Rapid improvement in postpartum pulmonary hypertension associated with hereditary hemorrhagic telangiectasia: A case report and review of literature

Abstract

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 SUMMARY In this paper, we report a postpartum case of HHT combined with PAH, presenting with worsening dyspnea. Genetic testing revealed that the patient carried a heterozygous variant of activin receptor-like kinase 1. The patient received various treatments, including diuretics, anticoagulants, sildenafil, macitentan, inhalation of nitric oxide, and iloprost. Changes in PaO2/FiO2, pulmonary artery systolic pressure as assessed by echocardiography, and N-terminus pro-brain natriuretic peptide levels suggested that, except for iloprost inhalation, the other treatments appeared to have limited efficacy. CONCLUSION To our knowledge, this is the first report on efficacy of pulmonary vasodilators in postpartum patients with HHT and PAH.