Retroaortic Innominate Vein in Association With Pulmonary Atresia/Major Aortopulmonary Collaterals

Area of Science:

• Cardiovascular anatomy
• Congenital heart disease
• Systemic venous anomalies

Background:

• Retroaortic innominate vein (RAIV) is an extremely rare anomaly of systemic venous return, with low prevalence in the general population.
• RAIV has been associated with conotruncal abnormalities and right aortic arch.
• A high frequency of RAIV has been noted in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) at specialized centers.

Purpose of the Study:

• To evaluate the prevalence and anatomic characteristics of retroaortic innominate vein (RAIV).
• To investigate the association between RAIV and specific congenital heart diseases, particularly PA/VSD/MAPCAs.

Main Methods:

• Single-center retrospective review of medical records from 2002 to 2022.
• Identification of patients with RAIV among approximately 20,000 evaluated patients.
• Analysis of associated diagnoses and specific anatomic features in patients with RAIV.

Main Results:

• Ninety-nine patients with RAIV were identified.
• The majority of RAIV patients (65%) had PA/VSD/MAPCAs.
• Other common diagnoses included Tetralogy of Fallot (16%), and Double Outlet Right Ventricle (4%).
• In the PA/VSD/MAPCAs cohort, 69% had a right aortic arch and 30% had absent central pulmonary arteries.

Conclusions:

• Retroaortic innominate vein (RAIV) is highly prevalent in patients with conotruncal abnormalities, especially PA/VSD/MAPCAs.
• RAIV is a significant finding in this specific congenital heart disease population.
• Understanding RAIV characteristics is crucial for surgical planning in complex congenital heart disease.