The coronary arteries in Marfan's syndrome. A morphologic study

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.