Late-Onset Fabry Disease Presenting With Ventricular Tachycardia Originating From Typical Inferolateral Scar

2020 ◽  
Vol 36 (11) ◽  
pp. 1832.e1-1832.e4
Author(s):  
Giulia De Angelis ◽  
Zoi Tsoumani ◽  
Anna Reid ◽  
Gaetano Nucifora ◽  
Richard Bruce Irwin ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Fabry Disease ◽  
Late Onset

scholarly journals Late-onset Fabry disease revealed by ventricular tachycardia: a Case report

2021 ◽  
Author(s):  
Geoffroy Ditac ◽  
Kévin Gardey ◽  
Antoine Jobbé-Duval ◽  
Alain Fouilhoux ◽  
Gilles Millat ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Case Report ◽  
Fabry Disease ◽  
Late Onset

Phenotypic target organ and biomarker variation within a family with late onset Fabry disease

2021 ◽  
Vol 132 (2) ◽  
pp. S27
Author(s):  
Jennifer Coker ◽  
Ashlee R. Stiles ◽  
Deeksha Bali ◽  
Sara P. Young ◽  
Marie T. McDonald ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Late Onset ◽  
Target Organ

scholarly journals Plasma globotriaosylsphingosine (lysoGb3) could be a biomarker for Fabry disease with a Chinese hotspot late-onset mutation (IVS4+919G>A)

2013 ◽  
Vol 426 ◽  
pp. 114-120 ◽  
Author(s):  
Hsuan-Chieh Liao ◽  
Yu-Hsiu Huang ◽  
Yann-Jang Chen ◽  
Shu-Min Kao ◽  
Hsiang-Yu Lin ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Late Onset

scholarly journals Study of indications for cardiac device implantation and utilisation in Fabry cardiomyopathy

Heart ◽  
2019 ◽  
Vol 105 (23) ◽  
pp. 1825-1831 ◽  
Author(s):  
Ravi Vijapurapu ◽  
Tarekegn Geberhiwot ◽  
Ana Jovanovic ◽  
Shanat Baig ◽  
Sabrina Nordin ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Ventricular Tachycardia ◽  
Fabry Disease ◽  
Premature Death ◽  
Sustained Ventricular Tachycardia ◽  
Implantable Cardioverter Defibrillators ◽  
Imaging Data ◽  
Cardiac Device ◽  
Device Implantation ◽  
Cardiac Devices

BackgroundFabry disease is a treatable X-linked condition leading to progressive cardiomyopathy, arrhythmia and premature death. Atrial and ventricular arrhythmias contribute significantly to adverse prognosis; however, guidance to determine which patients require cardiovascular implantable electronic devices (CIEDs) is sparse. We aimed to evaluate indications for implantation practice in the UK and quantify device utilisation.MethodsIn this retrospective study, we included demographic, clinical and imaging data from patients in four of the largest UK Fabry centres. Ninety patients with Fabry disease were identified with CIEDs implanted between June 2001 and February 2018 (FD-CIED group). To investigate differences in clinical and imaging markers between those with and without devices, these patients were compared with 276 patients without a CIED (FD-control).ResultsIn the FD-CIED group, 92% of patients with permanent pacemakers but only 28% with implantable cardioverter-defibrillators had a class 1 indication for implantation. A further 44% of patients had defibrillators inserted for primary prevention outside of current guidance. The burden of arrhythmia requiring treatment in the FD-CIED group was high (asymptomatic atrial fibrillation:29%; non-sustained ventricular tachycardia requiring medical therapy alone: 26%; sustained ventricular tachycardia needing anti-tachycardia pacing/defibrillation: 28%). Those with devices were older, had greater LV mass, more scar tissue and larger atrial size.ConclusionsArrhythmias are common in Fabry patients. Those with cardiac devices had high rates of atrial fibrillation requiring anticoagulation and ventricular arrhythmia needing device treatment. These are as high as those in hypertrophic cardiomyopathy, supporting the need for Fabry-specific indications for device implantation.

First case of late onset cardiac phenotype Fabry disease due to an AluYb8 insertion in exon 7 of the GLA gene

2019 ◽  
Vol 20 (10) ◽  
pp. 1182-1182
Author(s):  
Constantin Gatterer ◽  
Dietrich Beitzke ◽  
Raute Sunder-Plassmann ◽  
Gere Sunder-Plassmann ◽  
Senta Graf
Keyword(s):  
Fabry Disease ◽  
Late Onset ◽  
Cardiac Phenotype ◽  
First Case ◽  
Gla Gene

scholarly journals Prevalence of Anderson-Fabry Disease in Male Patients With Late Onset Hypertrophic Cardiomyopathy

Circulation ◽  
2002 ◽  
Vol 105 (12) ◽  
pp. 1407-1411 ◽  
Author(s):  
B. Sachdev ◽  
T. Takenaka ◽  
H. Teraguchi ◽  
C. Tei ◽  
P. Lee ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Fabry Disease ◽  
Late Onset ◽  
Male Patients

Hypertrophic cardiomyopathy in late-onset variant of Fabry disease with high residual activity of α-galactosidase A

2008 ◽  
Vol 39 (3) ◽  
pp. 233-237 ◽  
Author(s):  
Yoshiro Nagao ◽  
Haruko Nakashima ◽  
Yukiko Fukuhara ◽  
Michie Shimmoto ◽  
Akihiro Oshima ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Fabry Disease ◽  
Late Onset ◽  
Residual Activity
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