Case reportElectrophysiologic findings in Fabry's disease with a short PR interval
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Cited by (60)
Screening of Fabry disease in patients with an implanted permanent pacemaker
2023, International Journal of CardiologyA retrospective investigation to establish new screening approach for the detection of patients at high risk of Fabry disease in male left ventricular hypertrophy patients
2022, Journal of CardiologyCitation Excerpt :In electrographic findings, short Pend-Q time (Pend-Q interval < 40 msec) and high voltage (SV1 + RV5 > 4.0 mV) were associated with FD in our study. A short PR interval (<120 ms) was observed in one of the five patients with FD, although some early studies on FD showed a shortened PR interval and its usefulness for detecting cardiac involvement in FD [11,12]. Niemann et al. reported that a short PR interval (<120 ms) was not relevant (around 16%) in their large cohort of patients with untreated FD in all stages of cardiomyopathy [21].
Value of Electrocardiography to Distinguish Fabry Disease from Sarcomeric Hypertrophic Cardiomyopathy
2022, American Journal of CardiologyCitation Excerpt :Cardiac arrhythmias and ECG findings are commonly reported in FD, even in prehypertrophic patients.8–10 A short PR interval is frequently described and seems to be the most common finding.9–11 Electrophysiological studies have proved that it is caused by fast conducting times within the atrium, atrioventricular node, and the intraventricular conduction system.11–14
ECG-based score estimates the probability to detect Fabry Disease cardiac involvement
2021, International Journal of CardiologyCitation Excerpt :Conflicting evidence has described shortening of PR-interval [18,22], P-wave [11,17] and PR-segment [16] as signs of FD cardiomyopathy. Atrioventricular blocks, repolarization abnormalities, prolonged QT intervals and LBBB have been reported in late FD cardiomyopathy [24]. A pre-hypertrophic FD phenotype, identified by low T1 values at CMR, has been recently related to increased Sokolow-Lyon index, higher prevalence of repolarization abnormalities, and fQRS, as compared to FD patients without signs of cardiac involvement [10,11,23].
Primary Restrictive, Infiltrative, and Storage Cardiomyopathies
2020, Diastology: Clinical Approach to Heart Failure with Preserved Ejection Fraction