8th March 2024

Introduction

Coarctation of the aorta (CoA) is a congenital stenosis which forms in the region of the ligamentum arteriosum at the descending aorta. It accounts for 4-6% of all congenital cardiovascular defects, with a prevalence of approximately 4 per 10,000 live births. Most cases are sporadic, with a male predominance. The severity of CoA depends on the degree of coarctation and narrowing. As a response to increased left ventricular afterload from left ventricular outflow tract obstruction, remodelling in the form of left ventricular hypertrophy occurs. There is also development of collateral blood supply from the mammary, intercostal and scapular vessels to bypass the stenosis. Amongst patients with CoA, up to 85% of patients have a bicuspid aortic valve (mainly fusion of the left and right coronary cusps). 

Up to 35% of patients with Turner’s syndrome have CoA. Other associated abnormalities include: 

• Ventricular septal defect
• Patent ductus arteriosus 
• Valvular and subvalvular aortic stenosis 
• Congenital mitral valve abnormalities 
• Aneurysm of the Circle of Willis 
• Aortopathies (aortic aneurysm and dissection) 
• Collateral circulation within the spinal canal causing spinal subarachnoid haemorrhage