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

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          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 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.

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          Most cited references7

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          Native T1 Mapping and Extracellular Volume Mapping for the Assessment of Diffuse Myocardial Fibrosis in Dilated Cardiomyopathy

          Kakuya Kitagawa, Hajime Sakuma, Masaaki Ito … (2018)
          The purpose of this study was to examine the histological correlation of native myocardial T1 and extracellular volume fraction (ECV) measurement at 3-T for the assessment of diffuse pathological changes in the myocardial tissue, including myocardial fibrosis and extracellular space in dilated cardiomyopathy (DCM).
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            Angina in fabry disease reflects coronary small vessel disease.

            Andrea Frustaci, Andrea Russo, Gaetano Tanzilli … (2008)
            Chest pain is frequently reported in Fabry disease (FD). However, its mechanism and clinical relevance are unclear. Basal troponin I level, exercise stress test, single-photon emission computed tomography imaging with (99m)Tc sestamibi, coronary angiography with thrombolysis in myocardial infarction (TIMI) frame count and left ventricular angiography and endomyocardial biopsy were obtained in 13 patients with FD with angina. Ratio of external to lumen diameter of intramural arteries (E/L ratio), myocyte diameter, and extent of fibrosis were morphometrically evaluated by using tissue sections. Controls for coronary angiography and histology were 25 patients with FD without angina and 20 mitral stenosis patients with normal left ventricular function. Troponin I level was elevated in 6 of the 13 patients. Exercise stress test showed evidence of myocardial ischemia, and single-photon emission computed tomography was positive for stress-induced perfusion defects in all patients with FD with angina. Epicardial coronaries were structurally normal but showed slow flow in all and were associated with aneurisms of posterior left ventricular wall in 3 cases. Histology showed remarkable lumen narrowing of most intramural arteries (mean E/L ratio=3.5+/-1.2; P<0.001 versus both control groups), because of hypertrophy and proliferation of smooth muscle and endothelial cells, both engulfed by glycosphingolipids. Replacement fibrosis exceeded that of both controls (P<0.001). Small vessel disease correlated with coronary slow flow and extent of fibrosis, but did not with patients' age, sex, and degree of left ventricular hypertrophy. patients with FD with angina have perfusion defects, slow coronary flow, and luminal narrowing of intramural arteries. Small vessel disease may contribute to symptomatic limitation and progressive myocardial dysfunction.
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              Usefulness of an Implantable Loop Recorder to Detect Clinically Relevant Arrhythmias in Patients With Advanced Fabry Cardiomyopathy.

              Frank Weidemann, Sebastian Maier, Stefan Störk … (2016)
              Patients with genetic cardiomyopathy that involves myocardial hypertrophy often develop clinically relevant arrhythmias that increase the risk of sudden death. Consequently, guidelines for medical device therapy were established for hypertrophic cardiomyopathy, but not for conditions with only anecdotal evidence of arrhythmias, like Fabry cardiomyopathy. Patients with Fabry cardiomyopathy progressively develop myocardial fibrosis, and sudden cardiac death occurs regularly. Because 24-hour Holter electrocardiograms (ECGs) might not detect clinically important arrhythmias, we tested an implanted loop recorder for continuous heart rhythm surveillance and determined its impact on therapy. This prospective study included 16 patients (12 men) with advanced Fabry cardiomyopathy, relevant hypertrophy, and replacement fibrosis in "loco typico." No patients previously exhibited clinically relevant arrhythmias on Holter ECGs. Patients received an implantable loop recorder and were prospectively followed with telemedicine for a median of 1.2 years (range 0.3 to 2.0 years). The primary end point was a clinically meaningful event, which required a therapy change, captured with the loop recorder. Patients submitted data regularly (14 ± 11 times per month). During follow-up, 21 events were detected (including 4 asystole, i.e., ECG pauses ≥3 seconds) and 7 bradycardia events; 5 episodes of intermittent atrial fibrillation (>3 minutes) and 5 episodes of ventricular tachycardia (3 sustained and 2 nonsustained). Subsequently, as defined in the primary end point, 15 events leaded to a change of therapy. These patients required therapy with a pacemaker or cardioverter-defibrillator implantation and/or anticoagulation therapy for atrial fibrillation. In conclusion, clinically relevant arrhythmias that require further device and/or medical therapy are often missed with Holter ECGs in patients with advanced stage Fabry cardiomyopathy, but they can be detected by telemonitoring with an implantable loop recorder.
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                Author and article information

                Contributors
                Flavio D'Ascenzi: Role: Handling Editor
                Rahul Mukherjee: Role: Editor
                Borislav Dinov: Role: Editor
                Mohammed Majiid Akhtar: Role: Editor
                Ross Thomson: Role: Editor
                Journal
                Journal ID (nlm-ta): Eur Heart J Case Rep
                Journal ID (iso-abbrev): Eur Heart J Case Rep
                Journal ID (publisher-id): ehjcr
                Title: European Heart Journal: Case Reports
                Publisher: Oxford University Press
                ISSN (Electronic): 2514-2119
                Publication date Collection: December 2019
                Publication date (Electronic): 21 October 2019
                Publication date PMC-release: 21 October 2019
                Volume: 3
                Issue: 4
                Pages: 1-5
                Affiliations
                [1 ] Department of Cardiology, Nagahama Red Cross Hospital , 14-7, Miyamae-cho, Nagahama-shi, Shiga 526-0053, Japan
                [2 ] Department of Cardiovascular Medicine, Shiga University of Medical Science Hospital , Tsukinowa, Seta-cho, Otsu-shi, Shiga 520-2134, Japan
                Author notes
                Corresponding author. Tel: +81 749 63 2111, Email: kodamak0101@ 123456gmail.com
                Author information
                http://orcid.org/0000-0003-4393-5191
                http://orcid.org/0000-0002-5372-1624
                http://orcid.org/0000-0001-7686-4364
                Article
                Publisher ID: ytz192
                DOI: 10.1093/ehjcr/ytz192
                PMC ID: 7042139
                SO-VID: 5945831c-2175-4f64-b317-3d4a937b7f8e
                Copyright © © The Author(s) 2019. Published by Oxford University Press on behalf of the European Society of Cardiology.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                Date received : 14 March 2019
                Date revision received : 2 April 2019
                Date accepted : 01 October 2019
                Page count
                Pages: 5
                Categories
                Subject: Case Reports

                Keywords: fabry disease,vasospastic angina pectoris,ventricular fibrillation,case report
                Data availability:
                Keywords: fabry disease, vasospastic angina pectoris, ventricular fibrillation, case report

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