World Health Organization group 5 pulmonary hypertension

Abstract

World Health Organization (WHO) group 5 pulmonary hypertension (PH) entails a heterogeneous group of disorders that may cause PH by unclear and/or multiple mechanisms. In particular, group 5 includes PH caused by hematologic disorders, systemic diseases, metabolic disorders, chronic renal failure, and disorders leading to pulmonary vascular occlusion or compression. This article discusses common pathogenic mechanisms leading to group 5 PH, followed by a detailed overview of epidemiology, pathogenesis, and disease-specific management of the individual group 5 conditions. Off-label use of vasomodulatory therapies, typically indicated for pulmonary arterial hypertension (WHO group 1 PH), in group 5 conditions is also discussed.

Keywords: Fibrosing mediastinitis; Lymphangioleiomyomatosis; Metabolic disorders; Myeloproliferative disorders; Pulmonary Langerhans cell histiocytosis; Renal failure; Sarcoidosis; Splenectomy.