Синдром Марфана: діагностика та лікування серцево-судинних уражень

Abstract

Purpose – to analise of results, long-term experience of surgical treatment of cardiovascular diseases in patients with Marfan syndrome to improve quality and prolong life. Materials and methods. A conducted prospective analysis of consecutive patients (292) with Marfan syndrome treated at the institute during 1980–2018. The age of patients was 7–57 years, mean 31.6±9.4 years. Among them, men – 220 (75.3%), women – 72 (24.7%). Marfan syndrome was diagnosed according to the criteria of Gent Nosology (2010). 24 (8.2%) patients were not operated on for various reasons. The remaining 268 (91.8%) were managed surgicaly: 257 – ascending aortic aneurysm; 4 – abdominal; 7 patients – only mitral valve insufficiency. Results. Hospital mortality was 9.3% (25 patients). Long-term results were studied in 224 (92.2%) patients from all who had been discharged from the clinic within 6 months – 20 years on average 63 months. Good long-term results were admitted in 145 (64.7%), satisfactory in 41 (18.3%), unsatisfactory in 14 (6.3%) patients. 24 (9.9%) patients died in the long term observation. Conclusions. Aortic aneurysms in patients with Marfan syndrome are formed at a young age. The most common cause sof death in the unoperated cases are rupture of aneurism or heart failure. The «gold-standarg» remains the Bentall De Bono operation. The patients who underwent surgical treatment requires observation during all-life period. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. The authors declare no conflicts of interests. Key words: Marfan syndrome, aortic aneurysm, aortic dissection.