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Descending aortic replacement after Nuss for pectus excavatum in a Marfan patient-Case report
Dawn Jaroszewski1, MennatAllah Ewais1, Patrick DeValeria1
1Division of Cardiothoracic Surgery, Mayo Clinic Arizona, United States.
Introduction:
The Nuss procedure for pectus excavatum (PE) repair has been successfully performed in Marfan syndrome (MFS) patients however there is concern for future risk of aortic dilation/rupture and need for emergent access with support bars in place.
Case Presentation:
We present a 45 year-old male with MFS that required descending aortic replacement shortly after modified Nuss repair.
Discussion:
The majority of MFS patients have severe PE and repair with the Nuss procedure is not uncommon. The risk for life threatening aortic dilation, dissection, or rupture in such patients is a concern when utilizing this technique. Our work has been reported in line with the CARE criteria.
Conclusion:
Nuss repair should be considered in MFS patients with technique modifications and careful consideration of future risk of aortic dilation and rupture.
Insights
Marfan syndrome (MFS) patients with severe pectus excavatum (PE) undergoing Nuss repair face risks of aortic complications. Modifications and careful monitoring are crucial for managing these risks post-surgery.
Area of Science:
- Cardiothoracic Surgery
- Genetics
- Medical Engineering
Background:
- Marfan syndrome (MFS) patients often present with severe pectus excavatum (PE).
- The Nuss procedure is a common surgical approach for PE repair in MFS patients.
- Concerns exist regarding the risk of aortic dilation or rupture following Nuss repair in MFS.
Purpose of the Study:
- To highlight the potential risks associated with the Nuss procedure in Marfan syndrome patients.
- To present a case illustrating the need for vigilance regarding aortic complications after PE repair in MFS.
Main Methods:
- Case presentation of a 45-year-old male with MFS undergoing modified Nuss repair.
- Review of literature regarding Nuss procedure in MFS and associated aortic risks.
Main Results:
- The patient required descending aortic replacement shortly after modified Nuss repair.
- The Nuss procedure in MFS patients carries a risk of life-threatening aortic dilation, dissection, or rupture.
Conclusions:
- Nuss repair can be considered for MFS patients with severe PE.
- Technique modifications and careful risk assessment for aortic dilation are essential.
- Post-operative monitoring for aortic complications is critical in MFS patients after Nuss repair.
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