Management of aortic disease in children with FBN1-related Marfan syndrome.
Eur Heart J
; 45(39): 4156-4169, 2024 Oct 14.
Article
en En
| MEDLINE
| ID: mdl-39250726
ABSTRACT
Marfan syndrome (MFS) is a hereditary connective tissue disorder with an estimated prevalence of 15000-110 000 individuals. It is a pleiotropic disease characterized by specific ocular, cardiovascular, and skeletal features. The most common cardiovascular complication is aortic root dilatation which untreated can lead to life-threatening aortic root dissection, mainly occurring in adult patients. Prompt diagnosis, appropriate follow-up, and timely treatment can prevent aortic events. Currently there are no specific recommendations for treatment of children with MFS, and management is greatly based on adult guidelines. Furthermore, due to the scarcity of studies including children, there is a lack of uniform treatment across different centres. This consensus document aims at bridging these gaps of knowledge. This work is a joint collaboration between the paediatric subgroup of the European Network of Vascular Diseases (VASCERN, Heritable Thoracic Aortic Disease Working Group) and the Association for European Paediatric and Congenital Cardiology (AEPC). A group of experts from 12 different centres and 8 different countries participated in this effort. This document reviews four main subjects, namely, (i) imaging of the aorta at diagnosis and follow-up, (ii) recommendations on medical treatment, (iii) recommendations on surgical treatment, and (iv) recommendations on sport participation.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Síndrome de Marfan
Límite:
Child
/
Humans
Idioma:
En
Revista:
Eur Heart J
Año:
2024
Tipo del documento:
Article
País de afiliación:
Bélgica
Pais de publicación:
Reino Unido