ACUTE AND CHRONIC AORTIC DISEASE WITH MARFANS-SYNDROME AND ARTERIAL-HYPERTENSION - A COMPARISON OF ANATOMY, CLINICAL FINDINGS AND PROGNOSIS

The purpose of this study was to compare thoracic aortic pathologies o f 30 patients with Marfan's syndrome and a group of 78 patients with a rterial hypertension. With a mean age of 35 +/- 12 years, patients wit h Marfan's syndrome were younger than hypertensives (59 +/- 11) (p < 0 .01) and Marfan's syndrome, women (52%) were more frequently affected than in hypertensive patients (21%; p < 0.05). While aortic dissection and intramural hemorrhage in patients with Marfan's syndrome were usu ally confined to the ascending thoracic aorta (62%), in the hypertensi ve patients aortic disease frequently extended to distal segments of t he thoracic or abdominal aorta (p < 0.05). Aortic pathology was compli cated by aortic regurgitation in 95% of the patients with Marfan's syn drome and in 56% of the hypertensive patients (p < 0.01). Signs of ren al, mesenteric or coronary malperfusion, cardiac effusion and severe h ypotension (p < 0.05) were more frequently seen in the hypertensive gr oup. While there was no early death in the Marfan group, only 67% of t he patients having aortic dissection or intramural hematoma survived t he first 30 days in the hypertensive group (p < 0.01). Aortic dissecti on, intramural hematoma and aortic aneurysms were as often found in th e Marfan's syndrome patients with 60%, 10%, and 30%, as in patients wi th arterial hypertension with 55%, 6%, and 38% (n.s.). One-year surviv al rates were high in patients with Marfan's syndrome (93%) as well as in patients with arterial hypertension (73%) (n.s.). The diagnosis of aortic dissection, intramural hematoma, and aneurysms could in all ea ses be established by the use of noninvasive imaging techniques, such as TEE, XCT, and MRI, Moreover, noninvasive imaging modalities were bo th highly sensitive and specific for the diagnosis of aortic regurgita tion, pericardial effusion, or mediastinal hematoma complicating acute aortic disease.