Journal article
Usefulness of Alcohol Septal Ablation in the Left Ventricular Outflow Tract Obstruction in Fabry Disease Cardiomyopathy
The American journal of cardiology, Vol.150, pp.110-113
07/01/2021
DOI: 10.1016/j.amjcard.2021.03.042
PMID: 34011439
Abstract
Fabry disease (FD) is an X-linked linked genetic disorder caused by α-galactosidase A deficiency. The typical clinical manifestation is left ventricular hypertrophy, often mimicking hypertrophic cardiomyopathy (HC). In contrast to sarcomeric HC, left ventricular outflow tract obstruction (LVOTO) is less frequent. We describe 6 male patients with genetically confirmed FD and symptomatic LVOTO. All of them underwent a transcatheter alcohol septal ablation with an immediate effect on the obstruction in all cases and without any serious complications. The median LVOT maximal pressure gradient was 85 (60 to 170) mm Hg. The hemodynamic effect persisted during subsequent follow-up (ranging from 6 months to 16 years). Five patients reported substantial symptomatic improvement. Four patients were receiving specific FD therapy before the interventional procedure. In conclusion, alcohol septal ablation appears to be effective in the treatment of LVOTO in patients with FD and appears to be comparable to the limited published experience with surgical septal myectomy. Despite some important differences between FD HC and sarcomeric HC, the recommendation for treating LVOTO should be similar.
Details
- Title: Subtitle
- Usefulness of Alcohol Septal Ablation in the Left Ventricular Outflow Tract Obstruction in Fabry Disease Cardiomyopathy
- Creators
- David Zemánek - Charles UniversityJosef Marek - Charles UniversityGabriela Dostálová - Charles UniversitySudheera Magage - Charles UniversityLenka Roblová - Charles UniversityTomáš Kovárník - Charles UniversityAleš Linhart - Charles University
- Resource Type
- Journal article
- Publication Details
- The American journal of cardiology, Vol.150, pp.110-113
- DOI
- 10.1016/j.amjcard.2021.03.042
- PMID
- 34011439
- ISSN
- 0002-9149
- eISSN
- 1879-1913
- Language
- English
- Date published
- 07/01/2021
- Academic Unit
- Electrical and Computer Engineering
- Record Identifier
- 9984627326702771
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