Hypoplastic left heart with intact atrial septum and levoatriocardinal vein: a challenge in identifying aortic arch...

Area of Science:

• Cardiology
• Medical Imaging
• Pediatric Cardiac Surgery

Background:

• The levoatriocardinal vein (LAV) is a rare venous anomaly that can provide an alternative route from the left atrium to the systemic circulation.
• It is occasionally associated with congenital heart defects, particularly left-sided obstructive lesions.
• Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect where the left side of the heart is underdeveloped.

Observation:

• This report details a rare case of HLHS coexisting with a levoatriocardinal vein.
• The patient also presented with an aberrant right subclavian artery, adding complexity to the anatomy.
• Diagnosis and pre-operative assessment were performed using cardiac magnetic resonance imaging and angiography (CMR/A).

Findings:

• The levoatriocardinal vein was identified as a significant finding in this HLHS case.
• Cardiac MRI/A provided detailed anatomical visualization, crucial for surgical planning.
• The combination of HLHS, LAV, and aberrant right subclavian artery represents a complex congenital cardiac anomaly.

Implications:

• Accurate pre-operative imaging is vital for managing complex congenital heart diseases like HLHS with associated anomalies.
• Cardiac MRI/A is a powerful tool for delineating intricate vascular anatomy in pediatric patients.
• Understanding rare venous anomalies like LAV is important for surgical and interventional strategies in congenital heart disease.