Aortopathy in Congenital Heart Disease in Adults: Aortic Dilatation with Decreased Aortic Elasticity that Impacts Negatively on Left Ventricular Function

Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, tetralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessitates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: "aortopathy".

Fatal traumatic rupture of ascending aortic aneurysm having idiopathic cystic medial necrosis: An autopsy case.

This report describes an autopsy case pertaining to death due to traumatic rupture of aortic aneurysm. A 21 year old deaf and dumb male was assaulted with kicks over the chest. Autopsy revealed external injuries over the body. Internally, a fusiform aneurysmal dilatation of the lumen of the aortic root and tubular segment of the ascending aorta were observed, with tear of the anterior wall of the ascending aorta and resultant haemopericardium. Histologically, the wall of the aneurysm revealed cystic medial necrosis, which appears to idiopathic in nature. A Common complication of aortic aneurysms is dissection, with subsequent spontaneous rupture. In this case, there was no evidence of dissection and the rupture was traumatic in nature. Death was certified as due to cardiac tamponade. The extent of trauma to which the victim was subjected to, appears to be such as would have been insufficient to cause death in an otherwise normal individual. An account of the findings, along with a discussion of the pathology of aneurysms and cystic medial necrosis as also of the mechanism of rupture of aneurysms is provided.

A Solitary Aneurysm of the Superficial Femoral Artery as a Rare Cause of Cystic Medial Necrosis / 日本心臓血管外科学会雑誌

A 74-year-old woman presented with a pulsatile mass and pain in the right mid-thigh. Angiography demonstrated a ruptured solitary aneurysm at the superficial femoral artery (SFA), 45mm in diameter. The operation consisted of replacement of the aneurysm with a saphenous vein bypass grafting to the SFA. The pathological examination of the resected arterial wall revealed degenerative changes due to cystic medial necrosis. In the literature, there has been no report, as far as we know, on true aneurysm of the superficial femoral artery caused by cystic medial necrosis. Therefore, it is considered that our case is a very rare presentation of aneurysm with this particular etiology which occurred in the peripheral artery of a lower extremity.