Pulmonary Arterial Hypertension: a Pharmacotherapeutic Update

Curr Cardiol Rep. 2019 Nov 22;21(11):141. doi: 10.1007/s11886-019-1235-4.


Purpose of review: Pulmonary arterial hypertension (PAH) leads to progressive increases in pulmonary vascular resistance (PVR), right heart failure, and death if left untreated. This review will summarize and discuss recent updates in the classification and management of patients with PAH.

Recent findings: PAH requires careful hemodynamic assessment and is defined by a mean pulmonary artery pressure > 20 mmHg with normal left-sided filling pressures and a PVR ≥ 3 Wood units. Most patients with PAH require targeted pharmacotherapy based on multiparametric risk stratification. Significant improvements in clinical outcome have been realized through the approval of 14 unique pharmacotherapeutic options. The latest clinical recommendations provide the updated hemodynamic definition and clinical classification as well as evidence-based treatment recommendations. An important change is the focus on initial upfront combination therapy for most patients with PAH. Structured follow-up and escalation of treatment for those not achieving low-risk status is paramount.

Keywords: Chronic thromboembolic pulmonary hypertension (CTEPH); Pulmonary arterial hypertension; Pulmonary hypertension; WHO group 1 pulmonary hypertension.

Publication types

  • Review

MeSH terms

  • Algorithms
  • Biomarkers / blood
  • Cardiovascular Agents / therapeutic use
  • Disease Progression
  • Echocardiography
  • Heart Failure / etiology
  • Hemodynamics
  • Humans
  • Practice Guidelines as Topic
  • Pulmonary Arterial Hypertension / classification
  • Pulmonary Arterial Hypertension / diagnosis
  • Pulmonary Arterial Hypertension / drug therapy*
  • Pulmonary Arterial Hypertension / physiopathology
  • Pulmonary Artery / diagnostic imaging
  • Pulmonary Artery / drug effects
  • Pulmonary Artery / physiopathology
  • Risk Assessment


  • Biomarkers
  • Cardiovascular Agents