Marfan syndrome
A connective tissue disorder frequently associated with aortic root dilatation and risk of dissection.
Full Definition
Marfan syndrome is an inherited connective tissue disorder that significantly increases the risk of aortic complications, particularly aortic root dilatation, mitral valve prolapse, and aortic dissection. The condition affects the cardiovascular, skeletal, and ocular systems. In aortic surgery contexts, Marfan patients require lifelong surveillance and often prophylactic aortic root replacement when dimensions reach specific thresholds. The Ghent criteria are used for clinical diagnosis, incorporating family history, molecular genetic testing, and systemic features.
Usage
Usage note: Associated with specific aortic dimension thresholds for surgical intervention.
In Context
- "The patient with Marfan syndrome underwent prophylactic aortic root replacement at an aortic dimension of 4.7 cm." — Pre-operative assessment
- "Annual echocardiographic surveillance is recommended for all Marfan syndrome patients to monitor aortic dimensions." — Cardiology consultation