Combined severe pectus excavatum correction and aortic root replacement in Marfan's syndrome.

Area of Science:

• Cardiovascular Surgery
• Thoracic Surgery
• Medical Genetics

Background:

• Marfan syndrome is a genetic disorder affecting connective tissue, often leading to cardiovascular complications like annulo-aortic ectasia.
• Severe pectus excavatum can coexist with Marfan syndrome, posing complex surgical challenges.
• Surgical intervention is necessary for annulo-aortic ectasia to prevent aortic dissection or rupture.

Observation:

• A 23-year-old male patient with Marfan syndrome presented with annulo-aortic ectasia and severe pectus excavatum.
• The surgical approach involved a submammary skin incision, bilateral subperichondrial resection of abnormal costal cartilages, and parasternal division of intercostal muscles.
• A 23-mm St. Jude's composite graft was used for aortic root replacement.

Findings:

• Successful aortic root replacement using a composite graft.
• Chest wall reconstruction was achieved with a high sternal osteotomy and Gore-Tex strips for sternal support.
• The patient experienced an uneventful postoperative recovery, indicating the efficacy of the combined surgical technique.

Implications:

• This case demonstrates a viable surgical strategy for managing complex cardiovascular and thoracic deformities in patients with Marfan syndrome.
• The combined approach may offer a solution for patients requiring simultaneous aortic repair and pectus excavatum correction.
• Further research could explore long-term outcomes and refine surgical techniques for similar complex cases.