Cardiac magnetic resonance predicts ventricular arrhythmias in scleroderma: the Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS)

Abstract

Objectives Cardiac rhythm disturbances constitute the most frequent cardiovascular cause of death in SSc. However, electrocardiographic findings are not a part of risk stratification in SSc. We aimed to translate 24 h Holter findings into a tangible risk prediction score using cardiovascular magnetic resonance. Methods The Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS) was a prospective multicentre study including 150 consecutive SSc patients from eight European centres, assessed with 24 h Holter and cardiovascular magnetic resonance, including ventricular function, oedema (T2 ratio) and late gadolinium enhancement (%LGE). Laboratory/clinical parameters were included in multivariable corrections. A combined endpoint of sustained ventricular tachycardia requiring hospitalization and sudden cardiac death at a median (interquartile range) follow-up of 1 (1.0–1.4) year was generated. Results Only T2 ratio and %LGE were significant predictors of ventricular rhythm disturbances, but not of supraventricular rhythm disturbances, after multivariable correction and adjustment for multiple comparisons. Using decision-tree analysis, we created the SAnCtUS score, a four-category scoring system based on T2 ratio and %LGE, for identifying SSc patients at high risk of experiencing ventricular rhythm disturbance at baseline. Increasing SAnCtUS scores were associated with a greater disease and arrhythmic burden. All cases of non-sustained ventricular tachycardia (n = 7) occurred in patients with the highest SAnCtUS score (=4). Having a score of 4 conveyed a higher risk of reaching the combined endpoint in multivariable Cox regression compared with scores 1/2/3 [hazard ratio (95% CI): 3.86 (1.14, 13.04), P = 0.029] independently of left ventricular ejection fraction and baseline ventricular tachycardia occurrence. Conclusion T2 ratio and %LGE had the greatest utility as independent predictors of rhythm disturbances in SSc patients.

The Double-Edged Sword of T1-Mapping in Systemic Sclerosis—A Comparison with Infectious Myocarditis Using Cardiovascular Magnetic Resonance

“…Cardiovascular magnetic resonance (CMR) has already been successfully used for evaluating cardiac involvement in SSc [6][7][8]. CMR can detect myocardial oedema and replacement fibrosis using T2-weighted short tau imaging (STIR-T2) and late gadolinium enhancement (LGE) imaging respectively [9,10].…”

Arrhythmias and Conduction Disturbances in Autoimmune Rheumatic Disorders

“…[ 2 , 23 ] Patients with SSc have been found to have a higher mean heart rate (81 ± 11 BPM), with more cases of sinus tachycardia reported in limited SSc than in diffuse SSc. [ 19 , 21 – 23 ] Myocardial fibrosis that disrupts the normal electrical connectivity of cardiac tissue, left atrial dilation secondary to a higher prevalence of pulmonary hypertension, and more severe mitral and tricuspid regurgitation, have been proposed as possible underlying mechanisms for SA in SSc. [ 19 ]…”

Updating the Risk Stratification for Sudden Cardiac Death in Cardiomyopathies: The Evolving Role of Cardiac Magnetic Resonance Imaging. An Approach for the Electrophysiologist

“…Finally, SCD was observed in various infiltrative diseases such as sarcoidosis and systemic sclerosis [111]. Specifically, in systemic sclerosis, the presence of oedema and LGE are predictive factors for arrhythmia development [112].…”