Echocardiographic Findings in Scleroderma without History of Cardiovascular Disease

pp. 105-109

Abstract

Introduction: Scleroderma is a connective tissue disease characterized by extensive fibrosis of the skin and various organs. Heart failure and pulmonary hypertension are cardiovascular syndromes associated with this entity that determine prognosis. Doppler echocardiography plays a key role in the anatomical and functional assessment of scleroderma and allows the diagnosis of early alterations in ventricular relaxation or segmental contraction, as well as assessing the presence and degree of pulmonary hypertension.
Objective: The aim of the study was to describe relevant echocardiographic findings in patients with scleroderma without a history of cardiovascular disease.
Methods: A cross-sectional, descriptive study was conducted in 46 patients with confirmed diagnosis of scleroderma, who underwent a routine echocardiographic study. Left ventricular systolic function (LVSF) was assessed through ejection fraction (EF). Left ventricular systolic function impairment was defined as EF < 50%, systolic diameter (LVSD) and diastolic diameter (LVDD), wall thickness, regional assynergies (RA), diastolic function (E and A waves and transmitral flow ratio, defined as normal, type I (impaired relaxation), type II (pseudonormal), and type III (restrictive), and septal and lateral tissue Doppler (LVTD) with S, E and A waves and the E/E’ ratio. The left atrial (LA) area was assessed in cm² and LA dilation was defined as area > 20 cm². Right ventricular assessment included: qualitative systolic function (RVSF), TAPSE (tricuspid annular plane systolic excursion), diastolic diameter (RVDD) at the basal level, lateral tissue Doppler (RVTD) with measurement of S, E and A waves, and diastolic function (RVDF) by means of E and A waves and tricuspid flow. Systolic pulmonary pressure (SPP) was measured from the sum of the tricuspid reflux gradient and right atrial pressure (inferior vena cava diameter and inspiratory collapse). Presence of significant valve disease was quantified.
Results: Forty-six patients with mean age of 50 ± 7.7 years were included in the study. Eighty-four percent of patients were female, 26% had hypertension and 24% smoking habits. Echocardiographic findings showed preserved LVSF in 98% of patients, mean EF of 62 ± 9.95%, interventricular septum 9.7 ± 2 mm, LVDD 43 ± 8.5 mm, and RA in 2% of patients (n = 1). Normal LVDF was present in 48% of patients, type I in 44%, type II in 2% and type III in 4%. Mean left ventricular tissue Doppler S wave was 11 ± 0.16 cm/s and values < 11 were found in 45% of cases, with an E/E’ ratio of 5 (0% > 10). The LA area was 18 ± 3.1 cm² and 24% of patients presented dilation. Normal RVSF was found in 96% of patients (n = 44), RVDD was 32 ± 3.6 mm, TAPSE 24 ± 3.1 mm, impaired RV relaxation was found in 26% of cases and RVTD S wave was 14 cm/s. Mean SPP was 40 ± 11.7 mm Hg (range 25-110 mm Hg), with diagnosis of pulmonary hypertension in 48% of patients (n = 22). An increase in SPP ≥ 50 mm Hg (moderate-severe) was present in 15% of patients (n = 7). Pericardial effusion was found in 4 patients. There was no significant valve disease.
Conclusions: The echocardiographic findings of patients with scleroderma without history of cardiovascular disease showed a high prevalence of mild LV and RV diastolic dysfunction, LA dilation, decreased LVTD S wave (lower regional longitudinal contraction), and high incidence of pulmonary hypertension (48%).