Pulmonary arterial angioplasty in peripheral pulmonary arterial stenosis in RNF213 vasculopathy: effective but high-risk treatment with reperfusion injury

Abstract Background p.Arg4810Lys variant of the ring finger protein 213 (RNF213) vasculopathy is a nonsyndromatic involving multisystemic organs including pulmonary arteries. Peripheral arterial stenosis (PPAS) with diffuse and beaded appearance unique feature this disease no treatment option has been tried before. Methods We performed BPA for PPAS in RNF213 patients (n=7). Severity hypertension was assessed by echocardiography right heart catheterization at baseline. Several clinical hemodynamic parameters were followed up after each sessions. Results All had dyspnea on exertion. Right ventricular dysfunction observed six NT-proBNP elevated (949.2±1148.1 pg/mL). Baseline showed severe (70.1±19.2 mmHg). Pulmonary ballooning to all patients, but stenting (n=6) cutting balloon (n=1) necessary due elastic recoil. Improvement achieved 5.3 sessions average. Reperfusion edema seen four eventually leading death one patient. Conclusions angioplasty, when coupled stenting, an effective vasculopathy. Although reperfusion injury common fatal few cases, it only modality improve patient's symptom hemodynamics till now. Careful selection target population should be preceded perform intervention. Funding Acknowledgement Type funding sources: None.