Septal Myectomy in Patients with Hypertrophic Cardiomyopathy and Non-classical Anderson-Fabry Disease.

Alexandr Gurschenkov; Sofia Andreeva; Vadim Zaitsev; Pavel Khazov; Gleb Ischmukhametov; Alexandra Kozyreva; Polina Sokolnikova; Olga Moiseeva; Anna Kostareva; Mikhail Gordeev

doi:10.20944/preprints202407.1100.v1

Preprint Case Report Version 1 This version is not peer-reviewed

Version 1 : Received: 12 July 2024 / Approved: 13 July 2024 / Online: 15 July 2024 (10:16:20 CEST)

Anderson-Fabry disease (AFD) results from decreased enzyme activity of lyzosmal enzyme and intralyzosomal storage of its nonhydrolyzed. Cadiovascular complications, mainly in s form of HCM contribute substantially for AFD-patient’s mortality. Here we report three new cases of obstructive HCM (HOCM) due to unclassical presentation of AFD and isolated cardiac involvement. In all three cases the diagnosis of AFD was made postoperative by routine genetic and morphological testing. Together with previously published cases this report illustrates the potential safely and beneficial effect of septal surgical myectomy in patients with AFD-HOCM, as well as underlines the need for more thorough screening for clinical signs of AFD-associated cardiomyopathy and GLA variants among patients with HOCM.

hypertrophic cardiomyopathy; obstructive; myectomy, Anderson-Fabri disease, GLA

Medicine and Pharmacology, Cardiac and Cardiovascular Systems

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Septal Myectomy in Patients with Hypertrophic Cardiomyopathy and Non-classical Anderson-Fabry Disease.

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