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

This brief report reviews the clinical, procedural, and imaging data of 7 patients with p.Arg4810Lys variant of the ring finger protein 213 (RNF213) gene-related peripheral pulmonary arterial stenosis who underwent percutaneous transluminal pulmonary angioplasties (PTPAs) for demographics, clinical presentation, indications for angioplasty, 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 7 patients. Vascular stent placement because of resistance to balloon dilation and immediate elastic recoil was performed in 48 of 62 procedures (77%). Except for 1 death, 6 patients showed an improvement in dyspnea and 5 patients showed a decrease in mean pulmonary arterial pressure (mean, 55.5-42.7 mm Hg) and increase in 6-minute walk distance (mean, 415.5-484.3 m). Reperfusion edema occurred in 4 of 7 patients (57%), which was 6 of 38 sessions (16%).