Marfan syndrome (MFS) is a rare, autosomal‐dominant, multisystem disorder, presenting
with skeletal, ocular, skin, and cardiovascular symptoms. Significant clinical overlap with …

Marfan syndrome is a connective-tissue disease inherited in an autosomal dominant
manner and caused mainly by mutations in the gene FBN1. This gene encodes fibrillin-1, a …

Background Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan
syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We …

With advances in pediatric cardiology and cardiac surgery, the population of adults with
congenital heart disease (CHD) has increased. In the current era, there are more adults with …

Aortic aneurysms are a major health problem because they account for 1–2% of all deaths in
the Western population. Although abdominal aortic aneurysms (AAAs) are more prevalent …

Calcium channel blockers (CCBs) are prescribed to patients with Marfan syndrome for
prophylaxis against aortic aneurysm progression, despite limited evidence for their efficacy …

Монография посвящена анализу взаимосвязи т. н. малых аномалий сердца (МАС) и
диспластических фенотипов. Дан анализ современного состояния вопроса о …

The population of neonates and children with congenital heart defects presents about a 30%
prevalence of associated genetic syndrome or additional extracardiac anomalies and may …

Background Development of thoracic aortic aneurysms is the most significant clinical
phenotype in patients with Marfan syndrome. An inflammatory response has been described …

Since 2008, when angiotensin II type I receptor blockade with losartan was introduced in the
prevention of cardiovascular manifestation of Marfan syndrome (MFS), a specific treatment …