An autopsy case report of annuloaortic ectasia with cardiac tamponade ruptured from an aneurysm of the right Valsalva sinus

Annuloaortic ectasia (AAE) is a clinicopathologic condition with primary or secondary dilatation of the aortic annulus and aneurysm of the proximal thoracic aorta, leading to aortic regurgitation. We herein report an autopsy case of a Japanese 57-year-old male with AAE who died of a cardiac tamponade rupture from the sinus of the right coronary. The wall of the aortic root, particularly that of the sinus of the right coronary Valsalva, underwent extensive fibrosis with loss or fragmentation of the elastic lamina in the medial layer and perforation directly into the pericardial space. The adventitia of the proximal aorta to the aortic arch was diffusely fibrotic with both acute and chronic hemorrhage and chronic inflammatory infiltrate. However, the ascending aortic media was largely intact, except for focal laminar necrosis at the center of the medial layer; no medial cystic necrosis, laminar necrosis, or mesoaortitis/panaortitis was present in the thoracic or abdominal aorta, nor in the main aortic branches, which was suggestive of Takayasu disease and giant cell arteritis. Thus, this patient was diagnosed to have idiopathic AAE with sustained peri-aortic hemorrhage, and he finally died of a cardiac tamponade resulting from an aneurysmal rupture.