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[Thoracic aortic aneurysm in Marfan syndrome]
1Service de chirurgie thoracique et cardiovasculaire A, hôpital Louis-Pradel, Lyon Montchat.
Archives Des Maladies Du Coeur Et Des Vaisseaux
|May 20, 1998
Summary
Marfan syndrome commonly causes thoracic aortic aneurysms, with dissection risk increasing significantly above 50-60 mm. Surgical repair and beta-blockers improve life expectancy, but late complications may necessitate reoperation.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Medical Imaging
Context:
- Marfan syndrome is a genetic disorder primarily affecting connective tissue.
- Thoracic aortic aneurysms are a major cardiovascular complication, leading to dissection or rupture.
- Elastic tissue dystrophy causes progressive ascending aortic dilatation.
Purpose:
- To review the pathophysiology, diagnosis, and management of thoracic aortic aneurysms in Marfan syndrome.
- To highlight surgical advancements and long-term outcomes.
- To discuss the role of medical therapy and future genetic insights.
Summary:
- Thoracic aortic aneurysms in Marfan syndrome result from elastic tissue dystrophy, causing progressive dilatation and high risks of dissection/rupture, especially beyond 50-60 mm.
- Diagnosis relies on echocardiography, CT, and MRI, while beta-blockers can slow dilatation.
- Surgical treatment, including Bentall's procedure and its variants, has improved 5- and 10-year survival rates to 90% and 75-80%, respectively.
Impact:
- Improved understanding of Marfan syndrome's cardiovascular complications.
- Enhanced surgical techniques for ascending aortic aneurysms.
- Better long-term prognosis and management strategies for affected individuals.
- Potential for future genetic therapies.
- Highlights the need for lifelong monitoring and potential reoperation due to late complications.