Dissecting aortic aneurysm associated with congenital bicuspid aortic valve.

Abstract

Among 119 cases of fatal dissecting aneurysm of the aorta, exclusive of those iatrogenically caused or associated with arachnodactyly or aortic stenosis, there were observed 11 cases of congenital bicuspid aortic valve (9%). The ages ranged from 17 to 69 years, five of the patients being 29 years old or younger. Among the latter, three had coarctation of the aorta and one had Turner's syndrome without coarctation. In one of the older patients, aortic insufficiency was present. Hypertension was either established or inferred from cardiac weight in 73% of the cases. In each case, cystic medial necrosis of the aorta was present. Prolapse of valves other than the aortic was observed in 45% of the cases with bicuspid aortic valve. Compared to an estimated incidence of bicuspid aortic valve of about 1 to 2% in the population, the high incidence among subjects with dissecting aneurysm suggests a causative relationship between bicuspid aortic valve and aortic dissecting aneurysm.

Ascending Aortic Dilatation Associated With Bicuspid Aortic Valve

“…89 From a US cardiovascular registry of dissecting aortic aneurysms, 11 (9%) of the 119 specimens had a BAV, and 5 of those patients were aged Ͻ30 years. 88 BAV disease carries a 6.14% lifetime risk of aortic dissection, 9-fold higher than the risk in the general population. 88 Patients with Marfan syndrome have a much higher lifetime likelihood of aortic dissection (40%) than patients with BAV.…”

“…87 The increased risk of dissection and rupture associated with BAV is due to the higher prevalence and rate of aortic dilatation, which occurs at a significantly younger age relative to idiopathic ascending aortic aneurysms. 76,78,88,89 In a study of dissecting aortic aneurysms in patients aged Ͻ40 years old, 9 (24%) of the 38 cases were associated with BAV. 89 From a US cardiovascular registry of dissecting aortic aneurysms, 11 (9%) of the 119 specimens had a BAV, and 5 of those patients were aged Ͻ30 years.…”

Bicuspid aortic valve and coarctation: two villains part of a diffuse problem

“…Histologic examination of the ascending aorta frequently shows loss of smooth muscle cells and severe degeneration of the medial elastic fibres-so called "cystic medial necrosis". The presence of a bicuspid valve increases the risk of dissection at least ninefold, 5 and dissection tends to occur in younger patients than in those with tricuspid aortic valves. 6 While the jet above a stenotic aortic valve may cause haemodynamic perturbation and aortic dilatation, a functionally normal aortic valve is often associated with a dilated aorta.…”

Long-term outcomes after surgical repair of the coarctation of aorta beyond infancy period

“…In addition, patients with bicuspid aortic valves have a 9-18-fold higher incidence of developing an aortic aneurysm due to accelerated degeneration of the aortic media. [21,22] This indicates that a bicuspid aortic valve is a continuous pathological process. [23] Dilatation, aneurysm, and dissection may develop in patients due to loss of the structural support of the aortic wall.…”