Aortopathy associated with congenital heart disease: A current literature review

Abstract

In patients born with congenital heart disease, dilatation of the aorta is a frequent feature at presentation and during follow-up after surgical intervention. This review provides an overview of the pathologies associated with aortopathy, and discusses the current knowledge on pathophysiology, evolution, and treatment guidelines of the aortic disease associated with congenital heart defects.

Factors associated with aortic root dilatation after surgically repaired ventricular septal defect

“…A longer follow‐up period was also significantly correlated with the presence of ARD in both ARD definitions. The prognosis after surgical repair of uncomplicated VSD is excellent, with a reported 25‐year survival rate after surgical VSD repair of 89% . This suggests that long follow‐up periods are not rare in patients with surgically repaired VSD; hence, physicians should perform cardiovascular imaging to detect potential aortopathies, such as ARD .…”

“…The prognosis after surgical repair of uncomplicated VSD is excellent, with a reported 25‐year survival rate after surgical VSD repair of 89% . This suggests that long follow‐up periods are not rare in patients with surgically repaired VSD; hence, physicians should perform cardiovascular imaging to detect potential aortopathies, such as ARD . Additionally, atherosclerotic risk factors (eg, obesity and hypertension) are frequent in adults with congenital heart disease as well as the general population .…”

“…The most common finding in patients with CHD is ventricular septal defect (VSD), and patients with VSD generally have a long survival period after surgical repair . Previous studies have focused on aortic dilation in Marfan syndrome, Turner syndrome, bicuspid aortic valve, coarctation of the aorta, and single ventricular and conotruncal abnormalities, such as tetralogy of Fallot or truncus arteriosus . Case reports of aortic dilatation in patients with unrepaired VSD have been published; a recent European study reported that 15% of patients with surgically repaired VSD had aortic dilatation; however, this previous study did not evaluate factors associated with ARD.…”

Aortopathy in an Adult With Tricuspid Atresia and Left Ventricular Non-Compaction After Fontan Procedure

“…3 A few cases of dissection of the aorta or progressive aortic dilation with valvular regurgitation have been published. 4,5 When volume loading and hypoxia persist for a considerable period, and the time since Fontan procedure is long, or when the aortic valve is bicuspid, the aortic root may become dilated and result in aneurysmal changes.…”

“…regurgitation (AR) occur, resulting in a negative impact on systemic ventricular function, has been recognized in some CHD including coarctation of the aorta, bicuspid aortic valve, and conotruncal abnormalities such as tetralogy of Fallot. 3 Increasing evidence has suggested that functional single ventricle after Fontan procedure should be categorized as an aortopathy. 3-5 Herein we report the case of an adult after Fontan procedure for TA and LVNC in whom surgical intervention was necessary due to a progressively dilated aorta with AR.…”