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Abstract

ABSTRACT
Marfan syndrome is an autosomal dominant genetic disease characterized by mutations in the fibrillin-1 gene that lead to a connective tissue disorder affecting mainly the skeletal, ocular, and cardiovascular systems. Its reported prevalence in the general population is around 1/3,000–1/5,000, with no described racial predilection. Cardiovascular manifestations cause over 90% of the deaths in these patients, mainly due to aortic aneurysms and related complications. Medical therapy includes the administration of beta blockers and angiotensin II receptor inhibitors to slow the aortic growth rate, and prophylactic root surgery with either a composite root replacement (Bentall procedure) or valve-sparing procedures (reimplantation or remodeling) is the leading choice in patients with a diagnosed aneurysm. We report the case of a 26-year-old Black female with suspected Marfan syndrome and a family history of aneurysm and sudden death in first-degree relatives who underwent urgent repair of a 49.5 mm aortic root aneurysm with a composite root replacement procedure.
RÉSUMÉ
Le syndrome de Marfan est une maladie génétique autosomique dominante. Les manifestations cardiovasculaires de cette maladie causent plus de 90% des décès chez ces patients, principalement dus aux anévrismes aortiques et aux complications associées. La prise en charge comporte un traitement médical et une chirurgie prophylactique de la racine aortique (procédure de Bentall ou procédure d’épargne valvulaire). Nous rapportons le cas d’une femme noire de 26 ans avec un syndrome de Marfan suspecté et des antécédents familiaux d’anévrisme et de mort subite, qui a subi une réparation urgente d’un anévrisme de la racine aortique de 49,5 mm par une procédure de Bentall.

Keywords

Bentall operation Marfan Syndrome Aortic dissection Sub-Saharan Africa Chirurgie de Bentall Syndrome de Marfan Dissection aortique Afrique subsaharienne

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Mve Mvondo, C., Kobe, Z. F., Ngo Yon, L., Tsague Kengni, H. N., Dakleu, M., Ndobo Koe, V., Ela Bella, A., Haoua, T., Ngatchou, W., & Ngowe Ngowe, M. (2023). Successful Bentall Operation in a Black African Woman with Marfan Syndrome and Type A Aortic Dissection: A Case Report. HEALTH SCIENCES AND DISEASE, 24(9). https://doi.org/10.5281/hsd.v24i9.4625
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