Right Pulmonary Artery Size Is A Predictor Of Survival In Patients With Unrepaired Scimitar Vein
Carlos Bonilla-Ramirez, Iki Adachi, Michiaki Imamura, Jeffrey S. Heinle, Christopher Caldarone, Ziyad M. Binsalamah.
Baylor College of Medicine, Houston, TX, USA.
Scimitar vein (SV) repair at younger age has unfavorable outcomes. We identified factors associated with survival time in patients with unrepaired SV
Retrospective, single-center analysis of 56 patients diagnosed with scimitar syndrome between 1995 and 2019. Cox proportional hazard regression evaluated variables associated with survival time without SV repair.
Median follow-up was 6.2 years. Seven patients required SV surgery and aortopulmonary collaterals (APC) occlusion, 16 required SV surgery alone, 17 required APC occlusion alone, and 16 had no interventions. Median age at APC occlusion was 0.48 years (IQR 75 days-3 years). APC occlusion decreased pulmonary-to-systemic blood flow (Qp:Qs) (median 1.6 IQR 1.47-2.22 to median 1.45 IQR 1.22-1.5, p=0.002) and systolic pulmonary artery pressure (median 34 mmHg IQR 28.2-63 to median 28.5 mmHg IQR 25.2-32.7, p=0.004). Thirty-eight (67.85%) had additional sources of left-to-right shunting. 7/38 shunts were repaired without needing SV surgery. Median age at SV surgery was 7 years (IQR 2-19), with 79% five-year reintervention-free survival. Five-year survival for the entire cohort was 96%. There was no difference in 5-year survival between treatment groups. On multivariate analysis, a lower right pulmonary artery (RPA) to total pulmonary artery (PA) cross-sectional area (RPA:PA CSA) was associated with longer survival time without SV repair (HR 1.08, 95% CI 1.01-1.16, p=0.01).
APC occlusion can improve pulmonary hypertension and overcirculation in neonates and infants. Lower RPA:PA CSA is associated with longer survival time in patients with unrepaired scimitar syndrome.