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SUMMARYThe incidence and nature of cardiac involvement in Behçet's disease are not yet clearly documented. We first used transesophageal echocardiography in combination with resting and signal averaged electrocardiography to define cardiac involvement in Behçet's patients. Transthoracic and multiplane transesophageal echocardiography, and resting and signal averaged electrocardiography were performed in 35 Behçet's disease patients (9 women and 26 men, mean age: 38±12 years) and 30 normal subjects. Higher incidences of interatrial septum aneurysm (31% to 6%), mitral valve prolapse (25% to 3%), mitral regurgitation (40% to 6%) and aneurysmal dilatations of sinus valsalva and ascendan aorta were observed in the Behçet's disease patients than in the normal subjects. Mean QT dispersion and mean corrected QT dispersion values were significantly greater in the patients with Behçet's disease. Patients with interatrial septum aneurysm (and/or PFO), valvular dysfunction or proximal aorta dilatation had greater QT dispersion values than thase without these pathologies in the Behçet's group (63±11 vs 44±19 ms, 58±23 vs 41±24 and 60±27 vs 42±23 ms respectively, P<0.05). Positive signal averaged electrocardiography parameters were detected in 18 (51%) Behçet's disease patients compared with one (3%) in controls (P<0.001). Dilatation of the proximal aorta, interatrial septal aneurysm, mitral valve prolapse, and mitral regurgitation are the common findings of cardiac involvement in Behçet's disease. Increased dispersion of ventricular repolarisation and positive late potentials are also detected. QT dispersion is significantly higher in patients with these cardiac abnormalities. These findings suggest that cardiac involvement in this disorder is a diffuse process which involves both cardiac structure and vascular elements. (Jpn Heart J 2002; 43: 389-398) Key words: Echocardiography, Electrocardiography, Behçet's disease BEHÇET'S disease is known as a chronic relapsing, inflammatory process manifesting recurrent oral and genital aphthous ulcerations. Eye inflammation, skin lesions of erythema nodosum and acneiform eruptions, joint, central nervous system, large vessel and gastrointestinal system tract involvement are also seen in From the

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Background: Secondary pulmonary hypertension (PH) and cor pulmonale are the major clinical cardiovascular complications affecting prognosis in patients with chronic obstructive pulmonary disease (COPD). It is also known that endothelin-1 (ET-1) is a potent vasoconstrictor peptide produced by the pulmonary vascular endothelium, and ET-1 may be implicated in the pathogenesis of PH. Objectives: The purpose of this study was to investigate the presence of ET-1 in patients with COPD and to assess the correlation of ET-1 levels in the plasma and bronchoalveolar lavage (BAL) fluid (BALF) in COPD patients with or without PH. Methods: Twenty-two patients with COPD and 15 healthy controls were enrolled in the study. Peripheral venous blood samples were collected in all patients and controls. BAL was obtained in COPD patients, and ET-1 levels were measured by radioimmunoassay in all plasma and BALF samples. Results: Plasma ET-1 levels were 2.46 ± 0.55 and 1.70 ± 0.42 pmol/dl in patients with COPD and controls, respectively (p < 0.0001). Sixteen of the 22 patients with COPD (73%) had PH established by echocardiography. The ET-1 level in these patients amounted to 2.59 ± 0.50 pmol/dl, and it was 2.10 ± 0.54 pmol/dl in 6 patients with COPD without PH. In COPD patients with and without PH, BALF ET-1 levels were 0.19 ± 0.08 and 0.24 ± 0.01 pmol/dl, respectively (p > 0.05). Conclusions: These results suggest that ET-1 is detectable in both the peripheral blood and BALF of COPD patients, but the levels do not statistically differ between patients with and without PH.