PMID- 32123796
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220413
IS  - 2514-2119 (Electronic)
IS  - 2514-2119 (Linking)
VI  - 3
IP  - 4
DP  - 2019 Dec
TI  - Ventricular fibrillation associated with vasospastic angina pectoris in Fabry 
      disease: a case report.
PG  - 1-5
LID - 10.1093/ehjcr/ytz192 [doi]
AB  - 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-beta was initiated. During the fifth administration, he developed 
      ventricular fibrillation (VF). Electrocardiography conducted immediately before 
      VF revealed ST elevation in the inferior leads with reciprocated ST depression. 
      Cardiac magnetic resonance imaging showed no LGE in the myocardium. Coronary 
      angiography showed no organic stenosis; moreover, coronary spasms were induced by 
      an intracoronary acetylcholine injection. Ventricular fibrillation was not 
      observed as the patient received calcium antagonists. DISCUSSION: This report 
      suggests that vasospastic angina pectoris is associated with life-threatening 
      arrhythmias in patient with FD without LGE.
CI  - (c) The Author(s) 2019. Published by Oxford University Press on behalf of the 
      European Society of Cardiology.
FAU - Kodama, Kenji
AU  - Kodama K
AUID- ORCID: 0000-0003-4393-5191
AD  - Department of Cardiology, Nagahama Red Cross Hospital, 14-7, Miyamae-cho, 
      Nagahama-shi, Shiga 526-0053, Japan.
FAU - Ozawa, Tomoya
AU  - Ozawa T
AD  - Department of Cardiovascular Medicine, Shiga University of Medical Science 
      Hospital, Tsukinowa, Seta-cho, Otsu-shi, Shiga 520-2134, Japan.
FAU - Dochi, Kenichi
AU  - Dochi K
AUID- ORCID: 0000-0002-5372-1624
AD  - Department of Cardiology, Nagahama Red Cross Hospital, 14-7, Miyamae-cho, 
      Nagahama-shi, Shiga 526-0053, Japan.
FAU - Ueno, Yoshiki
AU  - Ueno Y
AUID- ORCID: 0000-0001-7686-4364
AD  - Department of Cardiology, Nagahama Red Cross Hospital, 14-7, Miyamae-cho, 
      Nagahama-shi, Shiga 526-0053, Japan.
LA  - eng
PT  - Case Reports
DEP - 20191021
PL  - England
TA  - Eur Heart J Case Rep
JT  - European heart journal. Case reports
JID - 101730741
PMC - PMC7042139
OTO - NOTNLM
OT  - Case report
OT  - Fabry disease
OT  - Vasospastic angina pectoris
OT  - Ventricular fibrillation
EDAT- 2020/03/04 06:00
MHDA- 2020/03/04 06:01
PMCR- 2019/10/21
CRDT- 2020/03/04 06:00
PHST- 2019/03/14 00:00 [received]
PHST- 2019/04/02 00:00 [revised]
PHST- 2019/10/01 00:00 [accepted]
PHST- 2020/03/04 06:00 [entrez]
PHST- 2020/03/04 06:00 [pubmed]
PHST- 2020/03/04 06:01 [medline]
PHST- 2019/10/21 00:00 [pmc-release]
AID - ytz192 [pii]
AID - 10.1093/ehjcr/ytz192 [doi]
PST - epublish
SO  - Eur Heart J Case Rep. 2019 Oct 21;3(4):1-5. doi: 10.1093/ehjcr/ytz192. 
      eCollection 2019 Dec.