This was similar to a previous study, in which a radiological suspicion of PVOD/PCH was presented in 71% of those with PVOD/PCH, 57% of patients with a clinical diagnosis of PAH and biallelic EIF2AK4 variants, 14% of patients with PAH with no variant, and 5% of those with BMPR2 variants [11]. Here, EIF2AK4 is linked to pulmonary arterial hypertension.