What is coronary artery atresia?

Coronary Artery Atresia: Definition, Diagnosis, and Management

Coronary artery atresia is a rare congenital cardiovascular malformation characterized by the absence of a coronary artery ostium or an imperforate coronary artery, resulting in potential myocardial ischemia and sudden cardiac death risk. 1

Definition and Pathophysiology

Coronary artery atresia is characterized by:

• Complete absence of a coronary ostium or an imperforate coronary artery 1
• Most commonly affects the left main coronary artery (LMCA) 2, 3
• May present as a blind-ending left main trunk without connection to the aorta 4
• Can occur in isolation or in association with other congenital heart defects 5, 6

Clinical Presentation

Patients with coronary artery atresia may present with:

• Chest pain and dyspnea 5
• Heart failure symptoms 2
• Myocardial ischemia 1
• Sudden cardiac death, particularly during exertion 1
• May remain asymptomatic until adulthood if adequate collateral circulation develops 3

Diagnostic Evaluation

Imaging Modalities

1. Coronary CT Angiography (CTA):

   • Currently the most useful imaging modality for evaluating coronary anatomy 1
   • Provides accurate depiction of the entire anomalous vessel including distal segments
   • Can identify high-risk anatomic features such as slit-like ostium morphology and proximal narrowing 1

2. Echocardiography:

   • Initial screening tool, especially in children
   • May have limited sensitivity in adults 1
   • Transesophageal echocardiography may provide better visualization when transthoracic windows are inadequate 1

3. Invasive Coronary Angiography:

   • Traditionally reserved for interventional procedures or when noninvasive imaging is inconclusive 1
   • Can identify anomalous coronary arteries but lacks optimal 3D information 1
   • Essential to clarify the extent of vulnerable territory of the anomalous vessel 1

4. Intravascular Ultrasound (IVUS):

   • Provides precise cross-sectional imaging of coronary lumen and wall thickness 1
   • Particularly useful in evaluating stenosis of anomalous vessels 1

Functional Assessment

• Stress testing to evaluate for inducible myocardial ischemia 1
• Assessment of ventricular function to detect consequences of coronary insufficiency 1

Management

Surgical Intervention

• Surgical revascularization is indicated for symptomatic patients or those with evidence of myocardial ischemia 1
• Coronary artery bypass grafting (CABG) is the most common surgical approach 5, 4
• Surgery should be performed by surgeons with specific training and expertise in congenital heart disease 1
• After surgical repair, resumption of physical activity can be considered after:
  • Complete sternal healing
  • No evidence of myocardial ischemia on testing
  • No complex ventricular arrhythmias 1

Medical Management

• May be considered in select cases without evidence of significant ischemia 1
• Heart failure medications may be needed depending on ventricular function 1
• Regular follow-up with stress testing to monitor for development of ischemia 1

Special Considerations

Sports Participation

• Athletes with coronary artery atresia should be assessed for symptoms and inducible myocardial ischemia 1
• Competitive sports participation should be restricted until surgical revascularization if evidence of ischemia is present 1
• After successful surgical repair and normal stress testing, sports participation can be reconsidered 1

Long-term Follow-up

• Any patient with coronary artery manipulation should be evaluated for coronary artery patency, function, and anatomic integrity at least once in adulthood 1
• Regular screening for myocardial ischemia is recommended 1

Associated Conditions

Coronary artery atresia may be associated with:

• Supravalvar aortic stenosis 5
• Coronary-pulmonary fistula 5
• Other congenital heart defects 6
• Mitral valve regurgitation due to papillary muscle ischemia 2

Prognosis

Prognosis depends on:

• Extent of myocardial territory affected
• Development of collateral circulation
• Presence of associated cardiac defects
• Timing of diagnosis and intervention

Without intervention, patients are at risk for myocardial infarction, heart failure, and sudden cardiac death 2, 3.