Systemic Sirolimus Therapy Is Associated With Reduced Intervention Frequency in Pulmonary Vein Stenosis.

Background: Early clinical outcomes data for adjunctive systemic sirolimus therapy (SST) for moderate to severe pediatric pulmonary vein stenosis (PVS) are promising but limited.

Objectives: The authors aimed to characterize a cohort of patients treated with SST to determine if SST was associated with a reduction in frequency of PVS interventions.

Methods: Medical records of 45 patients with PVS treated with SST for ≥1 month from 2015 to 2022 were retrospectively reviewed.

Hybrid Interventions for Pulmonary Vein Stenosis: Leveraging Intraoperative Endovascular Adjuncts in Challenging Clinical Scenarios.

Pediatric pulmonary vein stenosis (PVS) is often progressive and treatment-refractory, requiring multiple interventions. Hybrid pulmonary vein interventions (HPVIs), involving intraoperative balloon angioplasty or stent placement, leverage surgical access and customization to optimize patency while facilitating future transcatheter procedures. We review our experience with HPVI and explore potential applications of this collaborative approach.

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy.

Background: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus.

Methods: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair.

Results: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years).