Systemic sclerosis & Raynaud's

Sudden cardiac death, arrhythmias and abnormal electrocardiography in systemic sclerosis: A systematic review and meta-analysis

Semin Arthritis RheumPublished Systematic review and meta-analysis

Authors' conclusion

The incidence of SCD in SSc is estimated to be 1.0-3.3% annually, at least 10-fold higher than general population estimates.

Study context

Population
Participants with SSc in English-language studies reporting incidence/frequency of sudden cardiac death (SCD), arrhythmia and electrocardiography (ECG) abnormalities; MEDLINE/EMBASE to January 2023
Participants
13,609 participants79 studies
Comparison
SSc cohorts with known/suspected SSc-associated heart involvement (SHI), without SHI, and unselected; vs healthy controls where reported
Outcome
Frequency of sudden cardiac death (SCD), arrhythmia and conduction defects in SSc
Methodological detailsMethodology and outcomes were heterogeneous
  • Methodology and outcomes were heterogeneous

Pooled result

As reported in the abstract

Annual incidence of SCD
GroupValueStudiesFollow-up
SHI3.3% annually4301PY

SHI

Value3.3% annually
Studies4
Follow-up301PY
Estimated SCD incidence in cohorts without SHI (n = 1 study, 67.5PY)
≈2.9% annually
Estimated SCD incidence in general SSc cohorts unselected for cardiac disease (n = 4 studies, 1646PY)
2.0% annually

Further pooled results

OutcomeEstimate
Arrhythmias without SHI vs healthy controlsWithout SHI vs Healthy controls · NSVT; n = 2times more frequently 13.3 (95% CI 2–102)
Arrhythmias without SHI vs healthy controlsWithout SHI vs Healthy controls · paroxysmal supraventricular tachycardia; n = 4times more frequently 7 (95% CI 3–15)
Unselected SSc cohorts: frequent PVCs and abnormal ECGUnselected SSc vs Healthy controls · frequent PVCs; n = 2times more likely 10.5 (95% CI 2–59)
Unselected SSc cohorts: frequent PVCs and abnormal ECGUnselected SSc vs Healthy controls · abnormal ECG; n = 2times more likely 2.5 (95% CI 1–4)

Ambulatory ECG abnormalities in SHI cohorts

Non-sustained ventricular tachycardia (NSVT) (n = 4, 95%CI3.2-39.3%)
18%
Frequent premature ventricular complexes (PVCs) (n = 1, 95%CI34.8-93.3%)
70%
Atrial fibrillation (AF) (n = 1, 95%CI4.2-13.6%)
8%

Other ambulatory ECG abnormalities in cohorts without SHI

NSVT (n = 7, 95%CI6-14%)
9%
>1000 PVCs/24 h (n = 2, 95%CI1-13%)
6%
AF (n = 5, 0-21%)
7%

Original publication

Sudden cardiac death, arrhythmias and abnormal electrocardiography in systemic sclerosis: A systematic review and meta-analysis. Semin Arthritis Rheum. Published 19 June 2023. doi:10.1016/j.semarthrit.2023.152229

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