Original articleSystolic and diastolic function in patients with systemic sclerosis
Introduction
Systemic sclerosis is a connective tissue disorder of unknown etiology that affects multiple organs. It is characterized by fibrosis of the skin and other visceral organs such as heart, kidneys and lungs.
The pathogenesis of the heart involvement is not yet fully understood, but its complications usually include heart failure, arrhythmias and sudden cardiac death. Symptoms and signs of the heart involvement appear through the course of the disease, but one should not wait for their appearance to diagnose and treat these conditions. The early detection of cardiopulmonary involvement in SSc is clearly desirable, both for optimal treatment and for implementation of preventive measures in the early stages of the disease.
Previous echocardiographic studies in patients with SSc have reported a variable incidence of left ventricular hypertrophy with an uncertain relationship to hypertension [1]. Armstrong et al. then showed that in SSc subclinical changes occur in the myocardium even as early as the onset of the Raynaud's phenomenon [2]. Since then, there is increasing evidence that subclinical diastolic dysfunction, involving both left and right ventricles, occurs in various stages of the disease, often without clinically evident myocardial disease [3], [4], but there is still little evidence showing that the diastolic dysfunction could be an early marker of myocardial involvement in patients with SSc.
Autopsy studies showed patchy myocardial fibrosis in both ventricles despite the absence of serious coronary artery lesions [5]. Diastolic dysfunction is related with myocardial fibrosis which starts mainly in subendocardium and correlates with longitudinal myofibrils function [6].
Our study was designed to find correlations between the degree of cardiac involvement and the parameters that quantify the disease severity. We used Doppler echocardiography along with Tissue Doppler imaging (TDI). This is a modern and noninvasive ultrasound technique, easy to use, which allows the measurement of tissue velocities [7]. To be sure that the cardiac involvement was not due to other conditions we excluded from the study the patients with conditions that would affect the heart function such as diabetes mellitus, hypertension, renal involvement and any other systemic disease.
Section snippets
Patients with systemic sclerosis
Twenty consecutive patients (18 women and 2 men) with systemic sclerosis (SSc) according to the previously defined criteria for SSc (2, 3) were studied, according to the following inclusion criteria: age < 65 years; normal chest radiographs and normal left ventricular systolic function at echocardiography. In all cases, rest ECG was normal.
Exclusion criteria: chronic renal failure, diabetes, malignancy, pregnancy, high blood pressure, ischemic or rheumatic heart disease, cardiomyopathy or
Results
From the total of 20 patients with SSc, 4 had mild pulmonary hypertension and none had heart failure. Table 1 shows the clinical, epidemiological and serological findings of all 20 SSc patients. Among clinical manifestations teleangectasia, sclerodactyly, Raynaud's phenomenon, and lung involvement were the most frequently found. In all patients the presence of one or more serum autoantibodies was recorded. The mean duration of recognized disease refers to the SSc symptoms as seen by the
Discussion
Heart involvement in SSc patients is often clinically occult and its detection depends on the sensitivity of the diagnostic method used. It has been estimated that SSc heart disease is clinically evident in only 20–25% of cases [13]. The discrepancy between the percentage of symptomatic heart involvement and necropsy SSc myocardial fibrosis should be improved by the introduction of more sensitive and non-invasive diagnostic tools [14].
Our results emphasize that the diastolic dysfunction appears
Conclusions
Despite the fact that the global left ventricular systolic function was preserved and the LV mass was normal, we found diastolic dysfunction in both right and left ventricles, which had significant correlations with disease duration and Raynaud's phenomenon. This can be detected earlier by a modern and easy to use technique, TDI, which should be performed ideally in asymptomatic patients prior to the development of severe cardiomyopathy.
Learning points
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Diastolic function in systemic scleroderma is altered also in the presence of normal systolic function.
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The assessment of diastolic function is more accurate with tissue Doppler imaging.
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It is important to evaluate all the patients with systemic sclerosis before the onset of the symptoms.
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