Living with pulmonary hypertension (PH) can be a challenging experience defined by unexpected fatigue and shortness of breath. Simple activities, such as climbing a flight of stairs or carrying groceries, may suddenly feel exhausting. This condition, characterized by high blood pressure in the arteries affecting the lungs and the right side of the heart, impacts everyone differently. For some, symptoms progress slowly, while others experience rapid changes in their physical abilities.

Treatment is critical to reduce the strain on the heart and improve the ability to be active. Without management, the high pressure can weaken the heart muscle over time, leading to heart failure. Because PH is classified into different groups based on the underlying cause such as lung disease, heart issues, or genetic factors, treatment plans are highly specific. What works for one patient may not be suitable/safe for another (American Heart Association, 2023).

Overview of treatment options for Pulmonary Hypertension

The primary goal of treatment is to lower the pressure in the pulmonary arteries, improve breathing, and allow the heart to pump blood more efficiently. Strategies differ significantly depending on the type of PH. For Pulmonary Arterial Hypertension (PAH), treatment focuses on specific medications that open blood vessels. For other types, such as PH caused by lung disease, therapy targets the underlying condition, often using oxygen or bronchodilators.

While lifestyle changes like low-sodium diets and activity modification are important, medication is the cornerstone of management for most patients. In severe cases that do not respond to drug therapy, surgical interventions or lung transplantation may be considered. However, for the majority of patients, the focus remains on finding the right combination of oral, inhaled, or infused medications.

Medications used for Pulmonary Hypertension

For patients with Pulmonary Arterial Hypertension (Group 1 PH), doctors typically prescribe medications that target specific pathways to relax blood vessels.

Vasodilators (PDE5 Inhibitors): Drugs like sildenafil and tadalafil are often the first line of defense. Originally used for other conditions, these oral medications are highly effective at relaxing the pulmonary blood vessels.

Endothelin Receptor Antagonists (ERAs): Medications such as bosentan and ambrisentan are oral pills that block substances in the blood that cause vessels to constrict. Clinical experience suggests these are often used in combination with other therapies to maximize results.

Prostacyclin Analogs: For more severe symptoms, doctors may prescribe prostacyclin therapies like epoprostenol or treprostinil. These can be administered via inhalation, continuous intravenous (IV) infusion, or subcutaneous pump. These are potent medications used to rapidly improve blood flow.

Supportive Medications: Beyond specific PH drugs, many patients require diuretics (water pills) to reduce fluid buildup in the legs and abdomen. Anticoagulants (blood thinners) like warfarin may also be used to prevent blood clots in the lungs.

Patients can generally expect to see improvements in their exercise capacity such as walking further without breathlessness within a few months of starting therapy (Pulmonary Hypertension Association, 2022).

How these medications work

The major drug classes for Pulmonary Hypertension (PH) balance natural chemicals to control blood vessel width.

PDE5 Inhibitors protect nitric oxide, keeping blood vessels open by preventing the breakdown of chemicals in that pathway, thus maintaining relaxation and width.

ERAs block endothelin, a powerful constrictor, preventing it from tightening blood vessels.

Prostacyclins mimic a often-deficient natural hormone. They both powerfully widen blood vessels and prevent blood clotting, reducing the heart’s pumping resistance (National Heart, Lung, and Blood Institute, 2022).

Side effects and safety considerations

These powerful blood flow altering medications often cause side effects. Vasodilators commonly result in headaches, facial flushing, and dizziness from low blood pressure. ERAs, specifically bosentan, need monthly blood tests to monitor for liver stress.

Certain drugs, like ERAs and guanylate cyclase stimulators (e.g., riociguat), cause severe birth defects and are contraindicated in pregnancy. Women of childbearing age taking these drugs may need to enroll in special safety programs. Patients should seek immediate medical attention for fainting, sudden swelling, or severe chest pain. Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care.

References

1. American Heart Association. https://www.heart.org
2. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov
3. Pulmonary Hypertension Association. https://www.phassociation.org
4. Mayo Clinic. https://www.mayoclinic.org