About: Fabry disease: Percutaneous transluminal septal myocardial ablation markedly improved symptomatic left ventricular hypertrophy and outflow tract obstruction in a classically affected male     Goto   Sponge   NotDistinct   Permalink

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  • Fabryho choroba (galaktosidázová A deficience) je X-vázaná recesivní lyzosomální porucha, ve které se běžně vyskytuje levostranná ventrikulární hypertrofie (LVH) a pokud se jedná o těžký případ může imitovat hypertrofickou instruktivní kardiomyopatii. Použili jsme alkoholem indukovanou perkutánní transluminární septální myokardiální ablaci (PTSMA), která je bezpečnou a účinnou metodou při zmírnění obstrukci LVH u pacientů s hypertrofickou obstrukční kardiomyopatií (HCM). Zde popisujeme případ 53 letého muže klasicky postiženého Fabryho chorobou se symptomatickou HCM (NYHA třída III s námahovou anginou) léčeného na PTSMA. Popisovaný postup bezpečně a efektivně zmírnil symptomatickou obstrukci výstupu z levé komory srdeční při dlouhodobém sledování a pacientova klasifikace byla zmírněna na NYHA třída I-II. (cs)
  • Fabry disease (?-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.
  • Fabry disease (?-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. (en)
Title
  • Fabry disease: Percutaneous transluminal septal myocardial ablation markedly improved symptomatic left ventricular hypertrophy and outflow tract obstruction in a classically affected male
  • Fabryho choroba: Perkutánní transluminální septální myokardiální ablace značně zlepšila příznaky hypertrofie levé komory a obstrukce výtokového traktu u klasicky postiženého muže (cs)
  • Fabry disease: Percutaneous transluminal septal myocardial ablation markedly improved symptomatic left ventricular hypertrophy and outflow tract obstruction in a classically affected male (en)
skos:prefLabel
  • Fabry disease: Percutaneous transluminal septal myocardial ablation markedly improved symptomatic left ventricular hypertrophy and outflow tract obstruction in a classically affected male
  • Fabryho choroba: Perkutánní transluminální septální myokardiální ablace značně zlepšila příznaky hypertrofie levé komory a obstrukce výtokového traktu u klasicky postiženého muže (cs)
  • Fabry disease: Percutaneous transluminal septal myocardial ablation markedly improved symptomatic left ventricular hypertrophy and outflow tract obstruction in a classically affected male (en)
skos:notation
  • RIV/00216208:11150/05:00004829!RIV08-MSM-11150___
http://linked.open.../vavai/riv/strany
  • 333;339
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • N, S, V
http://linked.open...iv/cisloPeriodika
  • 4
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
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http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 521319
http://linked.open...ai/riv/idVysledku
  • RIV/00216208:11150/05:00004829
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • Fabry; disease; Percutaneous; transluminal; septal; myocardial; ablation; markedly; improved; symptomatic; ventricular; hypertrophy; outflow; tract; obstruction; classically; affected; male (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • US - Spojené státy americké
http://linked.open...ontrolniKodProRIV
  • [7B88776E4630]
http://linked.open...i/riv/nazevZdroje
  • Echocardiography
http://linked.open...in/vavai/riv/obor
http://linked.open...ichTvurcuVysledku
http://linked.open...cetTvurcuVysledku
http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 22
http://linked.open...iv/tvurceVysledku
  • Vojáček, Jan
issn
  • 0742-2822
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  • 11150
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