However, our study conferred different perspectives, the mean time from targeted therapy to last follow up was 39.3 months, one patient (Patient 5) was previously misdiagnosed with IPAH and received sequential combination therapies, biallelic EIF2AK4 mutations were detected in June 2017, and PVOD was finally considered when combined with his clinical characteristics. The gene discussed is EIF2AK4; the disease is idiopathic pulmonary arterial hypertension.