A 45-year-old known patient of Marfan syndrome, presented with history of progressive shortness of breath, palpitations and easy fatigability over a period of 6 months.
Clinical Findings
- Pectus excavatum (since childhood) 6.5 cm depth.
- Early diastolic murmur over aortic area.
- Marfan syndrome features present.
Primary Investigations & Findings
- 2-D transthoracic echo: Severe aortic regurgitation and ascending aortic aneurysmal dilatation of 7 cm
- CT angiogram: Fusiform
- aneurysm of ascending aorta measuring 7 cm
- CT Thorax: Ascending aortic aneurysm and sternal compression of right ventricle. Reduction of right lung volume, mediastinal shift to the left
Diagnoses
Ascending aortic aneurysm with severe aortic regurgitation and pectus excavatum.
Management Plan
Bentall procedure was done for the ascending aortic aneurysm and severe aortic regurgitation. Additional NUSS Bar fi¬xation was done for correction of pectus excavatum.
Aspects of Challenge in the Case
1) Sternotomy posed risk of RV tear due to its compression by the pectus excavatum.
2) Risk of aneurysm rupture during sternotomy.
3) Bentall procedure and its attendant unique risks such as bleeding and neurological sequela, etc.
4) NUSS Bar procedure for pectus excavatum correction, in the back ground of major cardiac surgery, as combined procedure.
5) Modified NUSS Bar fixation with sternal osteotomies.
Follow-up
Chest wall deformity corrected. He is on regular anti-coagulation and doing well.
