title: Left Ventricular Geometry and Blood Pressure as Predictors of Adverse Progression of Fabry Cardiomyopathy.
abstract: BACKGROUND
  In spite of several research studies help to describe the heart in Fabry disease (FD), the cardiomyopathy is not entirely understood. In addition, the impact of blood pressure and alterations 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 complemented by 2D-speckle-tracking, 3D-sphericity-index (SI) and standardized blood pressure 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 of LE-positive myocardium (r = 0.51; P<0.001) indicating an association of higher SI in more advanced stages of the cardiomyopathy. SI at baseline was positively associated with the increase of LE-positive myocardium during follow-up. The highest SBP (125±19 mmHg) and also the highest SI (0.32±0.05) was found in the subgroup with a rapidly increasing LE (ie, ≥0.2% per year; n = 16; P = 0.04). Multivariate logistic regression analysis including SI, SBP, EF, left ventricular volumes, wall thickness and NT-proBNP adjusted for age and sex showed SI as the most powerful parameter to detect rapid progression of LE (AUC = 0.785; P<0.05).
  
  
  CONCLUSIONS
  LV geometry as assessed by the sphericity index is altered in relation to the stage of the Fabry cardiomyopathy. Although patients with FD are not hypertensive, the SBP has a clear impact on the progression of the cardiomyopathy.
authors.affiliation: Department of Medicine I, Cardiology Unit, University of Würzburg, Würzburg, Germany.|Department of Pediatrics and Adolescent Medicine, University of Ulm, Ulm, Germany.|Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.
authors.forename: Johannes
authors.initials: J
authors.lastname: Krämer
authors.affiliation: ICREA-Universitat Pompeu Fabra, Barcelona, Spain and KU Leuven, Leuven, Belgium.
authors.forename: Bart
authors.initials: B
authors.lastname: Bijnens
authors.affiliation: Department of Medicine I, Cardiology Unit, University of Würzburg, Würzburg, Germany.|Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Störk
authors.affiliation: Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.|Department of Radiology, University of Würzburg, Würzburg, Germany.
authors.forename: Christian O
authors.initials: CO
authors.lastname: Ritter
authors.affiliation: Department of Medicine I, Cardiology Unit, University of Würzburg, Würzburg, Germany.|Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.
authors.forename: Dan
authors.initials: D
authors.lastname: Liu
authors.affiliation: Department of Medicine I, Cardiology Unit, University of Würzburg, Würzburg, Germany.|Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.
authors.forename: Georg
authors.initials: G
authors.lastname: Ertl
authors.affiliation: Department of Medicine I, Nephrology Unit, University of Würzburg, Würzburg, Germany.|Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Wanner
authors.affiliation: Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany.|Department of Internal Medicine II, Katharinen-Hospital Unna, Unna, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Weidemann
mesh_terms: D000328:Adult; D001794:Blood Pressure; D009202:Cardiomyopathies / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D015331:Cohort Studies; D018450:Disease Progression; D004562:Electrocardiography; D000795:Fabry Disease / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D005355:Fibrosis; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D016015:Logistic Models; D008279:Magnetic Resonance Imaging; D008297:Male; D015999:Multivariate Analysis; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D012372:ROC Curve; D014463:Ultrasonography
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain