A 36-year-old man sought medical attention because of pressure in his head and distention of the veins in his neck. Physical examination disclosed a murmur of aortic insufficiency, and a chest roentgenogram showed a dilated ascending aorta. Two-dimensional echocardiography and angiography demonstrated a large intimal tear in the ascending aorta and aortic insufficiency. The patient underwent a surgical procedure, which revealed that his symptoms were caused by compression of the superior vena cava by the dilated ascending aorta. The aortic dissection was successfully repaired by replacement of the aortic root with a composite graft into which the coronary ostia were reimplanted. After further assessment of the patient, including elicitation of a similar history in some paternal relatives, Marfan's syndrome was diagnosed. Physicians should be aware of the clinical manifestations, pathogenesis, and treatment of aortic dissection. With aggressive treatment, 60 to 90% of patients survived aortic dissection in 1985, whereas only 40 years ago, this entity was rarely recognized premortem or treated successfully.
ASJC Scopus subject areas