Five-year follow-up of left ventricular diastolic function in systemic sclerosis patients: determinants of mortality and disease progression.

Objectives: In systemic sclerosis (SSc), left ventricular diastolic dysfunction reflects primary myocardial involvement of the disease. We aimed to assess the abnormalities of the diastolic function, analyze the characteristics of the disease progression, and investigate the prognostic value of diastolic dysfunction in SSc patients. Patients and methods: A total of 34 SSc patients (57 +/- 12 years, 31 female) were involved in the study. The following traditional or tissue Doppler parameters of left ventricular diastolic function were obtained: E/A, lateral E', E/E', left ventricular mass index (LVM index), and maximal left atrial (LA) volume index. Measurements were repeated after 5.5 years. Results: At baseline, diastolic dysfunction was found in 62% of the SSc patients. Follow-up time was 5.4 +/- 1.2 years. A total of 6 patients died of heart failure. In univariate Cox regression analysis, age (HR = 1.08, p < 0.05), LVM index (HR = 1.07, p < 0.01), lateral E' (HR = 1.57, p = 0.05), and LA volume index (HR = 1.11, p < 0.01) were predictors of survival. During the follow-up, significant increase in LA volume index (27.5 +/- 9.7 vs. 35.4 +/- 10.6 cm(3)/m(2), p < 0.001) and E/E' was found (7.6 +/- 2.5 vs. 8.7 +/- 3.8, p < 0.05) while E' did not change (9.6 +/- 2.6 vs. 9.2 +/- 1.9 cm/s, NS). The increase in LA volume index showed positive correlation (r = 0.46, p < 0.05) while the decrease in E' values showed negative correlation (r = -0.54, p < 0.01) with the duration of the SSc. Conclusion: In SSc patients, left ventricular diastolic dysfunction is highly prevalent and is associated with increased risk of mortality. Our data suggest that in the advanced phase of the disease, the myocardial fibrotic processes burns out while the increase of the filling pressure progresses continuously. (C) 2014 Elsevier Inc. All rights reserved.