Scimitar Syndrome Outcomes in Patients with Pulmonary Hypertension: A Single Center Experience

Abstract

<h3>Purpose</h3> Scimitar syndrome (SS) is a rare congenital heart disease characterized by anomalous pulmonary venous return, with associated right lung hypoplasia. This study aimed to describe the differences in characteristics and outcomes of SS patients with and without pulmonary hypertension (PH). <h3>Methods</h3> This was a retrospective cohort study that included all SS patients followed at a large pediatric institution from October 1989 through August 2021. <h3>Results</h3> A total of 84 patients diagnosed with SS were included (27% with PH). When comparing no-PH with PH patients, PH patients were diagnosed with SS at a younger age (PH: 11 days vs. no-PH: 449 days, p:0.034), frequently symptomatic by the time of diagnosis (PH: 87% vs. no-PH:79%, p:0.538), had often congenital heart disease (PH:87% vs. no-PH: 46%, p: 0.001), history of prematurity (PH: 18% vs. no-PH:11%, p: 0.716), and genetic syndromes (PH:9% vs. no-PH: 3%, p:0.301). Fifty-two percent of the SS PH patients were initiated on PH target therapy (58% phosphodiesterase inhibitors, 8% endothelin receptor antagonists and 33% dual PH target therapy). From the procedural perspective, PH patients underwent more APC occlusion procedures (PH:61% vs. no-PH:41%, p:0.218), had multiple interventional cardiology procedures (PH:83% vs. no-PH:38%, p<0.001) were younger when they underwent their first surgical scimitar vein repair (median age PH:26 months vs. no-PH:84 months, p:0.222), and had a higher incidence of restenosis after interventional cardiac procedures (PH:26% vs. no-PH:8%, 0.040). Only PH patients underwent extracorporeal mechanical circulatory support (9%), and overall had a higher rate of mortality (PH:22% vs. No-PH:5%, p:0.032). <h3>Conclusion</h3> Managing SS patients with PH is an understudied area that requires further data. This study demonstrated that such patients more frequently have a history of congenital heart disease, higher incidence of mortality, and have a greater risk of scimitar vein restenosis compared to non-PH SS patients.