CONGESTIVE CARDIAC-FAILURE AND AORTIC DISSECTION IN A YOUNG MAN WITH MARFANS-SYNDROME

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.