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Cited 3 time in webofscience Cited 3 time in scopus
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Anderson-Fabry disease presenting with atrial fibrillation as earlier sign in a young patient: A case reportopen access

Authors
Kim, HangyulKang, Min GyuPark, Hyun WoongPark, Jeong-RangHwang, Jin-YongKim, Kyehwan
Issue Date
26-Jun-2021
Publisher
BAISHIDENG PUBLISHING GROUP INC
Keywords
Fabry disease; Atrial fibrillation; Electrocardiography; Cardioversion; Glycosphingolipids; Case report
Citation
WORLD JOURNAL OF CLINICAL CASES, v.9, no.18, pp 4823 - 4828
Pages
6
Indexed
SCIE
SCOPUS
Journal Title
WORLD JOURNAL OF CLINICAL CASES
Volume
9
Number
18
Start Page
4823
End Page
4828
URI
https://scholarworks.gnu.ac.kr/handle/sw.gnu/3578
DOI
10.12998/wjcc.v9.i18.4823
ISSN
2307-8960
Abstract
BACKGROUND Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder that results from a deficiency of alpha-galactosidase A enzyme activity in which glycosphingolipids gradually accumulate in multi-organ systems. Cardiac manifestations are the leading cause of mortality in patients with AFD. Among them, arrhythmias comprise a large portion of the heart disease cases in AFD, most of which are characterized by conduction disorders. However, atrial fibrillation as a presenting sign at the young age group diagnosed with AFD is uncommon. CASE SUMMARY We report a case of a 26-year-old man who was admitted with chest discomfort. Left ventricular hypertrophy was fulfilled in the criteria by the Sokolow-Lyon index and atrial fibrillation on the 12 Leads-electrocardiography (ECG) that was documented in the emergency room. After spontaneously restored to normal sinus rhythm, relationships between P and R waves, including a shorter PR interval on the ECG, were revealed. The echocardiographic findings showed thickened interventricular septal and left posterior ventricular walls. Based on the clues mentioned earlier, we realized the possibility of AFD. Additionally, we noticed the associated symptoms and signs, including bilateral mild hearing loss, neuropathic pain, anhidrosis, and angiokeratoma on the trunk and hands. He was finally diagnosed with classical AFD, which was confirmed by the gene mutation and abnormal enzyme activity of alpha-galactosidase A. CONCLUSION This case is a rare case of AFD as a presentation with atrial fibrillation at a young age. Confirming the relationship between P and Q waves on the ECG through sinus rhythm conversion may help in differential diagnosis of the cause of atrial fibrillation and hypertrophic myocardium.
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