Rapid improvement in pulmonary hypertension related with hereditary hemorrhagic telangiectasia by iloprost inhalation in a postpartum patient, a case report

Background: Postpartum pulmonary arterial hypertension (PAH) complicated with hereditary hemorrhagic telangiectasia (HHT) is a rare condition. Diagnosing and treating PAH in patients with HHT can be challenging. To the best of our knowledge, no previous reports have investigated the efficacy of pulmonary vasodilators in improving hemodynamics in postpartum patients with this disease. Case presentation In this paper, we report a postpartum case of HHT combined with PAH, which presented with aggravation of dyspnea. Genetic testing revealed that the patient carried a heterozygous variant of activin receptor-like kinase 1 ( ACVRL1 ). Conclusions: The patient received virous treatments, including diuretics, anticoagulants, disidenafil, macitentan, inhalation of nitric oxide (iNO), and iloprost. Changes in PaO 2 /FiO 2 , pulmonary artery systolic pressure (PASP), and NT-proBNP suggested that, with the exception of iloprost inhalation, the other treatments appeared to have limited efficacy.