Onset and progression of the Anderson-Fabry disease related cardiomyopathy.

  • Christoph Kampmann
  • Ales Linhart
  • Frank Baehner
  • Tomas Palecek
  • Christiane M Wiethoff
  • Elke Miebach
  • Catharina Whybra
  • Andreas Gal
  • Jan Bultas
  • Michael Beck

Beteiligte Einrichtungen

Abstract

BACKGROUND: Cardiac involvement is responsible for substantial morbidity and mortality in Anderson-Fabry disease (AFD). We sought to document its onset and progression in a population of male and female AFD patients. METHODS: We performed a cross sectional echocardiographic study of a cohort of 177 male and female AFD patients with subsequent longitudinal follow-up of 76 patients (38 males and 38 females; mean follow-up 4.5 years) who did not receive enzyme replacement therapy. RESULTS: In this population, aged 3.3 to 70.8 years, a strong correlation between age and left ventricular mass indexed (LVMi, g/m(2.7)) was found in both males and females (P

Bibliografische Daten

OriginalspracheDeutsch
Aufsatznummer3
ISSN0020-7136
StatusVeröffentlicht - 2008
pubmed 18572264