[Marfan syndrome: therapy of cardiovascular manifestations].
With optimal clinical management of patients with Marfan syndrome, life expectancy may be improved substantially from 32 years to a nearly normal life span. Cornerstones of clinical management comprise genetic counseling, life-style management, and cardiovascular surveillance, which includes regular aortic imaging and endocarditis prophylaxis. Prophylactic use of beta-blocking agents as well as timing of elective surgery and the choice of the optimal surgical technique are difficult issues that obviously need to be individualized. Juvenile or postoperative patients as well as pregnant patients with Marfan syndrome pose particular problems. This review intends both to facilitate differential decision making based on referenced evidence and to provide guidelines for therapeutic strategies. Finally, a review of the most recent advances to somatic gene therapy is given.
von Kodolitsch
Y
Y
Universitätskrankenhaus Eppendorf, Innere Medizin II, Abteilung für Kardiologie, Hamburg.
Raghunath
M
M
Karck
M
M
Haverich
A
A
Nienaber
C A
CA
D000293:Adolescent; D000328:Adult; D002318:Cardiovascular Diseases / Q000235:genetics / Q000401:mortality / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D000071837:Fibrillins; D015316:Genetic Therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008017:Life Expectancy; D008297:Male; D008382:Marfan Syndrome / Q000235:genetics / Q000401:mortality / Q000628:therapy*; D008840:Microfilament Proteins / Q000235:genetics; D011247:Pregnancy; D011379:Prognosis
D000071837:Fibrillins; D008840:Microfilament Proteins
Das Marfan Syndrom: Therapie bei kardiovaskulären Manifestationen.