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Left ventricular geometry and blood pressure as predictors of adverse progression of Fabry cardiomyopathy

Please always quote using this URN: urn:nbn:de:bvb:20-opus-145131
  • 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 byBackground 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.show moreshow less

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Metadaten
Author: Johannes Krämer, Bart Bijnens, Stefan Störk, Christian O. Ritter, Dan Liu, Georg Ertl, Christoph Wanner, Frank Weidemann
URN:urn:nbn:de:bvb:20-opus-145131
Document Type:Journal article
Faculties:Medizinische Fakultät / Institut für diagnostische und interventionelle Radiologie (Institut für Röntgendiagnostik)
Medizinische Fakultät / Medizinische Klinik und Poliklinik I
Language:English
Parent Title (English):PLoS ONE
Year of Completion:2015
Volume:10
Issue:11
Pagenumber:e0140627
Source:PLoS ONE 10(11): e0140627 (2015). DOI: 10.1371/journal.pone.0140627
DOI:https://doi.org/10.1371/journal.pone.0140627
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Tag:2-dimensional speckle tracking; cardiovascular magnetic resonance; clinical manifestations; diagnosis; disease; fibrosis; identification; impact; myocardial infarction; therapy
Release Date:2018/11/06
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung 4.0 International