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BACKGROUND Bicuspid aortic valve (BAV) represents the most common cardiac congenital malformation in the adult age. It is frequently associated with dilatation, aneurysm and dissection of the ascending aorta. The purpose of the following study was to evaluate in patients with BAV: 1) the elastic properties of the ascending aorta, 2) the mechanical function(More)
OBJECTIVE To evaluate the mid-term results of endovascular stent-grafting for type B aortic dissection, in comparison with those of standard medical therapy in uncomplicated cases. METHODS Between January 1999 and 2004, among 56 patients (mean age 59.5+/-11.5 years) with type B aortic dissection, hypotensive medical therapy was the only treatment in 28(More)
OBJECTIVES This study sought to identify risk factors for rapid growth of the ascending aorta in patients with bicuspid aortic valve (BAV) disease, taking into account its phenotypic variability. BACKGROUND Phenotypic heterogeneity of BAV-related aortopathy has recently been widely recognized. However, few studies have addressed the determinants of aortic(More)
AIMS To evaluate if obesity has an additional negative impact on left ventricular (LV) geometry and function in normotensive pediatric patients >12 months after successful treatment of aortic coarctation (CoA). METHODS AND RESULTS We studied 40 CoA patients (mean age 14 ± 3 years, and male sex 70%), of them 10 were obese and 30 lean. Both groups were age(More)
We report the case of a 16-year-old boy with cardiomyopathy induced by inappropriate sinus tachycardia (IST). The patient was resistant to treatment with conventional rate-decreasing medications. Therapy with the selective sinus node If current inhibitor ivabradine was started. After 3 months of ivabradine therapy, an improvement in ejection fraction and a(More)
OBJECTIVES The aim of the present study was to evaluate the safety, tolerability, clinical and haemodynamic impact of add-on sildenafil in patients with congenital heart disease (CHD)-related pulmonary arterial hypertension (PAH) and Eisenmenger physiology after failure of oral bosentan therapy. METHODS Thirty-two patients with CHD-related PAH (14 male,(More)
The right ventricle (RV) can be described in terms of 3 components: the inlet, the apex, and the infundibulum. In the normal adult, the RV shows an arrangement suited for pumping blood against low resistance, with a mass about one sixth that of left ventricle (LV) mass, and a larger volume than the LV. The RV is able to manage a progressive increase in the(More)
BACKGROUND Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without Down's syndrome. METHODS WHO(More)
BACKGROUND Patients with small ventricular septal defects (VSDs) are thought to have excellent long-term survival, although complications may not be uncommon. METHODS We identified all patients aged ≥ 16 years with native isolated VSD between January 2000 and September 2013. Clinical outcomes were retrospectively reviewed. Transthoracic echocardiograms(More)