Желудочковая экстрасистолия у детей

The article presents updated data on the problem of premature ventricular contractions in children based on the clinical guidelines of the Russian Society of Cardiology and the Union of Pediatricians of Russia for the diagnosis, treatment and management of pediatric patients with premature ventricular contraction. The issues of diagnosis and treatment based on the principles of evidence-based medicine as well as important aspects of prevention of exacerbations and follow-up have been clarified in detail. The criteria for assessing the quality of care for patients with premature ventricular contractions have been presented.CONFLICT OF INTEREST. Not declared.В статье изложены обновленные данные о проблеме желудочковых экстрасистолий у детей, основанные на клинических рекомендациях Ассоциации детских кардиологов России и Союза педиатров России по диагностике, лечению и ведению пациентов детского возраста с желудочковой экстрасистолией. Подробно освещены вопросы диагностики, лечения, основанные на принципах доказательной медицины, а также важные аспекты профилактики обострений болезни и диспансерного наблюдения. Отображены критерии оценки качества медицинской помощи пациентам с желудочковыми экстрасистолиями.Публикация является обновленным вариантом клинических рекомендаций Ассоциации детских кардиологов России и Союза педиатров России по диагностике, лечению и ведению пациентов детского возраста с желудочковой экстрасистолией, опубликованных на сайте https://medi.ru/klinicheskie-rekomendatsii/zheludochkovaya-ekstrasistoliya-u-detej_14366/Авторы подтверждают отсутствие конфликта интересов, который необходимо обнародовать

Damages of Cardiovascular System at Mucopolysaccharidosis Type I: Clinical Cases

Background. Cardiac involvement in patients with mucopolysaccharidosis (MPS) type I, or Hurler syndrome, has various phenotypes.Clinical Case Description. In the first case symptoms were indicative of acute severe heart failure which was confirmed by laboratory and instrumental diagnostic methods. Left heart chambers dilatation and left ventricular hypocontractility were revealed on echocardiography. Atypical disease course with no improvement on multicomponent therapy of heart failure let us to think about metabolic disease, so we confirmed it with tandem mass spectrometry and molecular genetic testing. Therefore this led to timely enzyme replacement therapy onset and allogeneic bone marrow transplantation that positively affect the disease outcome. The second case showed classic course of MPS I. Its clinical signs such as musculoskeletal and ENT-organ manifestations allowed us to diagnosis and later confirm it by tandem mass spectrometry and molecular genetic testing at the age of 3. The cardiac pathology presented by mitral valve leaflets thickening and 2nd degree regurgitation has been diagnosed later. In our view, early treatment onset should slow down the progression of heart damage.Conclusion. Several clinical variants of cardiac pathology at MPS I are presented. The need of constant cardiovascular system monitoring in children with MPS I is shown. It is also mentioned that cardiac pathology can be the first manifestation of the disease

Premature Ventricular Contraction in Children

The article presents updated data on the problem of premature ventricular contractions in children based on the clinical guidelines of the Russian Society of Cardiology and the Union of Pediatricians of Russia for the diagnosis, treatment and management of pediatric patients with premature ventricular contraction. The issues of diagnosis and treatment based on the principles of evidence-based medicine as well as important aspects of prevention of exacerbations and follow-up have been clarified in detail. The criteria for assessing the quality of care for patients with premature ventricular contractions have been presented.CONFLICT OF INTEREST. Not declared