In 2020, we also clarified that the RNF213 p.Arg4810Lys variant was present in approximately 8% of patients with idiopathic PAH, and these idiopathic PAH patients with the RNF213 p.Arg4810Lys variant responded poorly to current combination therapy using PAH-specific vasodilators compared to those who carried mutations in the bone morphogenic protein receptor type 2 (BMPR2) gene, which is the most common pathogenic gene in patients with PAH [9]. This evidence concerns the gene BMPR2 and pulmonary arterial hypertension.