Extensive aneurysms of sinuses of Valsalva precluding valve sparing aortic root reimplantation (David procedure)

A 55-year-old female noticed worsening exertional dyspnoea for two years. She was born with cleft palate and profound deafness. Significant physical findings included dysmorphism with micrognathia and acrocephaly and congenital deafness. Transthoracic echocardiogram revealed aneurysms involving the right and the non-coronary sinuses of Valsalva. Despite that, the native aortic valve retained preserved geometry. Computed tomography (CT)-scan demonstrated multiple aneurysms arising from all three sinuses of Valsalva. This displaced the right ventricle (RV) caudally and indented the RV outflow tract. A valve-sparing root reimplantation was planned. However, intraoperatively the root aneurysms were found to be very extensive such that no healthy tissue remained along the insertion lines of the aortic valve leaflets. The aortic annulus was not dilated (2 cm) and the left ventricular outlow tract was not involved in the disease process. Consequently, despite the presence of macroscopically normal leaflets and relatively undisturbed annular geometry, we were unable to reimplant the native aortic valve and proceeded to a modified Bentall procedure. Histologically, significant medial degeneration with loss of elastin and muscle was identified in the aortic sinus wall. Similar changes were also found affecting the native leaflets coupled with increased fibrous thickening.