Pulmonary Arterial Hypertension: Diagnosis, Treatment, and Management

Amanda Balbi

Senior Managing Editor, Consultant360

Balbi A. Pulmonary arterial hypertension: diagnosis, treatment, and management. Consultant360. Published online June 4, 2021.


Pulmonary arterial hypertension (PAH) is a rare disorder in which there is high blood pressure in the arteries that bring blood from the right side of the heart to the lungs, usually with idiopathic origins.1 However, PAH can also be heritable/genetic or associated with other conditions.1 An estimated 15 to 50 persons per million are affected in the United States.2

PAH is part of a larger category of pulmonary vascular disorders called pulmonary hypertension (PH). Classifications of PH include (1) PAH, (2) PH secondary to left heart disease, (3) PH from chronic lung diseases and/or hypoxia, (4) PH due to pulmonary artery obstructions, and (5) PH from unexplained or multifactorial mechanisms.2

The prognosis is poor for patients with PAH, so making a prompt diagnosis and treatment plan is crucial, especially for patients’ quality of life.

Screening and Diagnosis

To make a definitive diagnosis of PAH, an in-depth patient history, physical examination, and diagnostic workup are needed. The presenting symptoms are not unique to PAH, which can make diagnosing it difficult. The following symptoms are common among patients with PAH and should be elucidated during the history-taking and physical examination processes:1,3

  • Dyspnea, especially upon exertion
  • Chest pain or palpitations
  • Fainting episodes
  • Fatigue
  • Edema of the feet, legs, abdomen, and/or neck
  • Bluish lips and/or fingers


Studies have shown that women, non-Hispanic Black individuals, and individuals aged 75 years or older are also at an increased risk for PAH.1,5 Moreover, the following conditions increase the risk for PAH and should be screened further:4

  • Congenital heart disease
  • Connective tissue disease
  • Coronary heart disease
  • Hypertension
  • Liver disease, specifically cirrhosis
  • Blood clots to the lungs
  • Chronic lung disease, such as emphysema
  • Heart failure


The gold standard of diagnostic testing for PAH includes blood tests, electrocardiography scanning, lung function testing, 6-minute walk tests, doppler transthoracic echocardiography (TTE) scanning, and right heart catheterization.2,6,7


According to the latest guidelines, the TTE results should give the clinician an understanding of the disease progression, which will help guide treatment options.8 The first step in treatment is usually to counsel on general measures, prescribe supportive therapy such as oral anticoagulants and diuretics, refer to a PAH-specialized care center, and test for acute vasoreactivity to determine whether chronic calcium channel blocker therapy is warranted.8

More than 14 medications are currently approved to treat PAH, including vasodilators, antiproliferative agents, calcium channel blockers, and more.9 Among the recommended agents are:9

  • Epoprostenol
  • Treprostinil
  • Iloprost
  • Treprostinil
  • Bosentan
  • Ambrisentan
  • Sildenafil
  • Tadalafil
  • Amlodipine
  • Warfarin
  • Spironolactone
  • Digoxin


Surgical management may also be required for certain patients.10 If initial treatments fail, lung transplantation is recommended, along with combination drug therapy.8 Follow-up visits every 3 to 6 months to assess treatment efficacy and overall patient status are also recommended.8


PAH is a rare pulmonary vascular disorder that carries a poor prognosis. Early detection and treatment can help improve the quality of life for patients with PAH, but advanced disease may warrant lung transplantation. With more research on potential treatment options, clinicians can expect more approved medications and updated guidelines for PAH.




1. Pulmonary arterial hypertension. National Organization for Rare Diseases. Published 2021. Accessed June 4, 2021.

2. Levine DJ. Pulmonary arterial hypertension: updates in epidemiology and evaluation of patients. Am J Manag Care. 2021;27(3 Suppl):S35-S41.

3. Pulmonary arterial hypertension symptoms and diagnosis. American Lung Association. Updated October 23, 2020. Accessed June 4, 2021.

4. Pulmonary hypertension. Centers for Disease Control and Prevention. Updated December 3, 2019. Accessed June 4, 2021.

5. Silveyra P, Fuentes N, Rodriguez Bauza DE. Sex and gender differences in lung disease. In: Wang YX, ed. Lung Inflammation in Health and Disease, Volume II. Springer; 2021:227-258.

6. Arcasoy SM, Christie JD, Ferrari VA, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med. 2003;167(5):735-740.

7. Fisher MR, Forfia PR, Chamera E, et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med. 2009;179(7):615-621.

8. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67-119.

9. FDA-approved treatments for pulmonary hypertension. Stanford Medicine. Accessed June 4, 2021.

10. Mayer E, Jenkins D, Lindner J, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011;141(3):702-710.