Cardiovascular complications of Marfan syndrome in children in the Crimea

• Main Author: G. E. Sukhareva
• Format: Article
• Language: Russian
• Published: Ltd. “The National Academy of Pediatric Science and Innovation” 2019-03-01
• Series: Rossijskij Vestnik Perinatologii i Pediatrii
• Subjects: children; marfan syndrome; heart; complications; mitral valve insufficiency; expansion of the ascending aorta
• Online Access: https://www.ped-perinatology.ru/jour/article/view/828
• ISSN: 1027-4065; 2500-2228

Marfan syndrome was described more than 125 years ago, but it still remains a very complex and urgent medical problem.The objective. To evaluate the nature and severity of cardiovascular complications of Marfan syndrome. In the Republic of Crimea, there was analyzed the follow-up study of 15 patients with Marfan syndrome: 1 – 3 years old – 1 child (6.7%), 3–7 years old – 3 children (20%), 8–12 years old – 3 children (20%), 13– 17 years old – 8 children (53.3%). In 2 cases there was the familial form of the disease.The methods. The diagnosis of Marfan syndrome was based on the study of genealogical data, analysis of the morpho-phenotype and the results of the clinical examination. The severity of cardiovascular complications was assessed according to clinical and instrumental studies. The results. Heart failure was manifested by mitral valve insufficiency and expansion of the ascending aorta. The article contains own clinical observations of a teenager with acute aortic dissection, which led to the death due to the parents refusing the operation. Three other children successfully underwent surgery, in particular, surgery after Bentall and David. The remaining children with an aortic aneurysm are still followed-up and do not require surgery.The conclusion. These clinical examples prove that parents are not always aware of the severity of the child’s condition. It is important to emphasize that the cooperation of the parents, the cardiologist and the patient himself contributes to the preservation of life, improvement of its quality and duration.