Monozygotic twins with Marfan's syndrome and ascending aortic aneurysm.

نویسندگان

  • Héctor Jorge Redruello
  • Tomas Francisco Cianciulli
  • Eduardo Fernandez Rostello
  • Barbara Recalde
  • Jorge Alberto Lax
  • Victorio Próspero Picone
  • Sandro Mario Belforte
  • Horacio Alberto Prezioso
چکیده

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.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Different clinical course of ascending aortic aneurysm in monozygotic twins.

A 45-year-old woman without medical history of cardiovascular disease came to the clinic. She was diagnosed as annuloectasia and aneurysm of ascending aorta and dissected aorta from above the aortic valve to renal artery level (type A). There are no laboratory abnormalities including CBC, biochemistry, and serology tests etc. She does not have any Marfanoid features. Surprisingly, when we revie...

متن کامل

Aneurysms of the ascending aorta due to medical degeneration associated with arachnodactyly (Marfan's disease).

Two cases are described of women showing aneurysm of the ascending aorta due to medial degeneration. In both there was evidence of Marfan's (1896) " dolichostenomelie " (arachnodactyly), a condition which in its fully developed form includes, as well as long, thin fingers and limbs, such changes as joint hyperextensibility, dolichocephaly, and dislocation of the optic lens (Rados, 1942). Severe...

متن کامل

Aortic root complications in Marfan's syndrome: identification of a lower risk group.

OBJECTIVES The purpose of this study was to examine clinical and echocardiographic predictors of outcome in a cohort of patients with Marfan's syndrome. BACKGROUND Serial echocardiographic measurements of aortic root dimensions are an important clinical method for monitoring patients with Marfan's syndrome. However, there are few data on the prognostic importance of echocardiographic variable...

متن کامل

Idiopathic cystic medionecrosis and aneurysms of the ascending aorta.

SUMMARY Two patients with ascending aortic aneurysms due to cystic medical necrosis are described. One of them was phenotypically normal while the other had features of Marfan's syndrome. Both were disabled by dyspnoea and angina which required corrective surgery.

متن کامل

Supra-aortic stenosis associated with Marfan's syndrome.

The association of arachnodactyly with other skeletal abnormalities and with ocular and cardiac malformations is now well recognized, and numbers of cases illustrating the syndrome have been reported in recent years. The main cardiac malformations have been degeneration of the aortic media with dissecting aneurysm or aneurysmal dilatation, usually of the ascending aorta, but coarctation, change...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:
  • European journal of echocardiography : the journal of the Working Group on Echocardiography of the European Society of Cardiology

دوره 8 4  شماره 

صفحات  -

تاریخ انتشار 2007