Pulmonary Atresia with Intact Ventricular Septum: Correlation of Preoperative Computed Tomography-Derived Parameters with Echocardiographic Tricuspid Valve Z-Score and Surgical Outcomes

The role of preoperative cardiac computed tomography (CT) in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) remains unclear. This study was aimed to elaborate the role of preoperative CT-derived anatomical and functional findings in planning treatment strategies in neonates with PA-IVS. The presence of ventriculocoronary arterial connections was evaluated by CT. CT-derived ventricular volumetric parameters were compared and correlated with echocardiographic tricuspid valve (TV) z-score in 12 neonates with PA-IVS. Cardiac CT and echocardiographic findings were compared between definite surgical types (median follow-up, 4 years). Ventriculocoronary arterial connections were identified with CT in 58.3% of cases (7/12) and associated with higher incidence of Fontan procedure (42.9%, 3/7) and high mortality (28.6%, 2/7). The CT-derived and echocardiographic TV z-scores exhibited a high correlation (R = 0.924, p