Moreover, medications and interventions for aortopathy in complex CHD are described. In this review, we will discuss the aortic complications of several complex CHDs, with emphasis on their pathophysiology, clinical manifestations and outcomes. This aortic dilatation in CHD may either be intrinsic or a secondary result from increased volume overload of the aortic root (right to left shunting), or a combination of both. There are reported variables that alter the structure of ascending aortic media in humans such as aging, hypertension, pregnancy (via increased hemodynamics in the setting of genetic abnormality), chromosomal abnormality and genetics ( 2) ( Table 1). These observations led us to hypothesize that many CHD cause aortic medial degeneration that reflects a common developmental fault, which weakens the aortic wall.
CHD, such as single ventricle with pulmonary stenosis/atresia, truncus arteriosus communis (TAC), complete transposition of the great arteries (TGA), hypoplastic left heart syndrome (HLHS), tetralogy of Fallot (TOF) and the Fontan procedure, are also associated with aortic medial degeneration and aortic dilatation ( 1). Aortic dilation in Marfan syndrome, bicuspid aortic valve and coarctation of the aorta (COA) is well documented, and these disorders are associated with ascending aortic and/or para-coarctation medial degeneration ( 3, 4). Accepted for publication Dec 17, 2018.ĭilation of the ascending aorta in congenital heart diseases (CHD) may exceed hemodynamic or morphogenetic expectations, or the aorta may become aneurysmal and rupture ( 1, 2). Keywords: Aortopathy aortic medial degeneration aortic dilatation aortic aneurysm aortic dissection On the other hand, valve-sparing operations have been developed in order to preserve the aortic valve. The Bentall operation comprises root replacement with an aortic tube graft and mechanical valve, and has been widely applied. In aortopathy, concomitant aortic valve and aortic root repair/replacement is required. However, the results, except for Marfan syndrome, are limited.
Several medications have been studied, including β-blockers, angiotensin II type I (AT1) receptor blockers (ARBs) and Ca-antagonists. The major purpose of medical treatment for aortopathy is to reduce the structural changes within the aortic wall and slow down the progression of aortic dilatation to reduce the risk of cardiovascular events. The association of aortic pathophysiological abnormalities, aortic dilatation and aorto-ventricular interaction is clinically referred to as “aortopathy”. This aortic medial degeneration in CHD may either be intrinsic or secondary to the increased volume overload of the aortic root due to right-to-left shunting, or a combination of both. Accordingly, the incidence of ascending aortic dilatation, dissection and rupture is higher in the former. In Marfan syndrome, bicuspid aortic valve and COA, medial degeneration is more extensive and severe than in another complex CHD. Medial degeneration in the ascending aorta is prevalent among the above CHD. A significant subset of adults with complex CHD exhibit progressive dilatation of the aortic root even after repair due to aortic medial degeneration. Congenital heart diseases (CHD), such as truncus arteriosus, transposition of the great arteries (TGA), tetralogy of Fallot (TOF), hypoplastic left heart syndrome (HLHS), single ventricle with pulmonary stenosis/atresia and the Fontan procedure, are also associated with aortic root dilatation, aneurysm and rarely, dissection, which can be fatal and require aortic valve and root surgery. Abstract: A bicuspid aortic valve and/or coarctation of the aorta (COA) are often associated with dilatation of the ascending aorta and para-coarctation.
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