Pulmonary Arterial Hypertension (PAH)

Pulmonary arterial hypertension (PAH) is a rare and progressive disease in which the arteries of the lungs present with high blood pressure.1 In patients with PAH, the pulmonary arteries become thickened, blocked, or even destroyed, affecting the blood flow through the lungs and consequently increasing the blood pressure in the lungs.2,3 The heart must then work harder to pump blood through the lungs, and this extra effort eventually weakens it.3 With the progression of PAH, patients experience symptoms such as fatigue, shortness of breath, fainting episodes, chest pain, heart rhythm changes, and edema of the feet, legs, abdomen, and neck.4

PAH is a serious condition that can greatly impact the quality of life of patients. The approach to PAH treatment is multidisciplinary and involves specialists including pulmonologists, cardiologists, respiratory therapists, nutritionists, and psychologists. 


Primary care physicians may often identify symptoms of PAH and refer patients toward specialized care such as pulmonologists. Pulmonologists are doctors specializing in the respiratory system. Patients with PAH need their pulmonary function assessed regularly. These specialists often coordinate the care provided by the medical team, and as lung and heart conditions may present with similar symptoms, pulmonologists may work closely with cardiologists.5


Monitoring of cardiac function is essential for patients with PAH, as PAH can lead to heart failure.1 A cardiovascular specialist will help in the diagnosis of the disease by performing several tests such as electrocardiogram (ECG), echocardiogram, and right heart catheterization.6,7 Cardiologists also care for PAH patients by creating treatment plans tailored to their needs.8

Respiratory Therapists

Respiratory therapists are specialists trained in pulmonary medicine who help patients with lung diseases.9,10 These specialists work together with physicians and nurses to assist in the diagnosis of respiratory conditions by performing pulmonary tests. They also help patients by providing respiratory therapy, including administering oxygen and conducting pulmonary rehabilitation.11 These specialists can provide guidance to patients with PAH and their families regarding the management of the disease.12 


Different foods and vitamins may interact with medications prescribed to PAH patients and contribute to edema. Controlling edema requires patients to control sodium consumption and fluid intake.13 Although further research is needed, recent data point to a correlation between iron deficiency and the severity of idiopathic PAH.14 A nutritionist can therefore help patients with PAH establish a healthy nutritional plan, ensuring adequate intake of calories and vitamins and providing recommendations on fluid intake. 


PAH can result in a progressive decline of patients’ mental health. Patients with PAH often feel uncertain about their future, which may lead to feelings of anxiety and depression. Psychological support may have a positive impact on patients living with PAH.15

Preparing for a PAH Specialist Visit

When preparing for a specialist appointment, patients may find it useful to prepare a few notes before the visit, including a list of questions or concerns to ask or discuss with the care team. These concerns can focus on understanding the cause of their condition, further testing that will be required to monitor the disease, how advanced their disease is, and/or recommendations for exercise or travelling.16


1. Pulmonary arterial hypertension. National Organization for Rare Disorders (NORD). Accessed March 7, 2022.

2. Learn about pulmonary arterial hypertension. American Lung Association. Updated October 23, 2020. Accessed March 7, 2022.

3. Pulmonary hypertension: symptoms & causes. Mayo Clinic. September 14, 2021. Accessed March 7, 2022.

4. Pulmonary arterial hypertension symptoms and diagnosis. American Lung Association. Updated October 23, 2020. Accessed March 7, 2022.

5. Kimble B. Know your providers: what does a pulmonologist do? American Lung Association. May 14, 2019. Updated December 7, 2021. Accessed March 7, 2022.

6. Cardiologist. National Health Service (NHS). Accessed March 7, 2022.

7. Pulmonary hypertension: diagnosis & treatment. Mayo Clinic. September 14, 2021. Accessed March 7, 2022.

8. Pulmonary hypertension. Weill Cornell Medicine. Accessed March 7, 2022.

9. What is respiratory therapy? American Association for Respiratory Care. Accessed March 7, 2022.

10. What RTs do. American Association for Respiratory Care. Accessed March 7, 2022.

11. Pulmonary hypertension and the respiratory therapist’s role in diagnosis and treatment. RT Magazine. August 6, 2010. Accessed March 7, 2022.

12. Respiratory therapist. Mayo Clinic College of Medicine and Science. Accessed March 7, 2022.

13. Diet and nutrition. Pulmonary Hypertension Association. Accessed March 7, 2022.

14. Callejo M, Barberá JA, Duarte J, Perez-Vizcaino F. Impact of nutrition on pulmonary arterial hypertension. Nutrients. 2020;12(1):169. doi:10.3390/nu12010169

15. Zhang J, Yin Y, Wen Y, Shi F, Wang J. Anxiety and depression in patients with pulmonary arterial hypertension in Northwest China: a cross-sectional study. Front Psychiatry. 2022;12:758120. doi:10.3389/fpsyt.2021.758120

16. Questions to ask your doctor about pulmonary arterial hypertension. American Lung Association. Updated October 23, 2020. Accessed March 7, 2022.

Reviewed by Harshi Dhingra, MD, on 3/6/2022.