RESEARCH ARTICLE Left Ventricular Geometry and Blood Pressure as Predictors of Adverse Progression of Fabry

Background In spite of several research studies help to describe the heart in Fabry disease (FD), the car-diomyopathy is not entirely understood. In addition, the impact of blood pressure and alter-ations in geometry have not been systematically evaluated. Methods In 74 FD patients (mean age 36±12 years; 45 females) the extent of myocardial fibrosis and its progression were quantified using cardiac magnetic-resonance-imaging with late enhancement technique (LE). Results were compared to standard echocardiography com-plemented by 2D-speckle-tracking, 3D-sphericity-index (SI) and standardized blood pres-sure measurement. At baseline, no patient received enzyme replacement therapy (ERT). After 51±24 months, a follow-up examination was performed. Results Systolic blood pressure (SBP) was higher in patients with vs. without LE: 123±17 mmHg vs. 115±13 mmHg; P = 0.04. A positive correlation was found between SI and the amount o