, sudden cardiac death, and fibrofatty replacement of the right ventricle. Desmosomal defects impair ... Ver explicação completa e trilha adaptativa →