Key result
Bentall De Bono procedure with mechanical valve replacement achieves full recovery in a Marfan syndrome case.
Why the study?
Patients with Marfan syndrome carry a higher risk of aortic degeneration, and endovascular repair remains controversial, necessitating advanced surgical procedures such as the Bentall technique.
Case Report (n=1)
No
The Bentall procedure remains a viable and successful surgical option for complex aortic root and valve disease in patients with Marfan syndrome, despite potential severe perioperative complications.
Supports Bentall procedure in Marfan syndrome despite complications; leaves open need for larger studies before practice change.
BACKGROUND: Patients with Marfan syndrome have a higher risk of aortic degeneration than the rest of the population, presenting with aneurysms, ruptures and dissections. Although the Bentall procedure has been considered the gold standard in the surgical treatment of combined diseases of the aortic valve and ascending aorta; traditionally, aortic manifestations in patients with Marfan syndrome are repaired with open surgical techniques, since endovascular repair in this patients is still controversial. CASE REPORT: We describe the case of a patient with a history of Marfan syndrome and a diagnosis of severe aortic stenosis and aortic aneurysm, in need of an advanced and high expertise surgical procedure for its resolution and considering the difficulty given by his comorbidity. EVOLUTION: Bentall/De Bono surgery with mechanical aortic valve replacement was successfully performed; however, there was a trans-surgical bleeding of 3 600 ml. The patient required pacemaker support, vasopressor support, and administration of blood products. During his hospitalization in the ICU, he presented with pulmonary sepsis, treated with broad-spectrum antibiotics, with complete resolution of the condition after 62 days of hospitalization. After being discharged, he remained in follow-up, with no new complications related to his underlying disease to date. CONCLUSION: Surgical repair of aortic aneurysms in patients with Marfan syndrome has evolved thanks to scientific advances, as evidenced in this case, achieving a decrease in surgical mortality rate and improving the quality and life expectancy of patients with this underlying pathology.
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Pinos et al. (2022) conducted a case report in Marfan syndrome with ascending aortic aneurysm and severe aortic stenosis (n=1). Bentall De Bono procedure with mechanical aortic valve replacement was evaluated on Patient survival and resolution of aortic aneurysm/stenosis. The Bentall De Bono procedure with mechanical aortic valve replacement was successfully performed in a 43-year-old male with Marfan syndrome and aortic aneurysm, leading to full recovery despite complications.
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