S. Agrawal et al., CONGESTIVE CARDIAC-FAILURE AND AORTIC DISSECTION IN A YOUNG MAN WITH MARFANS-SYNDROME, Journal of accident & emergency medicine, 11(4), 1994, pp. 259-260
Citations number
NO
Categorie Soggetti
Emergency Medicine & Critical Care","Medicine, General & Internal
Marfan's syndrome is inherited as an autosomal dominant trait with var
iable penetrance. It affects the skeleton, eyes and cardiovascular sys
tem. There is disproportionate longitudinal bone growth resulting in l
ong limbs, arachnodactyly, dolichocephaly, high-arched palate and a lo
ng, narrow face. Recurrent joint dislocations and kyphoscoliosis are s
een. In the eye, lens dislocation is the most common abnormality but m
yopia and retinal detachment are also described. The cardiovascular ab
normalities deserve special attention because they pose the most serio
us threat to life. Aneurysmal dilation of the aorta is well-recognized
, most commonly occurring at the root of the aorta with natural progre
ssion to dissection and rupture. Cardiac failure may be precipitated b
y aortic or mitral valve prolapse, and rarely by myocardial infarction
.(1) Annual measurement of aortic diameter has been recommended in you
ng people despite a low prevalence of serious cardiovascular complicat
ions.(2) Other cardiovascular complications have been described for ex
ample, atrial fibrillation and interatrial septal aneurysm,(3) and dis
section of the internal carotid artery.(4) It appears that any major a
rtery can become ectatic.