Inherited Stanford A DeBakey I Aortic Dissection: Unveiling Genetic Predispositions and Surgical Management

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Muhammad Firdaus
Saskia Dyah Handari
Yan Efrata Sembiring

Abstract

BACKGROUND: Aortic dissection refers to the disruption of the medial layer of the aorta caused by intramural hemorrhage. This leads to the separation of the layers of the aortic wall, resulting in the establishment of a true lumen and a false lumen, which may or may not be connected. The prevalence of aortic dissection ranges from 0.2% to 0.8%. Additionally, a notable percentage, surpassing 20%, of individuals exhibit a familial inclination towards thoracic aorta dissection.


CASE PRESENTATION: A male patient in his middle age, 41 years old, with risk factors of uncontrolled hypertension, obesity, and a familial propensity to aortic dissection, presented to the emergency department exhibiting symptoms of chest pain. It characterized the sensation as having a tearing quality and indicated that it extended towards the abdomen region. The results of the test revealed several notable findings. A blood pressure reading of 235/133, a wider mediastinum and cardiomegaly on the chest X-ray, a slight increase in Hs-Troponin levels, and an electrocardiogram showed sinus rhythm with left ventricular hypertrophy. He underwent a computed tomography scan, which revealed the existence of an aortic dissection that extended from the ascending to the descending aorta without any aortic valve involvement. He was assessed with a Stanford A. DeBakey I aortic dissection, and underwent successful therapy for aortic arch replacement with the elephant trunk procedure, and plans to undertake an endovascular procedure for the descending aorta in the upcoming period.


CONCLUSION: Aortic dissection is a pathological disorder that may exhibit hereditary inheritance, and the choice of surgical technique is dependent on the specific underlying disease.

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1.
Firdaus M, Handari SD, Sembiring YE. Inherited Stanford A DeBakey I Aortic Dissection: Unveiling Genetic Predispositions and Surgical Management. SEE J Cardiol [Internet]. 2024 Jan. 20 [cited 2024 Apr. 23];5:6-10. Available from: https://seejca.eu/index.php/seejca/article/view/6066
Section
General Cardiology

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