Luo et al also reported that none of the patients with clinically diagnosed PAH and biallelic EIF2AK4 mutations developed pulmonary edema in response to pulmonary artery vasodilator therapies.[25] In the typical PVOD patients, the incidence of pulmonary edema with intravenous prostanoids has been reported as high as 44% after a median treatment duration of just 9 days. Here, EIF2AK4 is linked to pulmonary edema.