Pulmonary hypertension in patients with chronic and end-stage kidney disease

Pulmonary hypertension is defined as a mean pulmonary artery pressure ≥25 mm Hg and is a recently recognized complication of chronic kidney disease and end-stage renal disease. There is significant epidemiological overlap with kidney disease and the underlying causes of World Health Organization group 1-4 pulmonary hypertension (pulmonary arteriopathy, left heart disease, chronic pulmonary disease, and chronic thromboembolic disease, respectively). In addition, an entity of 'unexplained pulmonary hypertension,' group 5, in patients with chronic kidney disease and end-stage renal disease has emerged, with prevalence estimates of 30-50%. The pathogenesis of pulmonary hypertension in this population is due to alterations in endothelial function, increased cardiac output, and myocardial dysfunction leading to elevated left heart filling pressure, with recent data suggesting that left heart dysfunction may account for the vast majority of pulmonary hypertension in patients with kidney disease. Pulmonary hypertension is an independent predictor of increased mortality in patients on dialysis and those undergoing kidney transplantation. This review summarizes what is known about the epidemiology, pathogenesis, transplantation outcomes, mortality, and treatment of pulmonary hypertension in patients with chronic kidney disease and end-stage renal disease.