Treatments

Pulmonary Hypertension Treatments

Pulmonary hypertension (PH) is a progressive disease, which means it gets worse over time, even when treated with the latest approved treatments.

Medications

Several medications are approved to treat PH. How well medications work and the side effects may vary. This depends on the type of PH being treated.

Patients who do not improve enough within the first two months of medication therapy may need to switch to a different medication. The reason for the switch is that delay may allow the disease to progress and become harder to treat. Sometimes, more than one medication may be used.

Medication options may include:

• Diuretic therapy (water pills): to decrease leg swelling; may help patients breathe more easily
• Anticoagulant therapy (blood thinners): to decrease blood clots
• Calcium channel blockers: to lower blood pressure
• Endothelin receptor antagonists (ERAs): to prevent blood vessels from narrowing
• Phosphodiesterase inhibitors (PDE 5 inhibitors): to help the lungs make natural vasodilators, which open blood vessels and allow blood to flow more easily, and improve heart function
• Oxygen supplementation (home oxygen): to help patients breathe more easily and improve heart function

Procedures

Northwestern Medicine specializes in the following two surgeries that require special training and expertise:

• Lung transplantation: for patients whose symptoms continue to get worse and who have right heart failure. This surgery removes the diseased lungs and replaces with donor lungs. Heart-lung, double-lung and single-lung transplant options are shown to have been effective for PH.
• Atrial septostomy: for patients with PH and heart failure. The procedure creates a hole in the heart to connect the two top chambers (or atria) of the heart. This allows the blood to pass from the right side of the heart to the left side of the heart. This avoids the high resistance in the lungs caused by PH.