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Fabry Disease: Percutaneous Transluminal Septal Myocardial Ablation Markedly Improved Symptomatic Left Ventricular Hypertrophy and Outflow Tract Obstruction in a Classically Affected Male
Author(s) -
Magage Sudheera,
Linhart Ales,
Bultas Jan,
Vojacek Jan,
Mates Martin,
Palecek Tomas,
Popelová Jana,
Tintera Jaroslav,
Aschermann Michael,
Goldman Martin E.,
Desnick Robert J.
Publication year - 2005
Publication title -
echocardiography
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.404
H-Index - 62
eISSN - 1540-8175
pISSN - 0742-2822
DOI - 10.1111/j.1540-8175.2005.03191.x
Subject(s) - medicine , cardiology , ventricular outflow tract obstruction , percutaneous , left ventricular hypertrophy , hypertrophic cardiomyopathy , muscle hypertrophy , ablation , cardiomyopathy , alcohol septal ablation , ventricular outflow tract , obstructive cardiomyopathy , heart failure , blood pressure
Fabry disease (α‐galactosidase A deficiency) is an X‐linked recessive lysosomal storage disease in which left ventricular hypertrophy (LVH) is common, and if severe, may mimic hypertrophic obstructive cardiomyopathy. Alcohol‐induced percutaneous transluminal septal myocardial ablation (PTSMA) has been used as a safe and effective method to alleviate LVH obstruction in patients with hypertrophic obstructive cardiomyopathy (HCM). We describe a case of a classically affected Fabry 53‐year‐old male with symptomatic HCM (NYHA class III with exertional angina) who was treated with PTSMA. The procedure safely and effectively alleviated symptomatic left ventricular outflow tract obstruction at long‐term follow‐up, and the patient's NYHA classification was reduced to NYHA class I to II.