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Volume 11
Issue 9
10.3390/jcdd11090293
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Case Report

Septal Myectomy in Patients with Hypertrophic Cardiomyopathy and Nonclassical Anderson–Fabry Disease

by
Alexandr Gurschenkov
1,
Sofiya Andreeva
2,
Vadim Zaitsev
1,
Pavel Khazov
1,
Gleb Ischmukhametov
1,
Alexandra Kozyreva
2,
Polina Sokolnikova
2,
Olga Moiseeva
1,
Anna Kostareva
2,3,* and
Mikhail Gordeev
1
1
Institute of Cardiovascular Science, Almazov National Medical Research Centre, 197341 Saint Petersburg, Russia
2
World-Class Research Centre for Personalized Medicine, Almazov National Medical Research Centre, 197341 Saint Petersburg, Russia
3
Department of Woman and Children’s Health, Karolinska Institutet (KI), 171 77 Stockholm, Sweden
*
Author to whom correspondence should be addressed.
J. Cardiovasc. Dev. Dis. 2024, 11(9), 293; https://doi.org/10.3390/jcdd11090293
Submission received: 11 July 2024 / Revised: 15 August 2024 / Accepted: 10 September 2024 / Published: 20 September 2024
(This article belongs to the Special Issue Hypertrophic Cardiomyopathy: Pathogenesis, Diagnosis and Management)
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Abstract

Anderson–Fabry disease (AFD) results from decreased enzyme activity of lysosomal enzymes and intralysosomal storage of nonhydrolyzed forms. Cardiovascular complications, mainly in the form of HCM, contribute substantially to AFD patient mortality. Here, we report three new cases of obstructive HCM (HOCM) in nonclassical presentations of AFD and isolated cardiac involvement. In all three cases, the diagnosis of AFD was made postoperatively by routine genetic and morphological testing. Together with previously published cases, this report illustrates the potential safety 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.
Keywords: hypertrophic cardiomyopathy; obstructive; myectomy; Anderson–Fabry disease; GLA hypertrophic cardiomyopathy; obstructive; myectomy; Anderson–Fabry disease; GLA

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MDPI and ACS Style

Gurschenkov, A.; Andreeva, S.; Zaitsev, V.; Khazov, P.; Ischmukhametov, G.; Kozyreva, A.; Sokolnikova, P.; Moiseeva, O.; Kostareva, A.; Gordeev, M. Septal Myectomy in Patients with Hypertrophic Cardiomyopathy and Nonclassical Anderson–Fabry Disease. J. Cardiovasc. Dev. Dis. 2024, 11, 293. https://doi.org/10.3390/jcdd11090293

AMA Style

Gurschenkov A, Andreeva S, Zaitsev V, Khazov P, Ischmukhametov G, Kozyreva A, Sokolnikova P, Moiseeva O, Kostareva A, Gordeev M. Septal Myectomy in Patients with Hypertrophic Cardiomyopathy and Nonclassical Anderson–Fabry Disease. Journal of Cardiovascular Development and Disease. 2024; 11(9):293. https://doi.org/10.3390/jcdd11090293

Chicago/Turabian Style

Gurschenkov, Alexandr, Sofiya Andreeva, Vadim Zaitsev, Pavel Khazov, Gleb Ischmukhametov, Alexandra Kozyreva, Polina Sokolnikova, Olga Moiseeva, Anna Kostareva, and Mikhail Gordeev. 2024. "Septal Myectomy in Patients with Hypertrophic Cardiomyopathy and Nonclassical Anderson–Fabry Disease" Journal of Cardiovascular Development and Disease 11, no. 9: 293. https://doi.org/10.3390/jcdd11090293

APA Style

Gurschenkov, A., Andreeva, S., Zaitsev, V., Khazov, P., Ischmukhametov, G., Kozyreva, A., Sokolnikova, P., Moiseeva, O., Kostareva, A., & Gordeev, M. (2024). Septal Myectomy in Patients with Hypertrophic Cardiomyopathy and Nonclassical Anderson–Fabry Disease. Journal of Cardiovascular Development and Disease, 11(9), 293. https://doi.org/10.3390/jcdd11090293

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