Multimodality Imaging in Presurgical Evaluation of Total Anomalous Pulmonary Venous Connection: Single-Center Practice Variability and a Systematic Review of Diagnostic Error

Background: Pulmonary vein anatomy varies widely in total anomalous pulmonary venous connection (TAPVC), with diagnostic accuracy affecting surgical planning and prognosis. Echocardiography is the preferred screening modality. Cross-sectional imaging (cardiac computed tomography [CT] or magnetic resonance [MR]) may offer added value. Methods: As the first step in a quality improvement initiative, we sought to describe practice patterns in presurgical imaging in TAPVC with attention to diagnostic error. We also evaluated the available literature on diagnostic accuracy of imaging in TAPVC. Patients with TAPVC who underwent repair at our center (January 1, 2006, to September 15, 2022) were included. Results: Thirty-three of 167 patients (20%) underwent presurgical CT or MR. Initial echocardiogram incorrectly diagnosed the subtype of TAPVC in 9% (15/167). Patients with birth weight under 3 kg, born preterm, or with an initial diagnosis of mixed-type TAPVC were more likely to have misdiagnosis by initial echocardiogram. Computed tomography or MR miscategorized 3/33 patients. A systematic literature review revealed 10 studies that evaluated CT imaging diagnostic accuracy in TAPVC, one of which also included MR studies. Among 171 total patients, there were no patients with inaccurate anatomic diagnosis by CT or MR. Conclusions: Cross-sectional angiography is used increasingly in this population and with diagnostic excellence, although it is not infallible. Efforts to improve echocardiographic quality as well as multimodality imaging in high-risk patients may improve diagnostic accuracy and surgical planning.