Summary
Congenital sinus Valsalva aneurysm (SVA) is a rare anomaly and the least common of all aortic aneurysms. Secondary causes are atherosclerosis, syphilis, cystic medial necrosis or infective endocarditis. Sixty-six per cent of these aneurysms arise from the right sinus, 25% from the non coronary sinuses and the remaining 9% from the left coronary sinus. SVA results from a localized dystrophy of connective tissue in the sinuses which leads to discontinuity between aortic media and the fibrous annulus of the aortic valve. Patients with non ruptured SVA are usually asymptomatic and SVA is often detected by routine echocardiography. Diagnosis can be confirmed by echocardiography, magnetic resonance imaging, computed tomography, aorto-/arteriography or cardiac catheterization. Complications depend on the size of aneurysm, which is growing slowly and therefore rarely manifests in childhood. Non ruptured aneurysm can obstruct the right ventricular outflow tract, cause aortic regurgitation due to distortion of the aortic valve, compress the left coronary artery or the conduction system. Aneurysm can gradually enlarge leading to rupture into the cardiac chambers that surround the aortic root. Rupture of SVA may occur spontaneously, but rarely before the age of 20 years. Rupture of right sinus aneurysms occurs into the right atrium or right ventricle, that of the non coronary sinus into the right atrium, both ruptures producing a left-to-right-shunt. Ruptured aneurysms from the left sinus are rarely observed; however, when it happens, then rupture occurs into the left atrium or left ventricle or into the pericardial space. Therefore, they do not produce a left-to-right-shunt. Non ruptured aneurysm should be repaired electively. Hospital mortality is below 5% and the late results are excellent in absence of aortic valve damage. In patients with ruptured SVA, urgent surgical repair is recommended. *Author: Prof. H. Kaemmerer (December 2003)*.
