Partial anomalous pulmonary venous return in adults. Insight into pulmonary hypertension

• Published in: Archives of Cardiovascular Diseases Supplements Vol. 15; no. 1; pp. 136 - 137
• Main Authors: Rahnama, N., Ghaye, B., Kubangumusu, L., Pasquet, A., Poncelet, A., Kefer, J., Moniotte, S., De Beco, G., Pierard, S.
• Format: Journal Article
• Language: English
• Published: Elsevier Masson SAS 01.01.2023
• ISSN: 1878-6480
• DOI: 10.1016/j.acvdsp.2022.10.263

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital heart disease (CHD) where pulmonary veins drain into pulmonary circulation leading to a left-to-right shunt. PAPVR can lead to serious consequences, i.e. pulmonary hypertension (PH). To date, predictors of PH are not well established. The current surgical Qp/Qs cut-off (> 1.5) in guidelines for PAPVR surgery is based on a consensus and has not been studied in the literature. The aims of this study were: (1) to determine PH prevalence in PAPVR population; (2) to identify PH predictive factors; (3) to assess the clinical relevance of the current surgical cut-off for the Qp/Qs ratio. Patients aged ≥ 18 years were enrolled. Patients with other potential causes of PH were excluded. PH was defined based on right heart catheterization (RHC). For patients without RHC, PH was considered when echocardiographic systolic transtricuspid peak velocity was ≥ 3.4m/s. Fifty patients were included. Mean patient age was 50±18 years with a 60% female predominance. PAPVR was mostly arising from the right lung (76% of cases) and was associated with CHD in 18 patients (36%). Most of them (83%) were sinus venosus atrial septal defect (ASD). PH was identified in 7 patients of the cohort (14%) and 2 of them had Eisenmenger syndrome. A Qp/Qs ratio>1.5 was significantly predictive of PH (OR=17, P=0.017), while association with ASD, the presence of more than one abnormally implanted vein and age were not predictive. The ROC curve performed indicates an excellent predictive value of Qp/Qs and identifies the threshold of 1.95 as the optimal cut-off point for PH prediction (Figs. 1 and 2). Prevalence of PH in PAPVR population is 14%. Among them, almost 30% had an Einsenmenger syndrome. A Qp/Qs ratio>1.5 was signicantly predictive of PH. The optimal Qp/Qs cut-off of 1.95 for PH prediction is in accordance with current guidelines for PAPVR surgery.