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Impact of cardiovascular operation on survival in the Marfan patient
L G Svensson1, E S Crawford, J S Coselli
1Department of Surgery, Baylor College of Medicine, Houston, Texas.
Abstract:
This report is concerned with the surgical treatment of cardiovascular manifestations of Marfan syndrome in 151 patients by 280 operations, 70 before referral, 203 by the authors during a 16-year period, and seven by others in the follow-up interval. This therapy resulted in mitral valve replacement in 13, aortic valve replacement in 135, aortic segment replacement in 151 (including total aortic replacement in 10), nearly total aortic replacement in 26, entire thoracic aortic replacement in seven, and replacement of the entire descending and abdominal aorta in 20 patients. The early (30-day) survival was 94%, and the 5- and 10-year Kaplan-Meier survival was 75% and 56%, respectively. Fusiform aneurysms were present in the ascending aorta, aortic arch, or both in 135, the descending aorta in two, the thoracoabdominal aorta in four, and the abdominal aorta in seven. Aortic dissection occurred in 102 (67%) patients, and 34 (22%) patients with ascending aortic operations required reoperation. This study suggests that the results of current techniques warrant a more aggressive approach to these problems to permit better long-term results.
Insights
Surgical repair of cardiovascular issues in Marfan syndrome patients shows good early survival. Aggressive surgical strategies are recommended for improved long-term outcomes in aortic disease management.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Thoracic Surgery
Background:
- Marfan syndrome frequently involves cardiovascular complications, primarily aortic aneurysms and dissections.
- Surgical intervention is crucial for managing these life-threatening manifestations.
- Long-term outcomes and optimal surgical strategies require continued investigation.
Purpose of the Study:
- To evaluate the surgical treatment outcomes for cardiovascular manifestations of Marfan syndrome.
- To assess early and long-term survival rates following various aortic and valve surgeries.
- To determine the efficacy of current surgical techniques and inform future approaches.
Main Methods:
- Retrospective analysis of 280 surgical procedures in 151 Marfan syndrome patients over 16 years.
- Detailed review of surgical interventions including valve replacements and aortic segment replacements.
- Kaplan-Meier survival analysis to estimate long-term patient survival.
Main Results:
- High early survival (94%) with 5- and 10-year survival rates of 75% and 56%, respectively.
- Aortic dissection occurred in 67% of patients; 22% required reoperation after ascending aortic surgery.
- Prevalence of fusiform aneurysms in the ascending aorta/arch (135 patients) and dissection (102 patients).
Conclusions:
- Current surgical techniques offer favorable early survival for Marfan syndrome cardiovascular complications.
- A more aggressive surgical approach is suggested to improve long-term patient survival and outcomes.
- Further research into optimizing surgical strategies for complex aortic disease in Marfan syndrome is warranted.