Partial anomalous pulmonary venous return, the neglected congenital malformation: Catheterization assisted echocardiography

Author(s): Nour Rahnama, Agnes Pasquet, Karlien Carbonez, Joelle Kefer, Stephane Moniotte, Alain Poncelet, Geoffroy de Beco, Benoit Ghaye, Sophie Pierard

Background: Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart disease that can lead to major complications in adulthood, such as Pulmonary Arterial Hypertension (PAH). Methods and Results: Fifty PAPVR patients aged ≥ 18 years were included. All underwent transthoracic echocardiography and multislice imaging. Of the 26 incidentally diagnosed patients, 14(54%) did not have a cardiac follow-up. Of the patients who had an echocardiography and were identified at high risk of PH (n=16), 5(31%) did not have Right Heart Catheterization (RHC) investigation. Moreover, the correlation between RHC-derived Qp/Qs and echocardiographic Qp/Qs was weak (r²=0.29, p=0.016), especially if there is an associated atrial septal defect (r=0.009, p=0.98). Conclusion: There is a lack of follow-up for patients newly diagnosed with PAPVR and the inadequate management of these patients in current clinical practice, both in terms of screening for PAH and identifying at-risk profiles, as well as the lack of recourse to Right Heart Catheterization (RHC) when this is recommended.