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[Marfan syndrome and heart valve diseases]
1Deutsches Herzzentrum Berlin Klinik für Herz-, Thorax- und Gefässchirurgie Augustenburger Platz 1 13353 Berlin, Germany. pasic@dhzb.de
Insights
Surgical interventions for Marfan syndrome (MFS) cardiovascular complications, including aortic valve preservation, are detailed. Long-term outcomes of preserving the native aortic valve in MFS patients require further investigation.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Connective Tissue Diseases
Background:
- Marfan syndrome is a hereditary connective tissue disorder with high cardiovascular mortality.
- Aortic root dilatation and dissection are common, life-threatening complications.
Purpose of the Study:
- To report surgical treatment outcomes for 243 Marfan syndrome patients with cardiovascular complications.
- To evaluate classical surgical techniques (Bentall-DeBono, Cabrol) and aortic valve-sparing procedures.
Main Methods:
- Review of surgical cases involving 243 Marfan syndrome patients.
- Application of Bentall-DeBono and Cabrol procedures for annulo-aortic ectasia.
- Aortic valve preservation with concomitant mitral valve surgery.
Main Results:
- Surgical treatment was performed on 243 patients with Marfan syndrome and cardiovascular issues.
- Classical methods and aortic valve-sparing techniques were employed.
- Mitral valve surgery was performed based on established indications.
Conclusions:
- Surgical management of cardiovascular complications in Marfan syndrome is feasible.
- Aortic valve preservation and mitral valve reconstruction are possible in select Marfan syndrome patients.
- Long-term results of aortic valve-sparing procedures in Marfan syndrome remain to be determined.
Abstract:
Marfan syndrome is a hereditary disease of the connective tissue with increased mortality mostly due to changes of the cardiovascular system. We describe our experience with the surgical treatment of 243 patients with Marfan syndrome and cardiovascular complications. We report the results of treatment of annulo-aortal ectasia using the classical surgical methods of Bentall DeBono and Cabrol and the method for preservation of the native aortic valve and concomitant surgery of the mitral valve. Reconstruction or replacement of the mitral valve is mainly based on the classical indications. Preservation of the native aortic valve and reconstruction of the mitral valve in patients with Marfan syndrome is possible but the long-term results are still unknown.