Current insights in diagnosis and management of the cardiovascular complications of Marfan's syndrome.
Marfan's syndrome is an inherited disorder of connective tissue, caused by mutations in the fibrillin-1 gene located on chromosome 15. Diagnosis is still based on a combination of major and minor clinical features. Prognosis is mainly determined by the cardiovascular complications. Advances in surgical and medical treatment for these complications have dramatically improved the prognosis of the syndrome.
Department of Cardiology, Academic Medical Center Amsterdam, The Netherlands.
Gijs J
GJ
Nollen
Maarten
M
Groenink
Ernst E
EE
van der Wall
Barbara J M
BJ
Mulder
D002318:Cardiovascular Diseases / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn; D008382:Marfan Syndrome / Q000150:complications*