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Background Pulmonary arterial hypertension (PAH) is a life-threatening and debilitating complication of several connective tissue diseases. We aimed to evaluate the effects of long-term treatment with bosentan, an oral dual endothelin ET A /ET B receptor antagonist, in a cohort of patients with PAH related to connective tissue diseases. Material and methods In the present prospective, noncontrolled study, 13 patients (nine with systemic sclerosis, two with systemic lupus erythematosus, one with mixed connective tissue disease and one with overlap syndrome including scleroderma and myositis), mostly nonresponders to prostanoids therapy, were treated for 1 year with bosentan. Cardiac haemodynamics and the diagnosis of PAH were performed by Doppler ultrasound examination. Exercise capacity was assessed by 6-min walking test at baseline and at 3, 6 and 12 months of therapy. Results During bosentan treatment, progressive improvement of exercise capacity was observed.Walk distance increased in seven patients, remained unchanged in three and slightly decreased in three patients. A progressive significant decrease of right ventricular systolic pressure was also observed, whereas pulmonary artery mean pressure remained unchanged. Adverse effects related to bosentan (elevation of hepatic aminotransferases) were noted in two patients. Conclusion Long-term treatment with bosentan was effective in improving exercise capacity and pulmonary haemodynamics in patients with PAH related to connective tissue diseases.

Our purpose was to investigate the multi-detector-row CT angiography (MDCTA) application in endoleak detection.Fifty-nine patients that underwent endovascular aneurysm repair (EVAR) of an infrarenal abdominal aortic aneurysm with endoluminal stent graft were retrospectively studied. MDCTA scans were obtained after administration of 110-130 mL of contrast material using a 4-6 mL/sec flow rate. We made unenhanced, arterial (15-20 sec) and delayed (100 sec) acquisitions. For each patient four MDCTA datasets (pre-EVAR, 1, 6, and 12 months follow-up) were obtained. Each examination was studied by two observers. Kappa value was calculated in order to evaluate inter-observer agreement.Twenty-one endoleaks were detected in eighteen patients. Fifteen and eighteen endoleaks were detected by using biphasic arterial CT and biphasic delayed CT respectively (sensitivity of 71.4% and 85.7% respectively). We observed a positive correlation between the presence of endoleak and the increasement in aneurysm size. Interobserver agreement was 88.1% and kappa value was 0.685.Biphasic CT for endoleak detection is significantly superior to arterial and delayed phases respectively. We observed a good interobserver agreement.

We report the case of a 49-year-old man admitted to our Institute because he suffered acute abdominal pain induced by eating. Sonography don't revealed pathological findings. Then underwent a multi-detector row computed tomography angiography (MDCTA) that revealed an intimal flap separating true and false lumens that was located 2 cm from the origin of the superior mesenteric artery (SMA) and with an extension of 8 cm. Since the dissection was limited without occlusion of the SMA and the mesenteric marginal artery served as a collateral vessel on the distal side of the SMA, the treatment has been conservative. Improved CT technology facilitates the diagnosis of superior mesenteric artery dissection. Prompt diagnosis and treatment result in the lowest mortality rate and minimize the prevalence of intestinal infarction. Only 107 cases (including the present case) of isolated spontaneous SMA dissection without associated aortic dissection were identified from the literature.