A 17 year old boy with Marfan syndrome and a pectus excavatum desires repair. He undergoes a CT of the chest for preop planning with the following findings: his calculated Haller Index is 4 and his aortic root is 5.0 cm. He is without symptoms. The next step in management should be:

 

 

 

 

[size=20px]A Immediate cardiac surgery for aortic root replacement alone

 

B Immediate cardiac surgery evaluation for combined procedure

 

C Elective Nuss bar repair

 

D Elective Ravitch Repair

 

E Observation

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A

A

B

С

Correct answer

B Immediate cardiac surgery evaluation for combined procedure

 

This patient meets criteria for aortic root replacement now given the size of the root (5 cm) and the known diagnosis of Marfan syndrome which makes him at high risk for rupture. To delay root replacement for 3 years to allow pectus repair and bar removal is not recommended. Aortic root replacement now and delayed pectus repair is a possible option, but pectus repair following sternotomy carries a higher risk of complications given adhesions in the anterior mediastinum and is generally not recommended. The approach described by Javangula in 2006 has now been validated by other authors and is likely the optimal approach to patients who are in good health and able to tolerate a longer anesthetic.

 

The approach involves resection of the involved costal cartilages while leaving the sternum and internal mammary arteries intact. Entry into the mediastinum is done through a left parasternal incision through the bed of the left cartilages with placement of a Cooley retractor, allowing for excellent visualization of the heart and aortic root while preserving the integrity of the sternum and its blood supply. Following root replacement an anterior sternal cortex osteotomy can be done and struts placed behind the sternum with gore tex, or most commonly the sternum can be stabilized with sternal plates. The chest wall closure is then completed. Although case series are small, this approach is well tolerated and carries minimal additional morbidity to aortic root repair alone.