A Case of Bicuspid Aorta with Stanford Type a Aortic Dissection

Abstract

Bicuspid aortic valve disease (BAV) is a congenital disease that frequently produces complications during adulthood, and is considered not only a problem of valvulogenesis, but also a more complex genetic alteration that involves the development of the heart and the aorta, which entails a high morbidity and mortality in these patients. In 50% of adults with this pathology, non-valvular anomalies are observed, with dilation of the ascending aorta being the most common. Genes such as NOTCH1, UFDL1, ACTA2, eNOS, among others, have been identified as responsible for the appearance of aneurysms, which increases the risk in these patients of complications such as aortic dissection and its subsequent rupture. Painless aortic dissection may be seen in less than 5% of patients and may have an atypical clinical presentation. A case and review of the literature is presented in which we can observe the great complexity of this pathology, in the case of a patient with BAV, annulo-ectatic dilation of the aortic root and ascending aorta who developed heart failure, syncope and presented as a complication Stanford type A aortic dissection, requiring highly specialized surgical treatment.