Aortic dilatation in complex congenital heart disease

Koichiro Niwa


A bicuspid aortic valve and/or coarctation of the aorta (COA) are often associated with dilatation of the ascending aorta and para-coarctation. 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. A significant subset of adults with complex CHD exhibit progressive dilatation of the aortic root even after repair due to aortic medial degeneration. Medial degeneration in the ascending aorta is prevalent among the above CHD. In Marfan syndrome, bicuspid aortic valve and COA, medial degeneration is more extensive and severe than in another complex CHD. Accordingly, the incidence of ascending aortic dilatation, dissection and rupture is higher in the former. 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. The association of aortic pathophysiological abnormalities, aortic dilatation and aorto-ventricular interaction is clinically referred to as “aortopathy”. 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. Several medications have been studied, including β-blockers, angiotensin II type I (AT1) receptor blockers (ARBs) and Ca-antagonists. However, the results, except for Marfan syndrome, are limited. In aortopathy, concomitant aortic valve and aortic root repair/replacement is required. The Bentall operation comprises root replacement with an aortic tube graft and mechanical valve, and has been widely applied. On the other hand, valve-sparing operations have been developed in order to preserve the aortic valve.