Incidence and predictors of cardiac arrhythmias in patients with systemic sclerosis
Authors: Stronati G , Benfaremo D , Selimi A , Ferraioli Y , Ferranti F , Dello Russo A , Guerra F 
Published: May 2022

Abstract

Funding Acknowledgements
Type of funding sources: None.

Introduction
Heart involvement in systemic sclerosis patients is frequent and represents a negative prognostic factor with around 25% of SSc patients dying from cardiovascular related causes. Cardiac arrhythmias represent 6% of the overall causes of death in SSc patients. Not only a plethora of conditions typical of the disease seem to favour the presence of an arrhythmic substrate such as microvascular disease or fibrosis but autoimmunity itself has been recognized as a pathogenic mechanism for cardiac arrhythmias.

Purpose
The aim of our study was to investigate the incidence of arrhythmias in patients with systemic sclerosis and identify potential predictors.

Methods
Prospective longitudinal study enrolling all consecutive patients with a diagnosis of SSc and no overt cardiac disease nor pulmonary hypertension. Echocardiographic parameters and GLS were obtained at baseline and at each follow up as well as 12 lead ECG. Presence of atrial fibrillation (AF), atrial tachycardia (AT), ventricular ectopic beats &gt;1000/24 h (VEB), supraventricular ectopic beats (SVEB), bundle branch block (BBB) and atrioventricular block (AVB) was registered.

Results
160 patients (144 females, 90%, mean age 59±14 years) were enrolled from June 2016 to December 2021. At enrolment, 11.3% of patients with SSc presented a previous history of supraventricular arrhythmia (5.6% supraventricular ectopic beats, 3.1% atrial fibrillation, 2.5% atrial flutter) and 5.6% a history of ventricular arrhythmia (5.0% ventricular ectopic beats, 0.6% ventricular tachycardia). After a median follow-up of 3.1 years (95% CI 1.4-4.8 years), five patients (3.1%) died of cardiovascular causes, of which three (1.8%) experienced a sudden cardiac death. During the same period, 16 patients (10%) presented a new diagnosis of supraventricular arrythmia (8.7% atrial fibrillation, 1.3% atrial flutter) and two patients (1.2%) a new diagnosis of ventricular tachycardia.
PR interval significantly increased during the 3.1-year follow-up (154±27 vs. 178±32 ms; p=0.013), as well as corrected QT interval (420±23 vs. 436±19 ms; p=0.001). New diagnosis of complete right and left bundle branch block was made in 18 (11.2%) and two (1.2%) of all SSc patients.
Diagnosis of pulmonary arterial hypertension was associated with a 4-fold and a 10-fold increased risk of supraventricular and ventricular arrhythmias, respectively (Figure 1). A diffuse SSc variant (p=0.049), indexed right (p=0.020) and left atrial volumes (p=0.035), and E/E’ ratios (p=0.016) were all associated with an increased risk of supraventricular arrhythmias. TAPSE (p=0.040), as well as right (p=0.030) global longitudinal strain were associated with an increased risk of ventricular arrhythmias.

Conclusions
Ssc patients are often by an arrhythmic profile with an increased risk of supraventricular and ventricular events as well as sudden cardiac death. A comprehensive cardiological work up may help in lowering the risk of arrhythmic complications.