Intravenous digital subtraction aortography in the preoperative and postoperative evaluation of Marfan's aortic disease.

Seventeen patients with the skeletal stigmata of Marfan's syndrome or the physical and radiographic findings of Marfan's aortic disease (or both) were studied using intravenous digital subtraction aortography. Digital subtraction aortography revealed a dilated aortic root in 16 of the 17 patients. One of the 17 had mildly dilated aortic sinuses with a normal ascending aorta. All of the four children (less than or equal to 16 years of age) had aortic diameters which were greater than the 95th percentile for their body surface area. The average aortic root diameter in the adult patients was 6.8 cm (range, 4-11 cm). Three of the 17 had aortic dissection, two of which were detected by digital subtraction aortography and two by aortic root injection. Four of 13 adults and three children had pulmonary arterial diameters which exceeded 4 cm. Six patients underwent ascending aorta and valvular replacement and were studied with digital subtraction aortography after surgery. All preoperative and postoperative studies were of adequate diagnostic quality. Aortic root diameters calculated from intravenous digital subtraction angiograms correlated well with those derived from echocardiography (r2 = 0.92). We conclude that intravenous digital subtraction aortography is effective in diagnosing and following Marfan's aortic disease both before and after surgery.