Review Article
Aortic Disease in the Young: Genetic Aneurysm Syndromes, Connective Tissue Disorders, and Familial Aortic Aneurysms and Dissections
Table 2
Major Ghent criteria used to diagnose Marfan syndrome.
| System | Major criteria |
|
Skeletal system | Pectus carinatum | Pectus excavatum requiring surgery | Upper to lower segment ratio <0.86 or span to height ratio >1.05 | Arachnodactyly: wrist and thumb signs Pes planus Protrusio acetabuli | Scoliosis > 20 d or spondylolisthesis | Reduced extensions at the elbows (<170 d) |
| Ocular system | Ectopia lentis (dislocated lens) |
| Cardiovascular system | Dilatation of the ascending aorta | Aortic root dilatation |
| Dura | Lumbosacral dural ectasia |
| Family/genetic history | Family history | Genetic mutations known to cause Marfan syndrome Inheritance of DNA maker haplotype linked to MFS in the family |
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