From the Guidelines
The patient is at greatest risk for developing right heart failure if left untreated. This patient's presentation is consistent with coarctation of the aorta, a congenital narrowing of the aorta typically occurring distal to the left subclavian artery, as suggested by the systolic ejection murmur that radiates to the paravertebral region and rib notching (erosions of posterior ribs 6-8) seen on chest x-ray 1. The boy's exercise intolerance and leg pain during activity result from decreased blood flow to the lower extremities. If left untreated, coarctation leads to chronic pressure overload on the left ventricle, causing left ventricular hypertrophy and eventually left heart failure. This increased afterload is transmitted backward through the pulmonary circulation, ultimately resulting in right heart failure, as defined by the 2018 AHA/ACC guideline for the management of adults with congenital heart disease 1. While coarctation can also lead to complications like hypertension, stroke, and aortic dissection, right heart failure represents the most likely long-term complication from the options provided. Key points to consider in this diagnosis include:
- The presence of a systolic ejection murmur and rib notching on chest x-ray, which are classic findings for coarctation of the aorta
- The patient's symptoms of exercise intolerance and leg pain, which are consistent with decreased blood flow to the lower extremities
- The potential for long-term complications, including right heart failure, if left untreated Treatment typically involves surgical correction or balloon angioplasty with stenting to relieve the obstruction and prevent these complications, with the goal of improving exercise capacity and reducing the risk of long-term complications, as outlined in the guideline 1.
From the Research
Potential Complications of Untreated Coarctation of the Aorta
The patient's symptoms, such as tiredness, pain in the lower legs, and the presence of a systolic ejection murmur, suggest a possible diagnosis of coarctation of the aorta. If left untreated, this condition can lead to several complications, including:
- Hypertension
- Aortic aneurysm, dissection, and rupture
- Infective endocarditis
- Heart failure
- Premature coronary artery disease
- Stroke
Risk of Specific Complications
According to the studies, the patient is at greatest risk for developing:
- Heart failure, as it is one of the most common causes of death in untreated coarctation of the aorta patients 2
- Aortic dissection or rupture, which can occur due to the increased pressure on the aorta 3, 2
- Cerebral hemorrhage, which is a possible complication of untreated coarctation of the aorta 2
Long-Term Complications
Even if the coarctation is repaired, patients are still at risk for long-term complications, including: