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.

Recent studies, screening the hearts of subclinical consecutive SSc patients by delayed enhancement (DE) – cardiac magnetic risonance (CMR), found that 21-66% had midwall DE-CMR. We studied the hearts of symptomatic SSc patients through CMR and we evaluated the role of a combined approach, based on evaluation of clinical symptoms, laboratory findings, EKG-holter and CMR, to characterize cardiac involvement in SSc-patients. 

Methods:

Thirty-six SSc-patients with symptoms of cardiac involvement (dyspnea, palpitation) and/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 30.6% of patients. CMR study demonstrated T2 hyperintensity in 3 patients while none of the patients presented early gadolinium enhancement and 18 (50.0%) patients presented late gadolinium enhancement (LGE). We identified 3 different patterns of distribution of LGE: subepicardial, midwall and subedocardial. Eleven patients presented a single pattern of distribution, while 7 patients (38.8%) presented more than one: 61.1% of patients presented a midwall distribution of LGE, 33.3% of patients presented a subepicardial LGE with a linear distribution pattern and 22.2% presented a subendocardial LGE distribution. 38% of patients showed hypokinetic area and only one patient an akinetic area. The mean EF of left ventricle was 61.7±10.8%, and of right ventricle was 58.1±10.3%.  Hypokinetic and akinetic area corresponded with the LGE area. The extension of LGE on CMR was evaluated according to a standardized left-ventricular segmentation. When CMR demonstrated a DE, the mean number of involved cardiac segments was 3.3±2.7. Segment number 9 was involved in 44.4% of the patients, followed by segments number 3 and 5 (38.9%) and by segment 2 (27.8%). Patients with major abnormalities on EKG-holter presented a higher number of involved myocardial segments on CMR (3.7±2.3) with respect to the patients without EKG-abnormalities (0.9±1.4), (p=0.012). 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/extension-of-cardiac-damage-through-the-delayed-enhancement-of-cardiac-magnetic-resonance-predictive-value-of-a-combined-approach-based-on-clinical-and-laboratory-findings-ekg-holter-and-cardiac-mag/