RNF213 and chronic thromboembolic pulmonary hypertension: In conclusion, the RNF213 p.R4810K heterozygous variant was identified in 9 (6.4%) patients with CTEPH without known risk factors for venous thromboembolism, suggesting that some patients diagnosed with CTEPH have RNF213-associated vascular disease, which is associated with poor treatment response after BPA.