Quadricuspid aortic valve with ascending aortic aneurysm: report of a case and discussion of embryological mechanisms

Quadricuspid Aortic Valve

“…19,[24][25][26] However, in our study cohort, echocardiography showed that 29% of patients had dilatation of the aortic root, tubular ascending aorta, or both at the time of the index diagnosis of QAV. In addition, this aortic dilatation was associated with poorer survival free of aortic valve surgery or death, although the enlargement was only mild in 79% of these patients.…”

Death From Ascending Aortic Aneurysm Secondary to Quadricuspid Aortic Valve

“…12 In our case, we cannot exclude a concausal role of the QAV. In fact, despite the originality of our case (the QAV is usually an isolated cardiac artifact, and only 18% of the cases are associated with other conditions, such as AAA), 13 valve morphology has an effect on blood flow in the ascending aorta contributing to the development of aortic disease, [14][15][16][17][18] and the relationship between the morphology of the valve and aortopathy was also demonstrated in studies with computed tomography, angiography, and magnetic resonance. 16,19,20 Statistically, the function is normal only in 16% of cases, and it also histologically exhibits fibrotic thickening because of an uneven distribution of mechanical stress on the cusps 3 resulting in AI (50%-75% cases).…”

“…21 Therefore, AAA is the consequence of a chronic regurgitation over time by AI, 22 and in literature, type B is proposed as the most likely to develop AI because of the shear stress asymmetric cusp, although cases of type A have also been recorded. 13,[22][23][24] Furthermore, a common etiopathogenic hypothesis combines aortic and valvular pathology, recognizing the prevailing role the apoptosis of vascular smooth muscle cells plays in the degradation of the aortic wall. 25 This is particularly the case with genetically induced early apoptosis of the neural crest cells, which are those at the origin of both the aortic valve cusps and the arterial media.…”

Ascending aortic aneurysm associated with a quadricuspid aortic valve

“…In such cases, cystic medial necrosis might result in dilatation of the ascending aorta. 4,6 The pathological fi ndings in our patient showed grade II cystic medial necrosis of the ascending aortic wall.…”

“…To our knowledge, there are only two reports of QAV cases associated with an ascending aortic aneurysm. 4,5 We often encounter dilatation of the ascending aorta in patients with a bicuspid aortic valve. In such cases, cystic medial necrosis might result in dilatation of the ascending aorta.…”