Impaired myocardial perfusion reserve and fibrosis in Friedreich ataxia: a mitochondrial cardiomyopathy with metabolic syndrome.

Aims: Cardiomyopathy produces significant mortality in patients with Friedreich ataxia (FA), a genetic disorder that produces intra-mitochondrial iron accumulation. We sought to test the hypothesis that abnormal myocardial perfusion reserve and fibrosis represent early manifestations of cardiomyopathy.

Methods And Results: Twenty-six patients with genetically proven FA ages 36 ± 12 years without cardiomyopathy and eight controls underwent cardiac magnetic resonance with adenosine.

Predictors of early mortality in patients age 80 and older receiving implantable defibrillators.

Background: There are no upper age restrictions for implantable defibrillators (ICDs) but their benefit may be limited in patients > or = 80 years with strong competing risks of early mortality. Risk factors for early (1-year) mortality in ICD recipients > or = 80 years of age have not been established.

Methods: Two-center retrospective cohort study to assess predictors of one-year mortality in ICD recipients > or = 80 years of age.