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. 2020 Aug 1;59(8):1938-1948.
doi: 10.1093/rheumatology/kez494.

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

Affiliations

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

Sophie Mavrogeni et al. Rheumatology (Oxford). .

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.

Keywords: cardiovascular magnetic resonance; rhythm disturbance; scleroderma; sudden cardiac death; systemic sclerosis.

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Figures

<sc>Fig</sc>. 1
Fig. 1
The SAnCtUS scoring system Ventricular rhythm disturbances were more prevalent with increasing scores (P < 0.001). LGE: late gadolinium enhancement; OR: odds ratio; SAnCtUS: Scleroderma Arrhythmia Clinical Utility Study; ATA: anti-topoisomerase I antibodies; LVEF: left ventricular ejection fraction.

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