Assessment of aortic stiffness and ventricular functions in familial Mediterranean fever

To investigate systolic and diastolic ventricular functions, aortic elastic properties and the presence of pericardial effusion in familial Mediterranean fever (FMF) patients.

A case-controlled, cross-sectional study was performed on 44 FMF patients and 27 controls. Subjects with hypertension, diabetes mellitus and hyperlipidemia were excluded. Left and right ventricular functions were measured using echocardiography including two-dimensional, M-mode, and conventional Doppler as well as pulsed wave tissue Doppler imaging (TDI). Aortic elasticity was analyzed using M-mode tracing guided by the two-dimensional echocardiography. Statistical analysis was performed using Mann Whitney U, Spearman rho correlation and Fisher's exact tests.

Age, sex, body mass index, smoking status and lipids were comparable in patients and controls (p>0.05). None of the subjects had pericarditis and/or pericardial effusion. Aortic wall properties were similar between groups (p>0.05). The TDI parameters of mitral lateral annulus revealed significantly lower Em/Am ratios in patients compared to controls [1.77 (0.6-3.4) vs. 1.79 (0.9-4.8), p=0.02]. Mitral flow propagation velocity was significantly lower in patients than healthy subjects [63 (39-100) vs. 74 (40-94) cm/s, p=0.008]. Tricuspid annular plane systolic excursion (TAPSE) was significantly reduced in FMF group than in controls [2 (1.3-2.5) vs. 2.5 (1.7-3.2) cm; p<0.001]. Eight of the patients and one control had impaired TAPSE (<2 cm; p=0.025). There was no difference regarding right ventricular diastolic dysfunction (RVDD) as assessed by using standard Doppler echocardiography (p>0.05). However, pronounced RVDD was observed in FMF patients documented by TDI (Em/Am<1; 19 patients vs. 0 controls, p<0.001).

Subclinical myocardial involvement is present in a cohort of relatively young FMF patients who were also free of classical cardiovascular risk factors. Pericardium and aorta seem to be spared during attack free periods of FMF.