BACKGROUND Marfan syndrome (MFS) is a heritable disorder of connective tissue, affecting principally skeletal, ocular, and cardiovascular systems. The most life-threatening manifestations are aortic aneurysm and dissection. We investigated changes in the proteome of aortic media in patients with and without MFS to gain insight into molecular mechanisms leading to aortic dilatation. METHODS AN...

A42-year-old man was referred to our hospital for evaluation of systolic and diastolic murmurs at the left sternal border. Transthoracic 2D echocardiography revealed a quadricuspid pulmonary valve (Figure 1A) and a bicuspid aortic valve (Figure 1B). Transvalvular flow velocities at the pulmonary and aortic valves were 1.8 m/s and 1.7 m/s, respectively, suggesting mild pulmonary stenosis. Additi...

INTRODUCTION Bicuspid aortic valve disease (BAV) is the most common congenital cardiac malformation occurring in 1-2% of the general population [1] [2]. Normally, the aortic valve is separated into 3 valves. However, BAV involves fusing of the valvular leaflets during development, resulting in a 2-valve structure instead of the normal 3-valve structure. BAV predisposes patients to a thoracic ao...

Transcatheter aortic valve implantation (TAVI) is an established choice for the treatment of severe stenosis (AS) in patients who are deemed inoperable or at high surgical risk. There limited data safety and effi cacy method with bicuspid (BAV), most common congenital defect. Patients BAV were excluded from randomized clinical trials (RCTs), comparing TAVI to surgery due heterogeneous anatomy t...

We have described some of the cardiological findings in 66 patients with Williams-Beuren syndrome and analysed the two dimensional cross sectional echocardiograms in 61 of them in comparison with normal controls. Supravalvar aortic narrowing was shown in all patients examined echocardiographically and may be a useful diagnostic sign. We documented a 7.8% incidence of systemic hypertension, a 15...

We report a case of infective endocarditis due to Neisseria sicca complicated by intracranial and popliteal aneurysms and hepatic and splenic infarcts in a patient with a bicuspid aortic valve. No predisposing factor other than poor dental condition was found. The patient fully recovered after antibiotic therapy, aortic and mitral valve replacement, endovascular occlusion of the middle-cerebral...

Introduction Treatment guidelines for the thoracic aorta concentrate on size, yet acute aortic dissection or rupture can occur when aortic size is below intervention criteria. Functional imaging and computational techniques are a means of assessing haemodynamic parameters involved in aortic pathology. Sources of data Original articles, reviews, international guidelines. Areas of agreement C...

Noirhomme P et al. Valve sparing-root replacement with the reimplanta-tion technique to increase the durability of bicuspid aortic valve repair. David valve-sparing aortic root replacement: equivalent mid-term outcome for different valve types with or without connective tissue disorder. RS et al. Results of matching valve and root repair to aortic valve and root pathology. Repair of the bicuspi...