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Background Behçet’s disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet’s disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options. Case presentation Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special „skirted” conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. Conclusions The surgical treatment of cardiovascular manifestations of Behçet’s disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.

Background Sinus of Valsalva aneurysm (SVA) is an uncommon cardiac anomaly, with an incidence of less than 1% of open heart surgery cases. Its evolution is most frequently silent, being found incidentally or discovered in the event of its acute rupture. Non-ruptured giant SVAs may cause unusual clinical manifestations, as a consequence of their protrusion into the heart chambers or compression of the coronary vessels and are frequently associated with aortic insufficiency of various degrees of severity. The gold standard treatment for SVAs consists of complete replacement of the aortic root and valve. However, in certain cases, valve-sparing procedures may prove to be a more suitable alternative. Case presentation A 68-year-old male patient presented with dyspnea as symptom caused by a large (> 5 cm) right sinus of Valsalva aneurysm. The aneurysm was occupying most of the right ventricle and was associated with severe aortic regurgitation. The surgical treatment of the condition involved valve-sparing root reconstruction procedure (remodeling technique), completed with external stabilization of the aortic valve annulus via running suture annuloplasty. Following the uneventful intervention, the patient did well and his status improved. The follow-up transthoracic echocardiography obtained 1 month after surgery showed a fully competent aortic valve with no regurgitation. Conclusions Despite complete aortic root and valve replacement being considered the safest approach to large SVAs complicated with aortic insufficiency, valve-sparing procedures should not be overlooked in case of a dilated aortic root with uncalcified aortic valve. Performing valve-sparing by applying a remodeling technique operation completed with annuloplasty reduces aortic valve insufficiency, avoiding side-effects related to implanted valves.

of these excellent results is even more contrasted the 49% five year survival rate in the AAD group, not mentioned it’s 21% hospital lethality. The only conclusion might be taken out of these results: the prophylactic aortic root reconstruction is a safe and useful method, connected with excellent long term survival to prevent the occurrence of AAD.

Background We retrospectively analyzed 25 years of experiences with the button Bentall procedure in patients with aortic root pathologies. Even though this procedure has become widespread, there are only a few very long term follow-ups available in the clinical literature, especially regarding single surgeon results. Methods Between 1988 and 2013, a total of 147 patients underwent the Bentall procedure by the same surgeon. Among them there were 62 patients with Marfan syndrome. At the time of the surgery the mean age was 46.5 ± 17.6 years. The impact of surgical experience on long-term survival was evaluated using a cumulative sum analysis chart. Results The Kaplan-Meier estimated overall survival rates for the 147 patients were 91.8 ± 2.3 %, 84.3 ± 3.1 %, 76.3 ± 4.9 % and 59.5 ± 10.7 % at 1,5,10 and 20 years, respectively. Multivariate Cox regression analysis identified EuroSCORE II over 3 % (OR 4.245, 95 % CI, 1.739–10.364, p = 0.002), acute indication (OR 2.942, 95 % CI, 1.158–7.480, p = 0.023), use of deep hypothermic circulatory arrest (OR 3.267, 95 % CI, 1.283–8.323, p = 0.013), chronic kidney disease (OR 6.865, 95 % CI, 1.339–35.189, p = 0.021) and early complication (OR 3.134, 95 % CI, 1.246–7.883, p = 0.015) as significant risk factors for the late overall death. The survival rate for freedom from early complication was 94.3 ± 2.2 %, 88.0 ± 3.3 %, 82.9 ± 4.7 % and 69.2 ± 8.4 % at 1,5,10 and 20 years. The main pathological findings of the aortic wall were cystic medial degeneration in 75 %, fibrosis in 6 %, atherosclerosis in 13 % and no pathological alteration in 6 % of the samples. The overall survival rate was significantly lower in patients operated in first 15 years compared to patients operated in the last decade (log-rank p = 0.011). Conclusion According to our long-term follow-up the Bentall operation provides an appropriate functional result by resolving the lesions of the ascending aorta. Based on our results, 25–30 operations done is necessary to gain such a level of confidence and experince to aquire better results on long-term survival. In addition, we discussed that there were no co-morbidities affecting on the survival of Marfan patients and prophylactic aortic root replacement ensures a longer survival among patients with Marfan syndrome.

Background Approximately 300.000 patients might be treated in Hungary because of heart failure. Out of this 900-1000 might be affected by end stage heart failure. These patients should be potentially treated by heart transplantation (HTX). The etiologic reasons of heart failure among transplanted patient are mainly cardiomyopathic or ischemic in origin (48%-44%). The valvular or congenital origin is rear (4%-2%). The prevalence of Marfan syndrome is 1-2/ 10.000; it means that we can calculate with 1000-2000 Marfan patients (MP) in Hungary. As it is mentioned in the literature MPs are often affected by a left ventricle dysfunction due to the characteristic Marfan cardiomyopathy, which may show a rapid progression in case of concomitant longstanding aortic or/and mitral regurgitation.