Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease

J Cardiovasc Dev Dis. 2022 Jan 3;9(1):11. doi: 10.3390/jcdd9010011.

PMID: 35050221

Matthew Zada, Queenie Lo, Siddharth J Trivedi

Highlights: The goal of this study was to look at electrocardiographic (ECG) characteristics in FD patients to see if there were any gender differences, as well as to see if ECG parameters were linked to structural and functional changes on transthoracic echocardiography (TTE).

Abstract

Background: Fabry disease (FD) is an X-linked condition caused by a lack of -galactosidase A. Males (>30 years) and females (>40 years) frequently appear with cardiac symptoms, with left ventricular hypertrophy (LVH) being the most common.

Objective: The goal of this study was to look at electrocardiographic (ECG) characteristics in FD patients to see if there were any gender differences, as well as to see if ECG parameters were linked to structural and functional changes on transthoracic echocardiography (TTE).

Methods: The results of a retrospective cross-sectional investigation of 45 FD patients with concurrent ECG and TTE were compared to age and gender matched healthy controls.

Results: FD patients showed alterations in several ECG parameters particularly in males, including longer Pwave duration (91 vs. 81 ms, p = 0.022), prolonged QRS duration (96 vs. 84 ms, p < 0.001), higher R-wave amplitude in lead I (8.1 vs. 5.7 mV, p = 0.047), increased Sokolow-Lyon index (25 vs. 19 mV, p = 0.002) and were more likely to meet LVH criteria (31% vs. 7%, p = 0.006). FD patients with impaired basal longitudinal strain (LS) on TTE had a higher chance of meeting LVH criteria (41% vs. 0%, p = 0.018). Those with more advanced FD (increased LV wall thickness on TTE) were more likely to meet LVH criteria, but also had a prolonged ventricular depolarization (QRS duration 101 vs. 88 ms, p = 0.044).

Conclusion: As a result, male FD patients had greater ECG changes suggesting delayed atrial activation, delayed ventricular depolarization, and signs of LVH. ECG anomalies were linked to impaired basal LS, a TTE indication of early cardiac involvement. More severe ECG anomalies were linked to increased LV wall thickness on TTE, a sign of more advanced FD.

Keywords: Fabry disease, LV hypertrophy, basal longitudinal strain, electrocardiogram, global longitudinal strain, transthoracic echocardiography