Heart involvement in systemic sclerosis: an ultrasonic tissue characterisation study

BACKGROUND—Clinicoepidemiological findings indicate that symptomatic heart involvement in patients with systemic sclerosis (SSc) predicts a very poor prognosis. At necropsy studies, SSc heart involvement without significant coronary lesions is characterised by patchy myocyte necrosis and contraction band necrosis with collagen replacement leading to myocardial fibrosis. There is a discrepancy between the frequency of clinically evident myocardial disease (25%) and autoptical myocardial fibrosis (81%). OBJECTIVE—The aim of this study was to detect preclinical myocardial alterations in SSc patients by ultrasonic videodensitometric analysis. METHODS—Thirty five SSc patients (three male, aged 48.6 (11) SD years, range 22-65) with normal ventricular function and 25 age and sex matched healthy controls were studied. All patients had a negative maximal exercise stress; in all cases arterial hypertension, renal involvement, and diabetes were excluded. Echocardiographic images were digitised by a real time videodigitiser (Tomtec Imaging Systems). Quantitative texture analysis was performed on data from the septum and the posterior wall, obtaining mean gray level histogram (MGL) at both end-diastole (d) and end-systole (s). The cyclic variation index (CVI), was calculated according to the formula ((MGLd−MGLs)/MGLd) × 100. Left ventricular mass (LVM), body surface corrected, was calculated according to Penn convention. RESULTS—Comparable systolic and diastolic blood pressure, LVM, diastolic and systolic function were recorded in both SSc patients and controls. In contrast, in SSc patients the CVI, which is the expression of the intrinsic myocardial structural function, was significantly lower than in controls (septum: −18 (28)% v 35 (10)%, p