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- Title
BENTALL PROCEDURE IN FAMILIAL AGGREGATION: BICUSPID AORTIC VALVE- RELATED AORTOPATHY. CASE REPORT.
- Authors
Ibănescu, Raluca; Ibănescu, Corneliu; Munteanu, Andreea Catalina; Harpa, Marius
- Abstract
Background: Bicuspid aortic valve is the most common congenital heart defect with an estimated prevalence of 0.5-2% in the general population. It is often associated with progressive aortic valve disease (stenosis or regurgitation) and accute thoracic aortic aneurysm and dissection. A suggestive genetic role for TBX20 and NOTCH1 genes in bicuspid aortic valve and thoracic aortic aneurysms etiology underlines the importance of this transcription factors in cardiovascular disease and also indicate a strong genetic component to the development of this malformation. Objectives: Our objective is to present a case of bicuspid aortic valve- related aortopathy in two brothers treated with Bentall procedure. Material and methods: We present the casses of two 44 and 49 years old male brothers who are diagnosed with bicuspid aortic valve and ascending aortic aneurysm, presenting at the objective exam: chest pain, cough, DISPNHEA, asthenia, fatigue, sweating. Presurgical echocardiographic examination revealed an ascending aortic aneurysm and significant aortic valve regurgitation with a normal global contractility. without any other comorbidities. Angiocardiographic examination discovered ascending aortic aneurysm (of 52 mm, respectively 53 mm) and also dilated aortic annulus (of 24 mm respectively 25mm) requiring surgical replacement of the aortic root for both of them. These modifications led to surgical replacement of aortic valve and ascending aorta with mechanical valvular conduit and coronary arteries reimplantation, surgically known as Bentall procedure. Results: Postsurgical echocardiographic examination confirms a good postoperative status of the patients: normalfunctional valvular prothesiss with conserved global contractility which demonstrates adequate coronary perfusion. Conclusion: This case of the two brothers is a very rare example of familial aggregation by bicuspid aortic valve related aortophaty. Bentall procedure is the gold standard surgery in treatment of aortic root pathology which prevents severe complications related to this pathology: dissection and rupture. This procedure improves the quality of life by blurring the symptoms. The bicuspid aortic valve, known as a defect with high heritability, should be strongly investigated for screening to all the patiens and families with this malformation being associated with serious long-term health risks.
- Subjects
AORTIC valve diseases; BICUSPIDS; MITRAL valve; AORTIC valve transplantation; AORTIC valve; THORACIC aneurysms; AORTIC rupture; PERCUTANEOUS balloon valvuloplasty
- Publication
Acta Medica Marisiensis, 2019, Vol 65, p46
- ISSN
2068-3324
- Publication type
Article