Pulmonary hypertension (PH) - NYSORA

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Pulmonary hypertension (PH)

Learning objectives

  • Describe the definition, clinical classes and treatment of pulmonary hypertension
  • Manage patients with pulmonary hypertension

Definition & mechanisms

  • Pulmonary hypertension is defined as a mean pulmonary artery pressure (MPAP) >25 mmHg at rest or 30 mmHg on exercise
  • The cause of pulmonary hypertension is of critical importance as it defines subsequent treatment
  • Patients are classified into five clinical groups:
    • Group 1: Pulmonary arterial hypertension (PAH). Idiopathic PAH, connective tissue disease-associated PAH, and congenital heart disease (mainly Eisenmenger syndrome) are the most common subgroups; others include aortopulmonary, HIV, and drug/toxin-induced PAH
    • Group 2: PH associated with increased pulmonary capillary wedge pressure (post-capillary PH), due to left-sided heart disease
    • Group 3: Lung disease and chronic hypoxia-related PH
    • Group 4: Predominantly chronic thromboembolic PH (CTEPH)
    • Group 5: PH caused by multisystemic disorders or multiple/unknown mechanisms, including sarcoidosis and hematological conditions
  • Adequate coupling of the right ventricle to the pulmonary circulation is essential for maintaining cardiac output in patients with PH


  • Pulmonary vasodilator therapy is predominantly indicated for the treatment of patients with Group 1 PH and Group 4 PH, although the treatment of choice for CTEPH is pulmonary endarterectomy
  • Management of PH in patients with Group 2 and 3 PH is focussed on the underlying heart or lung disease
  • Transplantation (usually bilateral lung) remains the ultimate treatment option in PAH when other treatments fail


pulmonary hypertension, rv function, hemodynamics, vasodilator, sinus, rhythm, filling, persusion, inotropy, pulmonary vascular resistance, PVR, hypoxia, hypercarbia, acidosis, hypothermia, sympathetic stimulation, central venous pressure, systemic arterial pressure, hypotension, norepinephrine, vasopressin, nitrous oxide, NO, prostacyclin, iliprost, milrinone, sildenafil, preload, afterload, dobutamine, dopamine, extracorporeal membrane oxygenation, ICU, HDU

RV, right ventricle; PVR, pulmonary vascular resistance; ICU, intensive care unit; HDU, high dependency unit.

Suggested reading

  • Price L, Brame A, Martinez G, Mukerjee B, Harries C, Kempny A, et al. Perioperative management of patients with Pulmonary Hypertension undergoing Non-Cardiac Surgery: A Systemic Review and UK Consensus Statement. European Respiratory Journal. 2020;56(suppl 64):1467.
  • Elliot CA, Kiely DG. Pulmonary hypertension. Continuing Education in Anaesthesia Critical Care & Pain. 2006;6(1):17-22.

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