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Published on: June 20, 2014
The primary cardiomyopathy of systemic sclerosis on cardiovascular magnetic resonance imaging
Sanya Chhikara1, Adinan Kanda1, Fredrick M Ogugua1
1Cardiovascular Division, Department of Medicine, University of Minnesota Medical School, 420 Delaware Street SE, MMC 508, Minneapolis, MN 55455, USA.
Insights
The primary cardiomyopathy of systemic sclerosis (SSc) affects 21% of patients. This condition is linked to increased long-term risks of death or major adverse cardiac events (MACE), highlighting the importance of cardiac assessment in SSc patients.
Area of Science:
- Cardiology
- Rheumatology
- Medical Imaging
Background:
- Cardiac involvement in systemic sclerosis (SSc) can be primary or secondary.
- The prevalence and prognostic significance of primary SSc cardiomyopathy remain unclear.
- Cardiovascular magnetic resonance (CMR) is a valuable tool for diagnosing cardiac conditions.
Purpose of the Study:
- To determine the prevalence of primary SSc cardiomyopathy.
- To characterize its features using CMR.
- To investigate its association with long-term adverse cardiac outcomes.
Main Methods:
- Retrospective cohort study of SSc patients undergoing clinical CMR.
- Analysis of CMR findings, including left ventricular ejection fraction and late gadolinium enhancement (LGE).
- Assessment of the association between primary SSc cardiomyopathy and the incidence of death or major adverse cardiac events (MACE).
Main Results:
- The prevalence of primary SSc cardiomyopathy was 21%.
- A distinct LGE phenotype was observed in one-third of these patients.
- Primary SSc cardiomyopathy was associated with a 2-fold increased risk of death or MACE over a median follow-up of 3.6 years.
Conclusions:
- The primary cardiomyopathy of SSc occurs in 21% of patients and is associated with a distinct LGE pattern in a subset.
- Primary SSc cardiomyopathy is an independent predictor of worse long-term outcomes, including death or MACE.
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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