The Current Landscape of Pulmonary Hypertension in Myeloproliferative Neoplasms

Pulmonary hypertension (PH) is an increasingly recognized complication of Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). Recent data suggest prognostic implications of both cardiovascular morbidity and mortality and hematologic progression. Additionally, PH and MPNs share common pathophysiologic mechanisms that bridge these two seemingly different disorders. The landscape of PH in MPNs is evolving in the face of improved survivorship and reduced disease progression, leading to increased incidence and prevalence of concurrent and/or consequent cardiovascular disease. Prospective studies are necessary to refine screening protocols, risk stratification, and prognostic and therapeutic implications of PH in this population.