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

## TL;DR

This paper discusses three cases where patients with a rare genetic disorder had heart surgery to treat a specific heart condition, highlighting the potential benefits and the need for better screening.

## Contribution

The study presents new cases showing the safety and effectiveness of surgical myectomy in AFD-related heart conditions.

## Key findings

- Three new cases of obstructive HCM in nonclassical AFD were successfully treated with septal myectomy.
- AFD was diagnosed postoperatively through genetic and morphological testing in all three cases.
- The report emphasizes the importance of screening for AFD in patients with HOCM.

## 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.