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Vericiguat improves cardiac function and microcirculation of a male patient with Fabry disease: A case report

Fabry disease (FD) is a rare X chromosome‐linked disorder and can be easily misdiagnosed. Here, we report the case of a 69‐year‐old male patient with FD who developed heart failure and showed extremely high pulmonary artery pressure. His initial symptom was recurrent atrial fibrillation. The left an...

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Bibliographic Details
Published in:Annals of noninvasive electrocardiology 2024-05, Vol.29 (3), p.e13115-n/a
Main Authors: Zhou, Bingyang, Ren, Ning, Geng, Jie
Format: Article
Language:English
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Summary:Fabry disease (FD) is a rare X chromosome‐linked disorder and can be easily misdiagnosed. Here, we report the case of a 69‐year‐old male patient with FD who developed heart failure and showed extremely high pulmonary artery pressure. His initial symptom was recurrent atrial fibrillation. The left and right atrial inner diameters were large, and the ventricular wall was thick. Gene analysis which showed GLA c.215T>C p.Met72Thr mutation and single photon emission computed tomography indicated the diagnosis of FD with coronary microvascular dysfunction. The patient was prescribed anti‐heart failure drugs, including vericiguat. Following the treatment, his heart function and microvascular perfusion significantly improved, which might be due to the beneficial effects of vericiguat. Left ventricular myocardial flow reserve (LVMFR) detected by single photon emission computed tomography initially showed severe microvascular perfusion impairment (Figure 4) but significantly improved (Figure 5) following vericiguat treatment for 3 months in a Fabry disease male patient.
ISSN:1082-720X
1542-474X
DOI:10.1111/anec.13115