Aortic Intimal-Medial Tear Without Dissection, the Marfan Syndrome, and Its Forme Fruste Variety

Acute aortic dissection is a relatively common disease involving the aorta. All aortic dissections start with an intimal-medial tear prior to the medial dissection. Several cases of aortic intimal-medial tear without dissection have been reported previously, but only one article presented a photograph of the intimal-medial tear. Herein, we describe 16 patients whose ascending aortas were operatively excised because of what clinically was believed to be acute aortic dissection. Of the 16 patients, 14 had aortic intimal-medial tears without dissection and the other 2 had acute medial dissection of the aorta adjacent to a healed aortic intimal-medial tear without dissection. These aortic intimal-medial tears have been seen in the Marfan syndrome, but none of our 16 patients had the Marfan syndrome. At least 9 of the 16 patients, however, had had aortas similar to those seen in the Marfan syndrome (forme fruste variety). Although the 8 surgeons who operated on these 16 patients described the intimal-medial tears as “aortic dissection”, only 2 had acute dissection adjacent to a healed intimal-medial tear without dissection. In conclusion, although the aortic intimal-medial tear is the initiator of aortic dissection, some patients with intimal-medial tears have no accompanying dissection.

Introduction

Acute aortic dissection is the most common primary disease of the ascending aorta.¹ Aortic dissection begins with an intimal-medial tear and the dissection usually propagates in the outer media. On rare occasion an intimal-medial tear occurs in the ascending aorta without leading to medial dissection. Although previously seen in the ascending aorta in the Marfan syndrome,² the present study describes 16 patients seen in a 12-year period with classic intimal-medial tear in the ascending aorta unaccompanied by medial dissection. A description of these cases is the purpose of this report.