Pulmonary Arterial Hypertension (PAH)

In pulmonary arterial hypertension (PAH), thickening and narrowing of the pulmonary arteries increase blood pressure in the lungs.¹ Because of the changes affecting the pulmonary arteries and lungs, the heart must exert extra effort to pump blood. During disease progression and overwork, the heart muscle weakens, with the possibility of heart failure.^1,2

PAH has no cure; however, several treatments are available to control symptoms and improve patients’ quality of life.³ In addition, some steps can be taken to prevent complications of the disease. Prevention focuses primarily on measures to promote cardiac health, including adequate physical activity, a healthy and balanced diet, and avoidance of smoking.⁴ Other strategies and lifestyle changes may also decrease the risk for the development of PAH symptoms as patients live with the disease.

Vaccinations

PAH increases the risk for pneumonia, which is the cause of death in patients with PAH in 7% of cases. Vaccination against influenza and pneumococcal pneumonia is therefore recommended to prevent infection.⁵ 

Physical Activity

Patients with PAH can lead full, active lives. Various studies have demonstrated that training programs can improve muscle function and quality of life.^5,6  Regular moderate exercise can reduce blood pressure and consequently cardiac events in patients with PAH; however, a few precautions should be taken.⁷ Any physical program that puts a strain on the lungs and heart and increases the pulmonary artery pressure should be avoided.^4,5,7 Inappropriate exercises might include activities such as heavy lifting and bench press.⁷ Patients with PAH should always discuss physical activity with their medical care team before initiating any program and should consider participating in a supervised cardiopulmonary rehabilitation program if recommended.^4,7

Pregnancy Recommendations

Pregnancy in women with PAH can have life-threatening consequences for both mother and baby.^7,8 During pregnancy, increased plasma volume and decreased systemic vascular resistance result in an increase in cardiac output.⁸ PAH prevents the normal, pregnancy-related decline in systemic vascular resistance, leading to a further rise in pulmonary arterial pressure.  Women with PAH who are of childbearing age should be counseled regarding birth control to prevent pregnancy; they should also keep in mind that estrogen-based methods of contraception can exacerbate the disease because of the thrombogenic potential of estrogen, and that progesterone-based methods should be used instead.^7,8 To prevent complications, women who choose to become pregnant must be monitored at least monthly by a multidisciplinary care team including specialists in pulmonary hypertension, cardiologogy, obstetrics, anesthesiology, and neonatology.⁸

Nutrition

Certain foods and vitamins may interact with medications and contribute to the formation of edema.⁹ Research has also indicated that deficiencies of vitamin C, vitamin D, and iron may trigger conditions that exacerbate PAH.¹⁰ Patients with PAH should therefore maintain a nutritionally balanced diet to prevent disease progression. Consuming a healthy diet of lean meats, vegetables, whole grains, fruit, and low-fat dairy and reducing salt intake can prevent edema, nausea, and discomfort.^7,9 The intake of alcoholic drinks should be limited.⁷ Saturated fat, trans fat, and cholesterol should be avoided.³ 

Additional Preventive Measures

Patients with PAH should use supplemental oxygen during air travel and should avoid high altitudes. Avoidance of smoking is crucial for reducing cardiac events and sudden cardiac death. Other precautions include antibiotic therapy for significant respiratory tract infections and before dental procedures to prevent endocarditis in patients who have congenital heart defects.⁷

References

1. Learn about pulmonary arterial hypertension. American Lung Association. Updated October 23, 2020. Accessed March 9, 2022.

2. Pulmonary arterial hypertension. National Organization for Rare Disorders (NORD). Accessed March 9, 2022.

3. Treating and managing pulmonary arterial hypertension. American Lung Association. Updated October 23, 3030. Accessed March 9, 2022.

4. Prevention. Her Heart. Accessed March 9, 2022.

5. Galiè N, Humbert M, Vachiery J-L, 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). Eur Heart J. 2016;37(1):67-119. doi:10.1093/eurheartj/ehv317

6. Exercise and PH. Pulmonary Hypertension Association. Accessed March 9, 2022.

7. Pulmonary hypertension and CHD. American Heart Association. Accessed March 9, 2022.

8. Pieper PG, Hoendermis ES. Pregnancy in women with pulmonary hypertension. Neth Heart J. 2011;19(12):504-508. doi: 10.1007/s12471-011-0219-9

9. Diet and nutrition. Pulmonary Hypertension Association. Accessed March 9, 2022.

10. 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

Reviewed by Hasan Avcu, MD, on 3/28/2022.

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Diana Diez Gaspar, PharmD, PhD

Diana earned her PhD and PharmD with distinction in the field of Medicinal and Pharmaceutical Chemistry at the Universidade do Porto. She is an accomplished oncology scientist with 10+ years of experience in developing and managing R&D projects and research staff directed to the development of small proteins fit for medical use.