[Rare case of incomplete Shone syndrome in adult man with marfanoid morphotype]. / Un cas rare de complexe de Shone incomplet chez un homme de 35 ans avec un morphotype marfanoïde et revue de la littérature.

Shone syndrome is a rare and complex congenital heart disease. It is characterized in its complete form, by a constellation of the four potentially obstructive lesions of the left heart side. Incomplete Shone's complex includes 2 or 3 of the characteristic lesions which, include a supravalvular mitral membrane, valvular mitral stenosis (MS) by a parachute mitral valve (PMV), subaortic stenosis and aortic coarctation (Coa). We describe here, the case of a 35-year-old man, hospitalized for chronic dyspnea and in whom, the diagnosis of incomplete Shone's complex is retained by echocardiographic analysis. Observed lesions include a parachute mitral valve responsible for mild regurgitation and moderate mitral stenosis, bicuspid aortic valve, and pseudo interruption of the aorta with dilatation of the ascending aorta and a patent ductus arteriosus. This case is the 2nd on 3942 patients in Drighil congenital heart disease registry.

[Aortic stenosis: An update]. / Rétrécissement aortique : mise au point.

Aortic stenosis remains one of the most frequent valvulopathy worldwide, burdened with great mortality and morbidity, and for which there is not yet an effective preventive approach, although the pathophysiological mechanisms involved in its development are better understood nowadays. Its cure, however, has been revolutionized in the last decade by the advent of transcatheter aortic valve implantation, or TAVI (also named transcatheter aortic valve replacement or TAVR). The technique of TAVI has been refined and its indications has been extended, following the publication of large randomized controlled trials where it was compared to surgical aortic valve replacement with favorable results. Consequently, transfemoral TAVR has become the first line of treatment in case of symptomatic severe aortic valve stenosis. In this review, we describe the pathophysiological mechanisms leading to severe aortic stenosis and the main ongoing randomized controlled trials targeting them. We describe the indication for surgical or percutaneous aortic valve replacement and the main complications following the procedure.

Ten-year follow-up of unreplaced Valsalva sinuses after aortic valve replacement in bicuspid aortic valve disease.

BACKGROUND: Aortopathy is common in patients with bicuspid aortic valve (BAV). AIM: To evaluate the fate of unreplaced Valsalva sinuses in patients with BAV, 10 years after aortic valve replacement (AVR) with or without replacement of the ascending aorta (RAA). METHODS: We retrospectively reviewed all surgical patients with BAV who were operated on between January 2005 and December 2007. Patients who underwent AVR with or without RAA were included. Surgical data were entered prospectively. Ten-year clinical and echocardiographic follow-up data as well as survival data were collected by contacting the patients and their personal cardiologists, and by consulting the French national mortality registry. Overall, 25% of the patients had computed tomography angiographic assessment of the aortic root at follow-up. RESULTS: A total of 133 patients with BAV were operated on within the selected period. Thirty-two patients did not meet the inclusion criteria, and had primary Valsalva sinus surgery. Twenty-four patients underwent AVR with RAA and 77 patients had isolated AVR; all of these 101 patients were included in the study. The median follow-up was 9 years (up to 12 years). During follow-up, eight patients (7.9%) underwent late reoperation; two of them (2.0%) required root surgery. Ten-year freedom from reoperation was 86.2±4.7%. Ten-year freedom from dilatation of the Valsalva sinuses (>45mm) was 86.6±5.2%. Ten-year cumulative survival was 83.5±4%. CONCLUSIONS: Ascending aorta and Valsalva sinuses seem to have different fates after AVR in BAV disease. When the Valsalva sinuses are not dilated at the initial surgery, the risk of secondary dilatation at 10 years is low. Preservation of the sinuses is therefore justified in patients with BAV with a non-dilated root. In BAV with isolated aortic insufficiency, a more aggressive approach may be justified, especially in young patients.