Aortic dilatation in complex congenital heart disease

Area of Science:

• Cardiovascular Medicine
• Genetics
• Congenital Heart Disease

Background:

• Bicuspid aortic valve and coarctation of the aorta (COA) are linked to ascending aorta dilatation.
• Complex congenital heart diseases (CHD) like TGA, TOF, HLHS, and Fontan procedures are associated with aortic root issues, including fatal dissections.
• Aortic medial degeneration is prevalent in these conditions, leading to progressive aortic root dilatation even after repair.

Purpose of the Study:

• To review the pathophysiology and management of aortopathy in patients with congenital heart disease and bicuspid aortic valves.
• To highlight the increased risk of aortic dilatation, aneurysm, and dissection in these patient populations.
• To discuss current medical and surgical treatment strategies for aortopathy.

Main Methods:

• Literature review of studies on aortopathy in congenital heart disease, bicuspid aortic valve, and Marfan syndrome.
• Analysis of the prevalence and progression of aortic medial degeneration.
• Evaluation of medical therapies (beta-blockers, ARBs, Ca-antagonists) and surgical interventions (Bentall operation, valve-sparing procedures).

Main Results:

• Aortic medial degeneration is a key factor in ascending aorta dilatation and dissection in CHD and bicuspid aortic valve.
• Medial degeneration is more severe in Marfan syndrome, bicuspid aortic valve, and COA compared to other CHDs.
• Medical treatments have limited efficacy in slowing aortic dilatation, except possibly in Marfan syndrome.

Conclusions:

• Aortopathy, characterized by aortic abnormalities, is a significant concern in CHD and bicuspid aortic valve patients.
• Surgical repair, including valve-sparing options, is often necessary to manage aortic root dilatation and prevent cardiovascular events.
• Further research is needed to identify effective medical therapies to slow the progression of aortopathy.