Ventricular fibrillation associated with vasospastic angina pectoris in Fabry disease: a case report.

ABSTRACT:

Background

Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from a deficiency in alpha-galactosidase A. The major causes of death due to cardiac complications include life-threatening arrhythmias. In addition, life-threatening arrhythmias may be related to myocardial fibrosis assessed by late gadolinium enhancement (LGE).

Case summary

A 43-year-old man with sinus bradycardia and left ventricular hypertrophy was referred to our cardiology department. Family history includes unexplained hypertrophy and sick sinus syndrome in mother. Additionally, his plasma alpha-galactosidase A activity was low. He was subsequently diagnosed with FD. Enzyme replacement therapy using 1.0 mg/kg agalsidase-β was initiated. During the fifth administration, he developed ventr

SUBMITTER: Kodama K 

PROVIDER: S-EPMC7042139 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature