A case of Marfan syndrome in an adolescent with aortic dissection: should the recommendation for surgical intervention be changed?
We present a 15-year-old male patient with Marfan syndrome who died suddenly of aortic dissection and rupture. The commonly used criteria for elective aortic root replacement are an aortic root dimension >50 mm or an aortic dimension more than twice the normal predicted value. He did not fulfill these criteria. His ascending aortic dimension 17 days prior to his death was 48 mm and the aortic dimension to the predicted value was 1.8, although he showed progressive dilatation of the aortic root. A ratio of the ascending aortic dimension of more than 1.5 times that predicted, as proposed by Pyeritz, and a considerable increase in aortic dilatation beyond previous observations may lead to timely surgical repair.
Yoshinaga
M
M
Department of Pediatrics, Faculty of Medicine, Kagoshima University, Sakuragaoka 8-35-1, Kagoshima City, Kagoshima 890-8520, Japan.
Oku
S
S
Nomura
Y
Y
Nagai
E
E
D000293:Adolescent; D001018:Aortic Diseases / Q000473:pathology* / Q000601:surgery; D001019:Aortic Rupture / Q000473:pathology* / Q000601:surgery; D003661:Decision Support Techniques; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D011237:Predictive Value of Tests; D011859:Radiography