Pulmonary arterial hypertension

Med Clin (Barc). 2022 Jun 24;158(12):622-629. doi: 10.1016/j.medcli.2022.01.003. Epub 2022 Mar 9.
[Article in English, Spanish]


Pulmonary arterial hypertension (PAH) is a rare disease characterized by adverse remodeling of the arterial tree leading to increased vascular resistance with subsequent increase in right ventricular afterload and eventual development of heart failure. The nonspecific clinical manifestations and lack of knowledge of pathology lead to a poor prognosis associated with delay in diagnosis and initiation of treatment. The most recent recommendations focus on optimizing the early differential diagnosis with other causes of pulmonary hypertension to initiate appropriate treatment based on the mortality risk estimation. In the last years, with the improvement in the diagnostic process, the emergence of new specific treatments, and the creation of specialized referral units for this pathology, the prognosis, and quality of life of patients with PAH have improved significantly.

Keywords: Estratificación de riesgo; Hipertensión arterial pulmonar; Pulmonary arterial hypertension; Pulmonary vascular resistance; Pulmonary vasodilators; Resistencia vascular pulmonar; Right ventricle; Risk assessment; Vasodilatadores pulmonares; Ventrículo derecho.

Publication types

  • Review

MeSH terms

  • Heart Failure*
  • Heart Ventricles
  • Humans
  • Hypertension, Pulmonary* / diagnosis
  • Hypertension, Pulmonary* / etiology
  • Hypertension, Pulmonary* / therapy
  • Pulmonary Arterial Hypertension* / diagnosis
  • Pulmonary Arterial Hypertension* / etiology
  • Pulmonary Arterial Hypertension* / therapy
  • Quality of Life