Аннотация: Введение: врожденные портосистемные венозные шунты (ВПСШ) – это редкие сосудистые аномалии, которые возникают вторично по отношению к аномальному развитию или инволюции сосудистой сети плода. Они позволяют кишечной крови попадать в системный кровоток, минуя печень, что в долгосрочной перспективе приводит к различным симптомам и осложнениям. Сегодня, благодаря передовым методам визуализации, количество зарегистрированных случаев ВСПШ увеличивается, хотя по большей части это единичные клинические наблюдения или сообщения, обобщающие небольшие серии наблюдений. Общая частота ВПСШ оценивается в 1:30 000 рождений и 1:50 000 для тех, которые сохраняются после раннего детства. Материалы и методы: в статье представлены 44 зарубежных источника литературы, которые освещают вопросы патогенеза, классификации, клиники, диагностики и лечения ВПСШ. Заключение: ранняя диагностика и коррекция этой аномалии с помощью любой (эндоваскулярной или хирургической) окклюзии нивелирует симптомы и предотвращает отдаленный осложнения. В настоящее время, учитывая редкость данной патологии, нет крупного статистического анализа и не выработаны стандарты тактики ведения данной категории больных. Однако, дальнейших сбор материала, акцент на патофизиологии и анатомии этих поражений, поможет оказанию более эффективной помощи пациентам с врожденными портосистемными венозными шунтами. Список литературы 1. Kim MJ, Ko JS, Seo JK, et al. Clinical features of congenital portosystemic shunt in children. Eur J Pediatr. 2012; 171(2): 395-400. 2. Florio F, Nardella M, Balzano S, et al. Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol. 1998; 21(5): 421-424. 3. Baiges A, Turon F, Simуn-Talero M, et al. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. 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