Cardiac involvement in systemic sclerosis: differences between clinical subsets and influence on survival

Abstract

Cardiac involvement (CI) is a known complication of SSc associated with increased mortality. Our objective was to describe a cohort of patients with SSc and CI and to assess the differences between cutaneous subsets regarding their presentation and survival. Three hundred and ninety-three Spanish patients from a single center, diagnosed with SSc, were retrospectively studied for evidence of CI using noninvasive and invasive tests from 1976 to 2011. Clinical, epidemiological, immunological and therapeutic features of patients with CI were compared to those without it and within the different cutaneous subsets of SSc. CI was present in 173 (44 %) patients. Mitral regurgitation (67 %), conduction alterations (45 %) and left ventricle diastolic dysfunction (40 %) were the most common findings. Pericardial involvement and heart failure were more frequent in diffuse SSc (dcSSc) than in limited or sine scleroderma SSc. CI accounted for 20 % of deaths, and it was an independent mortality risk factor (HR 2.1, P = 0.02), but once CI was established, classical dcSSc mortality risk factors determined mortality. Patients with dcSSc developed CI faster than limited (HR 1.9, P = 0.003) or sine SSc patients (HR 2.5, P = 0.002), specially during the first year after SSc onset. We found statistically significant differences between the 3 SSc subsets in the presentation of pericardial involvement and heart failure. CI increased the mortality and appeared at a higher rate, especially during the first year after SSc onset. Screening for heart involvement should be performed at diagnosis and during follow-up.

Autoimmune myocarditis in systemic sclerosis: an unusual form of scleroderma heart disease presentation

“…Prognosis of SSc patients with CI seems worse, particularly when it becomes clinically evident 17. According to previous reports, CI doubles the mortality rate of SSc patients and is considered to be an independent mortality risk factor along with interstitial lung disease, pulmonary hypertension, scleroderma renal crisis, and age 18…”

<p>Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis</p>

“…Cardiac dysfunction may be seen in >40% of patients with SSc and includes primary myocardial fibrosis, fibrosis of the conduction system leading to arrhythmias, microvascular and atherosclerotic coronary vessel disease and hypertensive crisis. [16][17][18] Fibrosis of the myocardium may result in either diastolic (heart failure with preserved ejection fraction which is reported in around 18% of SSc patients) or less commonly systolic heart failure (heart failure with reduced ejection fraction which is described in around 2% patients). 16 Studies comparing echocardiography findings in patients with SSc-PAH and IPAH with similar hemodynamics showed that patients with SSc-PAH are more likely to have left atrial enlargement and other indications of left ventricular diastolic impairment.…”

Pulmonary Hypertension in Systemic Sclerosis

“…The diagnosis of PVOD is important since it may be harmful to prescribe pulmonary vasodilators since these vasodilators increase the risk for developing pulmonary edema. Also, post-capillary PH is seen in patients with systemic sclerosis, merely as a result of left ventricle diastolic dysfunction due to cardiomyopathy as indicated by an elevated PCWP > 15 mmHg [24,25].…”