Monozygotic twins with Marfan's syndrome and ascending aortic aneurysm.
Marfan's syndrome is a hereditary connective tissue disease, in which cardiovascular abnormalities (especially aortic root dilatation) are the most important cause of morbidity and mortality. In this report, we describe two 24-year-old twins, with a history of surgery for lens subluxation and severe cardiovascular manifestations secondary to Marfan's syndrome. One of the twins suffered a type A aortic dissection, which required replacement of the ascending aorta, and the other twin had an aneurysmal dilatation of the ascending aorta (46mm) and was prescribed medical treatment with atenolol and periodic controls to detect the presence of a critical diameter (50mm) that would indicate the need for prophylactic surgery.
Redruello
Héctor Jorge
HJ
Division of Cardiology and Cardiovascular Surgery, Hospitales del Gobierno de la Ciudad de Buenos Aires Bernardino Rivadavia and Cosme Argerich, Buenos Aires, Argentina. jredruello@hotmail.com.ar
Cianciulli
Tomas Francisco
TF
Rostello
Eduardo Fernandez
EF
Recalde
Barbara
B
Lax
Jorge Alberto
JA
Picone
Victorio Próspero
VP
Belforte
Sandro Mario
SM
Prezioso
Horacio Alberto
HA
D000328:Adult; D000784:Aortic Dissection / Q000000981:diagnostic imaging* / Q000209:etiology; D001011:Aorta / Q000000981:diagnostic imaging* / Q000473:pathology; D001014:Aortic Aneurysm / Q000000981:diagnostic imaging* / Q000209:etiology; D001262:Atenolol / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000000981:diagnostic imaging*; D014430:Twins, Monozygotic; D014463:Ultrasonography
D001262:Atenolol