Sclerodermic Cardiomyopathy-A State-of-the-Art Review

Abstract

Systemic sclerosis (SSc) is a chronic autoimmune disorder with unknown triggering factors, and complex pathophysiologic links which lead to fibrosis of skin and internal organs, including the heart, lungs, and gut. However, more than 100 years after the first description of cardiac disease in SSc, sclerodermic cardiomyopathy (SScCmp) is an underrecognized, occult disease with important adverse long-term prognosis. Laboratory tests, electrocardiography (ECG) and cardiovascular multimodality imaging techniques (transthoracic 2D and 3D echocardiography, cardiac magnetic resonance (CMR), and novel imaging techniques, including myocardial deformation analysis) provide new insights into the cardiac abnormalities in patients with SSc. This state-of-the-art review aims to stratify all the cardiac investigations needed to diagnose and follow-up the SScCmp, and discusses the epidemiology, risk factors and pathophysiology of this important cause of morbidity of the SSc patient.

Keywords: cardiac magnetic resonance; cardiomyopathy; echocardiography; speckle-tracking; strain; systemic sclerosis.

Figure 1

Different 2D transthoracic echocardiographic techniques used for evaluating the patients with systemic sclerosis and primary myocardial disease. Legend: Panel (A), mitral valve inflow profile by pulsed-wave (PW) Doppler, Tissue Doppler Imaging (TDI) for myocardial velocities. Panel (B), speckle-tracking echocardiography (STE) for assessing global longitudinal strain of the left ventricle (LV GLS). Panel (C), STE for assessing left atrial (LA) longitudinal strain. Panel (D), right ventricular (RV) evaluation comprised of TDI evaluation of RV free wall systolic velocity and RV free wall longitudinal strain. Images from the personal collection of the authors.

Figure 2

Most important findings by nuclear imaging in patients with systemic sclerosis and primary myocardial disease. Images from the personal collection of the authors.

Figure 3

42 years old male patient diagnosed with dcSSc, normal epicardic coronary arteries. (A) ECG tracing with trifascicular block (RBB, AFB, grade 1 AV block), (B) 2DTTE PSAX image at the base of the LV showing hyperechogenic area in the infero-basal segment of the LV (red arrow); (C) CMR image showing LGE in the infero-basal segment suggestive of subendocardial and mid-wall scar at this level (red arrow). dcSSc, diffuse cutaneous systemic sclerosis; RBB, right bundle branch block; AFB, anterior fascicular block; AV, atrio-ventricular; 2DTTE, two-dimensional transthoracic echocardiography; PSAX, parasternal short-axis view; LV, left ventricle; CMR, cardiac magnetic resonance; LGE, late gadolinium enhancement.