What are the causes of acute pulmonary hypertension (PH)?

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Last updated: March 2, 2025View editorial policy

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From the Guidelines

Acute pulmonary hypertension is caused by a variety of diseases, including idiopathic pulmonary arterial hypertension, left heart disease, lung diseases, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension with unclear and/or multifactorial mechanisms. The causes of acute pulmonary hypertension (PH) can be categorized into five groups:

  • Group 1: Pulmonary arterial hypertension (PAH), which includes idiopathic, heritable, and drug-induced PAH, as well as PAH associated with connective tissue disease, human immunodeficiency virus (HIV) infection, portal hypertension, and congenital heart disease 1.
  • Group 2: PH due to left heart disease, which includes left ventricular systolic dysfunction, left ventricular diastolic dysfunction, valvular disease, and congenital or acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies 1.
  • Group 3: PH due to lung diseases and/or hypoxia, which includes chronic obstructive pulmonary disease, interstitial lung disease, sleep-disordered breathing, and chronic exposure to high altitude 1.
  • Group 4: Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions, which includes chronic thromboembolic pulmonary hypertension, angiosarcoma, and other intravascular tumors 1.
  • Group 5: PH with unclear and/or multifactorial mechanisms, which includes haematological disorders, systemic disorders, metabolic disorders, and others 1. It is essential to identify the underlying cause of PH to provide targeted treatment and improve patient outcomes.

Some key points to consider when evaluating the causes of acute PH include:

  • A careful history evaluation to assess for risk factors, such as family history, history of drugs and toxins associated with PH, and symptoms such as dyspnea, fatigue, and palpitations 1.
  • The use of right heart catheterization to measure mean pulmonary arterial pressure and pulmonary vascular resistance, which can help to diagnose and categorize PH 1.
  • The importance of treating the underlying cause of PH, whether it is pulmonary embolism, left heart failure, or another condition, to improve patient outcomes 1.

Overall, the causes of acute pulmonary hypertension are diverse and require a comprehensive evaluation to diagnose and treat effectively.

From the FDA Drug Label

Hypoxic respiratory failure was caused by meconium aspiration syndrome (MAS; 49%), pneumonia/sepsis (21%), idiopathic primary pulmonary hypertension of the newborn (PPHN; 17%), or respiratory distress syndrome (RDS; 11%). Hypoxic respiratory failure was caused by MAS (35%), idiopathic PPHN (30%), pneumonia/sepsis (24%), or RDS (8%).

The causes of acute pulmonary hypertension (PH) include:

  • Meconium aspiration syndrome (MAS)
  • Pneumonia/sepsis
  • Idiopathic primary pulmonary hypertension of the newborn (PPHN)
  • Respiratory distress syndrome (RDS) 2 2

From the Research

Causes of Acute Pulmonary Hypertension (PH)

  • Acute respiratory distress syndrome 3
  • Left ventricular dysfunction 3
  • Pulmonary embolism 3
  • Cardiothoracic surgery 3, 4
  • Preexisting pulmonary vascular, lung, liver, or cardiac diseases 3
  • Decompensated right heart failure 5, 6
  • Critical illnesses such as septic shock and gastrointestinal bleeding 6

Underlying Pathophysiologic Derangements

  • Increased pulmonary vascular resistance and pulmonary arterial pressure 5
  • Endothelial dysfunction 7, 4
  • Right ventricular pressure overload and dysfunction 4

Triggers for Acute PH Exacerbation

  • Hypoxia and hypercarbia 5
  • Discontinuation of oral or intravenous PH medications 5
  • Underlying precipitants such as infection or thromboembolism 5, 6

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

The management of acute pulmonary arterial hypertension.

Cardiovascular therapeutics, 2011

Research

Critical care of patients with pulmonary arterial hypertension.

Current opinion in pulmonary medicine, 2020

Research

Sildenafil for Pulmonary Arterial Hypertension.

American journal of therapeutics, 2019

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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