Endovascular Treatment for Peripheral Pulmonary Arterial Stenosis in RNF213-Related Vasculopathy.

This brief report reviews the clinical, procedural, and imaging data of seven patients with p.Arg4810Lys variant of the ring finger protein 213 gene (RNF213)-related peripheral pulmonary arterial stenosis (PPAS) who underwent percutaneous transluminal pulmonary angioplasties (PTPAs) for demographics, clinical presentation, indications for BPA, and procedural and clinical outcomes. During median follow-up of 64.4 months since the first confirmed diagnosis, PTPA was performed for 62 segmental pulmonary arteries with 38 sessions of the procedure in seven patients. Vascular stent placement due to resistance to balloon dilatation and immediate elastic recoil was performed in 48/62 procedures (77%). Except for one death, six patients showed an improvement of dyspnea, five patients a decrease of mean pulmonary arterial pressure (mean 55.5 to 42.7mmHg) and increase of 6-minute walk distance (mean 415.5 to 484.3m). Reperfusion edema occurred in 4 out of 7 patients (57%), which is 6 out of 38 sessions (16%).