Christopher Orsborne, Joshua Bradley, Laura J Bonnett, Luke A Pleva, Josephine H Naish, David G Clark, Nik Abidin, Peter Woolfson, Gaetano Nucifora, Matthias Schmitt, Ana Jovanovic, Christopher A Miller, Anna B Reid
BACKGROUND: The cardiac manifestations of Fabry disease are the leading cause of death, but risk stratification remains inadequate. Identifying patients who are at risk of adverse cardiac outcome may facilitate more evidence-based treatment guidance. Contemporary cardiovascular cardiac magnetic resonance biomarkers have become widely adopted, but their prognostic value remains unclear. OBJECTIVES: The objective of this study was to develop, internally validate, and evaluate the performance of, a prognostic model, including contemporary deep phenotyping, which can be used to generate individual risk estimates for adverse cardiac outcome in patients with Fabry disease...
September 6, 2022: Journal of the American College of Cardiology