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Aortic Intimal-Medial Tear Without Dissection, the Marfan Syndrome, and Its Forme Fruste Variety
William C Roberts1, Minseob Jeong2, Charles S Roberts3
1Baylor Scott & White Heart and Vascular Institute; the Departments of Internal Medicine; Pathology.
Abstract:
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.
Insights
Acute aortic dissection often begins with an intimal-medial tear. However, this study found that 14 of 16 patients believed to have dissection actually had only intimal-medial tears without dissection.
Area of Science:
- Cardiovascular Medicine
- Thoracic Surgery
Background:
- Acute aortic dissection is a critical cardiovascular emergency.
- It is understood to originate from an intimal-medial tear in the aorta.
Observation:
- This study reviewed 16 patients surgically treated for suspected acute aortic dissection.
- 14 patients presented with intimal-medial tears without medial dissection.
- 2 patients had acute dissection adjacent to a healed intimal-medial tear.
Findings:
- The majority of patients (14/16) exhibited aortic intimal-medial tears without concurrent dissection.
- None of the patients had Marfan syndrome, but 9 showed aortic features resembling a forme fruste.
- Surgical descriptions of "aortic dissection" were often inaccurate, with only 2 cases showing actual dissection.
Implications:
- Aortic intimal-medial tears are the initiating event for aortic dissection.
- Clinical suspicion of aortic dissection may not always correlate with histological findings.
- Distinguishing intimal-medial tears from true dissection is crucial for accurate diagnosis and treatment.
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