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Marfan's syndrome. Broad spectral surgical treatment cardiovascular manifestations
Insights
Surgical intervention for Marfan syndrome cardiovascular issues shows promise. Aggressive treatment and regular follow-ups improve survival rates for patients with aortic aneurysms and valve insufficiency.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Thoracic Surgery
Background:
- Marfan syndrome frequently causes life-threatening cardiovascular complications.
- Cardiovascular issues lead to premature death in 50% of Marfan syndrome patients by age 32.
Purpose of the Study:
- To evaluate the efficacy of surgical interventions for cardiovascular manifestations in Marfan syndrome.
- To analyze outcomes and survival rates following surgical repair over a 16-year period.
Main Methods:
- Retrospective analysis of 79 operations in 41 Marfan syndrome patients.
- Surgical techniques included composite valve graft replacement for ascending aorta aneurysms and standard mitral valve replacement.
- Treatment of more distant aortic aneurysms utilized reconstruction methods similar to non-Marfan patients.
Main Results:
- Overall 15-year survival rate was 62%, with 3 early and 11 late deaths.
- Composite valve graft replacement was effective for ascending aorta aneurysms with valvular insufficiency.
- Standard mitral valve replacement safely combined with aortic graft procedures; distant aneurysms were reconstructible.
Conclusions:
- An aggressive surgical approach for cardiovascular manifestations in Marfan syndrome is recommended.
- Regular patient follow-up is crucial for early detection and management of new or existing lesions.
- A significant proportion of late deaths were due to treatable conditions, highlighting the need for proactive intervention.
Abstract:
Most patients with Marfan's syndrome have cardiovascular manifestations and complications of these abnormalities lead to death in 50% of patients by the age of 32. This report is concerned with the performance of 79 operations to control these problems in 41 patients during a 16-year period. There were 3 early deaths and 11 late deaths, with survival at 15 years in 62%. The cardiovascular manifestations assumed eight patterns of involvement, in brief, consisting of aneurysms of ascending aorta, mitral valve insufficiency, aortic dissection and dissecting aortic aneurysm, and degenerative distal fusiform aortic aneurysm. These lesions occurred in isolated form or in association with others, hence the larger number of patterns of involvement. Various methods were employed in treatment of aneurysms of the ascending aorta, which was associated with aortic valvular insufficiency in most. The most reliable method of controlling disease at this level was composite valve graft replacement of fusiform aneurysms and separate valve graft operation for dissections occurring in the previously uninvolved aorta. Isolated mitral valve insufficiency was relieved by standard mitral valve replacement and this operation could be safely combined with composite valve graft replacement of the ascending aorta. More distant aneurysms of the aorta, either degenerative or chronic dissections, were susceptible to reconstruction as employed in the nonMarfan patient. Treatment of multiple lesions was staged, treating the more symptomatic condition first. Regular follow-up examination is important in these patients to detect new lesions and to evaluate known lesions. An aggressive approach is suggested in their treatment because 63% of the 11 late deaths in this series were due to lesions that could be successfully treated by presently available methods.