Coarctation of aorta

Coarctation of the aorta (CoA) is a congenital heart defect characterized by a narrowing (stenosis) of the aorta, the large blood vessel that carries oxygen-rich blood from the heart to the body. The narrowing most commonly occurs in the area where the ductus arteriosus inserts, near the left subclavian artery, in a region known as the aortic isthmus. This condition is also sometimes referred to as aortic coarctation or aortic narrowing. CoA accounts for approximately 5–8% of all congenital heart defects and is more common in males than females. It is frequently associated with other cardiac anomalies, including bicuspid aortic valve (present in up to 85% of cases), ventricular septal defect, patent ductus arteriosus, and mitral valve abnormalities. CoA is also a recognized feature of Turner syndrome (45,X). The narrowing of the aorta creates an obstruction to blood flow, leading to high blood pressure (hypertension) in the upper body and reduced blood pressure and perfusion in the lower body. In neonates with severe coarctation, closure of the ductus arteriosus can precipitate acute heart failure, shock, and end-organ damage, representing a life-threatening emergency. Older children and adults with less severe coarctation may present with upper extremity hypertension, diminished or absent femoral pulses, a blood pressure gradient between the arms and legs, headaches, leg claudication, and epistaxis. Left ventricular hypertrophy may develop over time due to chronic pressure overload. Long-term complications include aortic aneurysm formation, stroke, coronary artery disease, and recurrent coarctation. Treatment depends on the severity and age at presentation. In critically ill neonates, prostaglandin E1 infusion is used to maintain ductal patency while preparing for intervention. Definitive treatment includes surgical repair (resection with end-to-end anastomosis, subclavian flap aortoplasty, or patch aortoplasty) or catheter-based balloon angioplasty with or without stent placement, particularly in older children and adults or for recurrent coarctation. Even after successful repair, lifelong follow-up is essential, as patients remain at risk for re-coarctation, persistent hypertension, aortic aneurysm, and associated cardiac lesions. With appropriate treatment and monitoring, most patients have a good long-term prognosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Coarctation of aorta