Interrupted aortic arch and aortic atresia with circle of Willis-dependent coronary perfusion

Area of Science:

• Cardiology
• Congenital Heart Disease
• Pediatric Cardiac Surgery

Background:

• Interrupted aortic arch (IAA) is a severe congenital heart defect.
• Aortic atresia, the complete absence of the aortic lumen, is typically incompatible with life.
• Type B IAA involves a discontinuity between the ascending and descending aorta.

Observation:

• A neonate presented with a rare combination of type B interrupted aortic arch and aortic atresia.
• An aberrant right subclavian artery was noted.
• Sufficient retrograde blood flow through the circle of Willis was present.

Findings:

• The aberrant right subclavian artery and circle of Willis facilitated retrograde perfusion of coronary arteries.
• This unique circulatory pathway allowed for survival despite the critical aortic anomalies.
• A successful two-ventricular repair was performed, correcting the defect.

Implications:

• This case highlights the potential for survival in seemingly unsurvivable congenital heart defects.
• Understanding aberrant vascular anatomy is crucial for surgical planning in complex cardiac cases.
• Successful surgical intervention offers hope for patients with complex interrupted aortic arch and aortic atresia.