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ECG monitoring in patients with Fabry disease

Session Poster session 2

Speaker Zdenka Fingrova

Congress : EuroHeartCare 2018

  • Topic : arrhythmias and device therapy
  • Sub-topic : Arrhythmias, General - Other
  • Session type : Poster Session
  • FP Number : 267

Authors : Z Fingrova (Prague,CZ), S Havranek (Prague,CZ), G Dostalova (Prague,CZ), L Golan (Prague,CZ), JC Lubanda (Prague,CZ), D Rob (Prague,CZ), A Linhart (Prague,CZ)


Z Fingrova1 , S Havranek1 , G Dostalova1 , L Golan1 , JC Lubanda1 , D Rob1 , A Linhart1 , 1General University Hospital, 2nd Department of cardiology and angiology - Prague - Czech Republic ,

European Journal of Cardiovascular Nursing ( 2017 ) 17 ( Supplement ), S78

Introduction: Fabry disease (FD) is an X-linked lysosomal storage disease caused by a defect in the gene encoding lysosomal enzyme α-galactosidase. Side to hypertrophic cardiomyopathy, heart rhythm disorders are common cardiac complications of FD. The aim of study was to quantify cardiac arrhythmias by use of 24-hr ECG monitoring in patients with FD.
Methods: We performed prospective analysis of 24-hr ECG monitoring in 51 consecutive patients (27 males; 19 – 79 years) with diagnosed FD in one center.
Results: Baseline rhythm was sinus rhythm in 47 (92%) subjects. In rest of patients, 2 (4%) manifested sustained atrial fibrillation and 2 (2%) had paced rhythm for advanced AV block. Median value of ventricular premature beats was 4 (range 0 – 2632) per 24 hours. Frequent ventricular premature beats were detected in 7 (14%) patients (in one case more than one morphology). Three subjects manifested unsustain ventricular tachycardia. Median value of supraventricular premature beats was 27 (range 0 – 2477) per 24 hours. Out of 47 patients with sinus rhythm, 7 (15%) subjects suffered from frequent supraventricular premature beats and 23 (45%) patients had episodes of short supraventricular tachycardia. Four (8%) subjects had 1st and one more patient 2nd degree of AV block.
Medians (range) of heart rate variability parameters in evaluable subjects were: MEAN NN 902 (715 – 1212) ms; SDNN 133 (58 – 206) ms, pNN50 11 (0.5 – 50) % and low/high frequency ratio 1.7 (0.8 – 3.4) ms. It has been documented significant difference in low/high frequency ratio between males and females [1.9 (1.3 – 3.4) vs. 1.5 (0.8 – 2.4); p<0.01].
Conclusion: Existence of heart rhythm disorders (conduction abnormalities included) on 24-hr ECG monitoring is a result of underline serious structural heart disease.

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