Tratamiento quirúrgico de las complicaciones de la aorta ascendente en el síndrome de Marfan. Resultados inmediatos y a largo plazo

Favaloro, Roberto; Casabé, J. Horacio; Segura, Mónica Morales; Abud, José; Casas, Jorge; López, Claudio; Dulbecco, Eduardo; Raffaelli, Héctor

doi:10.1157/13125000

Cited by 7 publications

(1 citation statement)

References 20 publications

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“…La expectativa de vida en los pacientes con síndrome de Marfan aumenta con la resolución quirúrgica, siendo la técnica Bentall de Bono la mejor. Con esta técnica se consigue una supervivencia tras la cirugía del 94%, 83% y 75% a los 2, 5 y 10 años respectivamente, con una mortalidad perioperatoria del 3.7% (n=2) [8].…”

Section: Discussionunclassified

Caso Clínico: Bentall De Bono, técnica de elección para paciente con aneurisma de aorta y síndrome de Marfan

Pinos

Rodríguez

et al. 2022

Rev Med HJCA

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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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Section: Discussionunclassified