Background/Purpose: Cardiac involvement is a relevant prognostic determinant in Systemic Sclerosis (SSc), but the diagnosis is often delayed due to the lack of a specific diagnostic algorithm. Objective of the present study is to define the role of a combined approach, based on evaluation of clinical symptoms, laboratory findings, EKG-holter and cardiac magnetic resonance (CMR), to characterize cardiac involvement in SSc-patients. 

Methods: Twenty-five SSc-patients with symptoms of cardiac involvement (dyspnea, palpitation) or signs of cardiac failure and elevation of cardiac enzymes (MB-CK and/or troponin T) underwent EKG-holter and cardiac magnetic resonance (CMR).  Median follow-up was 24±0.2 months. 

Results: Major EKG-holter modifications were present in 45% of patients. CMR study demonstrated T2 hyperintensity in 2 patients while none of the patients presented early gadolinium enhancement and 16 (64.0%) patients presented late gadolinium enhancement (LGE). We identified 3 different patterns of distribution of LGE: subepicardial, midwall and subedocardial. Sixteen (64.0%) patients presented almost one pattern of distribution, while 9 patients presented more than one: 81.3% of patients presented a midwall distribution of LGE, 50% of patients presented a subepicardial LGE with a linear distribution pattern and 37.5% presented a subendocardial LGE distribution. 28% of patients showed hypokinetic area and only one patient an akinetic area. The ejection fraction (EF), corrected for the age, was decreased in 7 patients (28%). The mean EF of left ventricle was 60.2±11.1%, and of right ventricle was 55.1±10.2%.  Hypokinetic and akinetic area corresponded with the LGE area. The subepicardial distribution pattern was more frequent in patients with an early disease, while patients with diffuse skin involvement presented more frequently with the subendocardial pattern. This latest distribution pattern was also associated with a reduction of EF and with major-EKG abnormalities. The extension of LGE on CMR was evaluated according to a standardized left-ventricular segmentation  (Cerqueira et al; Circulation 2002, 105:539-542). Patients with major abnormalities on EKG-holter presented a higher number of myocardial segments involved on CMR (4.8±2.3) with respect to the patients without EKG-abnormalities(2.7±0.9)(p=0.041). A weak correlation was found between NYHA-dyspnea class and the number of involved myocardial segments on CMR (R=0.5, p=0.02). After a mean follow-up of 24±0.2 months, 4 patients(16%) died for arrhythmias or heart failure. All patients who died at follow-up had severe dyspnea, elevated cardiac enzymes, myosytis,  major EKG-holter abnormalities, reduction of EF and LGE on CMR at baseline; 75% of patients who died had a subendocardial distribution pattern of LGE on CMR.

Conclusion: Our study suggests that a combined approach, based on clinical presentation, laboratory findings, EKG-holter examination and study of distribution of LGE on CMR, is useful to characterize the extension of myocardial damage and to identify patients with a poor outcome related to heart involvement in SSc.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/poor-outcome-in-patients-with-systemic-sclerosis-and-myocardial-involvement-a-combined-approach-based-on-clinical-and-laboratory-findings-ekg-holter-and-cardiac-magnetic-resonance/