Key result
Sequential hybrid repair of the aorta and bilateral iliac arteries was successfully performed in a patient with Marfan syndrome, with no new dissection or aneurysmal degeneration at 5-year follow-up.
Case Report (n=1)
No
Sequential hybrid repair using multi-branched and bifurcated grafts is a feasible and durable option for managing complex, extensive aortic aneurysmal degeneration in patients with Marfan syndrome.
Hybrid repair described in complex Marfan dissection; leaves open durability, safety, and generalizability questions.
Marfan syndrome is a connective tissue disorder associated with aortic dissection, aneurysmal degeneration and rupture. These cardiovascular complications represent the main cause of mortality, therefore repair is indicated. We present a 35-year-old woman who experienced acute onset of chest pain. Her imaging revealed a chronic DeBakey type I dissection with aortic root dilation and descending thoracic aneurysmal degeneration. She underwent a Bentall procedure and endovascular exclusion of the descending thoracic aortic aneurysm. She was closely followed and 2 years later a computed tomography angiography (CTA) revealed the aneurysmal degeneration of the thoracoabominal aorta and bilateral iliac arteries. The patient underwent a composite reconstruction using multi-visceral branched and bifurcated Dacron grafts. At 5 years from her last surgery, a CTA revealed no new dissection or further aneurysmal degenerations. Aortic disease in Marfan patients is a complex clinical problem that may lead to secondary or tertiary aortic reconstructions; close follow-up is mandatory.
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Hinojosa et al. (2017) conducted a case report in Marfan syndrome with chronic aortic dissection and aneurysmal degeneration (n=1). Sequential hybrid repair (Bentall procedure, TEVAR, and open thoracoabdominal/iliac reconstruction) was evaluated on Freedom from new dissection or aneurysmal degeneration. Sequential hybrid repair of the aorta and bilateral iliac arteries was successfully performed in a patient with Marfan syndrome, with no new dissection or aneurysmal degeneration at 5-year follow-up.
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