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The coronary arteries in Marfan's syndrome. A morphologic study
Abstract:
The coronary arteries of five hearts of patients known to have had Marfan's syndrome were studied microscopically. In four of the five cases the extramural coronary arteries showed structural changes consisting of accumulations of acid mucopolysaccharides and a concomitant disruption of the connective tissue elements. These changes closely resembled the changes that frequently occur in the aorta and that are generally described as "cystic medial necrosis". They are considered to be an important expression of wall weakening. In one case a laceration, probably traumatic, of a coronary artery led to formation of an "incomplete dissecting aneurysm." "Cystic medial necrosis" was found once in the proximal segment of the sinus nodal artery and once in the musculoelastic layer of a coronary artery that reached the atrioventricular node from the ventricular septum by perforating the anulus fibrosus. However, in contrast to findings in a previous report, the intramural coronary arteries were otherwise unaffected. The clinical significance of these data is as yet unknown particularly since these alterations are intramural and initially do not cause narrowing of the lumen. Nevertheless, the possibility of coronary arterial involvement by the metabolic disorder of Marfan's syndrome should always be considered during clinical assessment of this lesion.
Insights
Microscopic study of coronary arteries in Marfan syndrome revealed "cystic medial necrosis" in four of five patients, indicating potential wall weakening. Further research is needed to understand the clinical significance of these findings.
Area of Science:
- Cardiovascular Pathology
- Connective Tissue Disorders
Background:
- Marfan syndrome is a genetic disorder affecting connective tissue.
- Aortic abnormalities, such as cystic medial necrosis, are common in Marfan syndrome.
Purpose of the Study:
- To investigate microscopic structural changes in the coronary arteries of patients with Marfan syndrome.
- To compare coronary artery findings with known aortic pathology in Marfan syndrome.
Main Methods:
- Microscopic examination of coronary arteries from five hearts of patients with Marfan syndrome.
- Histological analysis to identify structural changes and accumulations of acid mucopolysaccharides.
Main Results:
- Four out of five cases showed structural changes in extramural coronary arteries, resembling cystic medial necrosis.
- These changes involved acid mucopolysaccharide accumulation and connective tissue disruption, indicating wall weakening.
- One case presented with a traumatic coronary artery laceration forming an incomplete dissecting aneurysm.
Conclusions:
- Coronary artery involvement, characterized by cystic medial necrosis, is present in Marfan syndrome.
- While intramural arteries were unaffected, the observed extramural changes may contribute to wall weakening.
- The clinical significance of these coronary alterations in Marfan syndrome requires further investigation.