The Utility of CT Angiography in Neonates with Pulmonary Atresia with Intact Ventricular Septum and Concern for Right Ventricular Dependent Coronary Circulation: Case Series

Up to one third of patients with pulmonary atresia with intact ventricular septum (PA-IVS) will have inadequate anterograde coronary blood flow and rely on fistulous connections from the right ventricle (RV) for myocardial perfusion, known as RV-dependent coronary circulation (RVDCC). Historically, identification of the extent of ventriculocoronary connections and coronary stenosis has required invasive imaging with cardiac catheterization and angiography. Cardiac computed tomography (CCT) potentially provides a less invasive imaging option for therapeutic planning in this group of patients. We describe six neonates with PA-IVS who underwent both CCT and cardiac catheterization at our institution prior to any surgical or transcatheter intervention between 2009 and 2019. Imaging was concerning for RVDCC in all six patients. The average tricuspid Z-score was − 4.19 (2.1 to − 5.34). Two patients underwent cardiac transplantation and two patients underwent ductal stenting. The overall mortality rate was 50%. CCT findings closely mirrored the findings of invasive cardiac catheterization and identified important morphological variations. The average radiation exposure (DLP) per CCT was (10.5 mGy cm, range 6–20). Technological improvements in CCT have enabled adequate visualization of coronary anomalies in children with comparable accuracy to cardiac catheterization, but considerably less radiation exposure. However, diagnosis of RVDCC requires direct right ventricular angiography. Therefore, the potential benefit of obtaining a CCT prior to catheterization for infants with PA-IVS is the ability to risk stratify, assist with procedural planning, and improve family counseling.