The primary cardiomyopathy of systemic sclerosis on cardiovascular magnetic resonance imaging

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Abstract
Aims: Cardiac disease in systemic sclerosis (SSc) may be primary or secondary to other disease manifestations of SSc. The prevalence of the primary cardiomyopathy of SSc is unknown. Cardiovascular magnetic resonance (CMR) imaging can help accurately determine the presence and cause of cardiomyopathy. We aimed to investigate the prevalence, the CMR features, and the prognostic implications of the primary cardiomyopathy of SSc. Methods and results: We conducted a retrospective cohort study of consecutive patients with SSc who had a clinical CMR for suspected cardiac involvement. We identified the prevalence, the CMR features of the primary cardiomyopathy of SSc, and its association with the long-term incidence of death or major adverse cardiac events (MACEs): heart failure hospitalization, ventricular assist device implantation, heart transplantation, and sustained ventricular tachycardia. Of 130 patients with SSc, 80% were women, and the median age was 58 years. On CMR, 22% had an abnormal left ventricular ejection fraction, and 40% had late gadolinium enhancement (LGE). The prevalence of the primary cardiomyopathy of SSc was 21%. A third of these patients had a distinct LGE phenotype. Over a median follow-up of 3.6 years after the CMR, patients with the primary cardiomyopathy of SSc had a greater incidence of death or MACE (adjusted hazard ratio 2.01; 95% confidence interval 1.03–3.92; P = 0.041). Conclusion: The prevalence of the primary cardiomyopathy of SSc was 21%, with a third demonstrating a distinct LGE phenotype. The primary cardiomyopathy of SSc was independently associated with a greater long-term incidence of death or MACE.

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