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Dissecting aortic aneurysm associated with congenital bicuspid aortic valve
Insights
Congenital bicuspid aortic valve is found in 9% of fatal aortic dissecting aneurysms, significantly higher than its general population incidence. This suggests a strong link between bicuspid aortic valve and aortic dissection risk.
Area of Science:
- Cardiovascular Pathology
- Genetics and Congenital Disorders
Background:
- Aortic dissection is a life-threatening condition.
- Congenital bicuspid aortic valve is a common congenital heart defect.
Purpose of the Study:
- To investigate the prevalence of congenital bicuspid aortic valve in fatal aortic dissecting aneurysms.
- To explore the potential relationship between bicuspid aortic valve and aortic dissection.
Main Methods:
- Retrospective analysis of 119 cases of fatal aortic dissecting aneurysm.
- Exclusion of iatrogenic causes, arachnodactyly, and aortic stenosis.
- Assessment for congenital bicuspid aortic valve and other associated conditions.
Main Results:
- Congenital bicuspid aortic valve was identified in 11 cases (9%) of fatal aortic dissecting aneurysms.
- Younger patients (≤29 years) with bicuspid aortic valve often had coarctation of the aorta or Turner's syndrome.
- Cystic medial necrosis of the aorta was present in all cases; hypertension was noted in 73%.
Conclusions:
- The 9% incidence of bicuspid aortic valve in fatal aortic dissection is significantly higher than the estimated 1-2% in the general population.
- This finding strongly suggests a causative relationship between congenital bicuspid aortic valve and the development of aortic dissecting aneurysms.
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.