Update on assessment and management of primary cardiac involvement in systemic sclerosis

Abstract

Primary cardiac involvement is a common and severe complication of systemic sclerosis, which may affect all of the hearts' structural components, including pericardium, myocardium, endocardium, cardiac valves, and conduction system. While cardiac disease can be clinically silent and only diagnosed in autopsy, new imaging modalities such as speckle-tracking echocardiography and cardiovascular magnetic resonance may reveal various abnormal findings in the majority of patients. Cardiovascular magnetic resonance evaluation should include assessment of left and right ventricular function, inflammation (STIR T2-weighted sequences (T2-W) for edema detection), and fibrosis (T1-weighted sequences 15 min after Gd-DTPA contrast medium injection (late-gadolinium enhancement). Notably, cardiac disease is responsible for about one-fourth of systemic sclerosis-related deaths. Systematic studies for the assessment and therapy of systemic sclerosis-related cardiac complications, as well as relevant guidelines from the European League Against Rheumatism and the American College of Rheumatology, are currently lacking. However, research advances reviewed herein allow for a better understanding of the mechanisms that alter cardiac function. Implementation of such knowledge should reduce cardiac morbidity and mortality in systemic sclerosis patients.

Keywords: Arrhythmia; Cardiovascular magnetic resonance imaging; Myocarditis; Pericarditis; Right heart involvement; Systemic sclerosis.

Fig. 1

Speckle tracking imaging in an SSc patient: cardiac ultrasound–derived speckle tracking in apical four-chamber view depicting slightly reduced longitudinal strain in all three layers (endocardium, mid-myocardium, and epicardium), suggesting early myocardial involvement in an asymptomatic systemic sclerosis patient with normal left ventricular ejection fraction.

Fig. 2

Improvement of myocarditis in an SSc patient following immunosuppressive treatment. (a) Cardiovascular magnetic resonance imaging in a systemic sclerosis patient with palpitations and dyspnea on exertion showing intramyocardial late-gadolinium enhancement in the inferior wall of the left ventricle due to myocarditis (black arrows). (b) The patient received immunosuppressive treatment with five monthly intravenous pulses of cyclophosphamide resulting in normalization of the inferior wall.

Fig. 3

Diagnostic work-up for cardiac evaluation in systemic sclerosis: in SSc patients with cardiac symptoms, clinical findings and initial assessment with electrocardiography, chest X-ray, biochemical markers (BNP/NT-pro-BNP and Troponin I), and echocardiography should guide the further need for diagnostic evaluation as described in Step 2. In asymptomatic SSc patients, screening for cardiac involvement should include physical examination at least twice a year, as well as annual electrocardiography and echocardiography. ECG = electrocardiography; CXR = chest X-ray; BNP = brain natriuretic peptide; TnI = troponin I; TDE = tissue Doppler echocardiography; LV = left ventricle; RV = right ventricle; FAC = fraction area contraction; EF = ejection fraction; VmaxTR = maximum velocity of tricuspid regurgitation; SPECT = single-photon emission computed tomography; CMR = cardiovascular magnetic resonance.

Fig. 4

Abnormal CMR findings in an asymptomatic SSc patient: cardiovascular magnetic resonance imaging in an asymptomatic systemic sclerosis patient with normal left ventricular ejection fraction. However, T1-late-gadolinium-enhanced (LGE) images show areas of intramyocardial fibrosis (white arrows).