CardiomyopathyPrevalence and Clinical Significance of Cardiac Arrhythmia in Anderson-Fabry Disease
Section snippets
Methods
From January 1993 to December 2003, 78 patients (43 men [55%]; mean age 43.5 ± 15.0 years, range 13 to 83) with AFD were assessed at The Heart Hospital, University College London, London, United Kingdom. The diagnosis of AFD was based on plasma α-galactosidase A levels and mutational analysis. Seven patients were identified during the screening of patients with unexplained left ventricular (LV) hypertrophy.3 The remainder were direct referrals for cardiac evaluation. Forty-one patients (6
Clinical evaluation
Seven patients (9%) were receiving β blockers, 2 were receiving sotolol for a history of paroxysmal AF, and 3 were receiving amiodarone for paroxysmal AF and 1 for nonsustained VT. Table 1 describes the prevalence of cardiac and AFD symptoms, and Table 2 lists baseline electrocardiographic and echocardiographic findings stratified by gender. There were no differences between men and women in the prevalence of cardiac symptoms. Men were more likely to have AFD symptoms and signs compared with
Discussion
AFD is an increasingly recognized cause of LV hypertrophy in middle-aged men and women.3, 9, 10 This study shows that arrhythmias are common in patients with AFD and that they are associated with significant morbidity.
Population-based studies have shown that the prevalence of AF in the normal population is <1%11; this prevalence is age dependant, with a prevalence of <1% in subjects <55 years old, increasing to ≥9% in those aged >80 years.11 In this study, the prevalence of AF was 4 times
Acknowledgment
We would like to thank Alan Milligan, RGJ, RGN, Linda Richfield, RGJ, RGN, Rebecca Bruce, RGN, Sian Goodwin, RGN, Melissa Blincoe, RGN, and Rebecca Bruce, RGN, from The Lysosomal Storage Disorders Unit and Amy Cole from The Charles Dent Metabolic Unit for their help in data collection.
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Cited by (178)
Screening of Fabry disease in patients with an implanted permanent pacemaker
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2022, American Journal of CardiologyComplete Atrioventricular Block After Kidney Transplantation in a Patient With Fabry Disease Receiving Enzyme Replacement Therapy: A Case Report
2022, Transplantation ProceedingsCitation Excerpt :Concentric left ventricular hypertrophy with an excessive left ventricular mass is the predominant structural finding in FD cardiomyopathy [18]. Complete atrioventricular blocks are rare in patients with FD, and it has been suggested that the accumulation of glycosphingolipids in and around the conduction system causes conduction disturbances in patients with FD [19–25]. Although information on cardiac pathology was not available in our case, there were no other identified causes for complete atrioventricular block, which strongly suggests that conduction disturbances are caused by FD.
Disease features and management of cardiomyopathies in women
2024, Heart Failure Reviews
Dr. Shah and Dr. Hughes received an educational grant from Transkaryotic Therapies Europe-5S AB, Danderyd, Sweden. Dr. Ward and Dr. Tome are funded through a British Heart Foundation Junior Research Fellowship, London, United Kingdom.