The physicians and team members of our Pulmonary Hypertension (PH) Program are fully focused on the care of PH patients. Their expertise and experience helps them determine the best course of treatment for each patient. We are one of only a select number of treatment centers nationwide to offer the highly complex and leading edge treatments we do.
Once the diagnostic phase has been completed, your pulmonary hypertension team will discuss the findings in detail with you and your family. Your primary and referring physicians will also receive a detailed summary so they can help you understand some of the recommendations and future plans.
Pulmonary hypertension treatments will be chosen based on several factors, including the severity of PH, the rate of disease progression and which medications are best for the patient. Treatment options generally fall into four categories: treating the underlying disease, targeted PH treatment, combination therapy, and surgery.
Treatments Targeting Underlying Disease
Treatments that target an underlying disease may include:
- Lung disease treatments, which may include medications, therapy and surgery.
- Heart disease treatments, which may include the use of Digoxin, an oral medication to improve the strength of the heart muscle, medications for blood pressure control and other heart treatments.
- Oxygen therapy, provides an extra oxygen supply to patients, usually through a portable container with plastic tubing that feeds oxygen to the nose and/or mouth.
- Continuous positive airway pressure (CPAP), a simple treatment for people diagnosed with sleep apnea that uses air pressure to open airways during sleep.
- Anticoagulants (“blood thinners”), used to decrease the formation of blood clots in lung blood vessels and improve the blood flow in the lungs.
- Diuretics (“water pills”), to remove excess water from the body, allowing the heart muscle to pump easier and more efficiently.
- Pulmonary rehabilitation, a comprehensive, individualized approach to treating respiratory problems in which therapy, education and exercise help patients achieve their maximum exercise potential.
Targeted Pulmonary Hypertension Treatment
Most pulmonary hypertension patients are treated with various medications that target one of three “pathways” that affect the blood vessels in the lungs. These medications are most commonly used in Group 1 pulmonary artery hypertension patients and only select patients in other groups. Pulmonary rehabilitation may also be included in the treatment plan.
Oral medications help slow the progression of the disease by relaxing blood vessels in the lungs, and may include:
- Endothelin receptor antagonists, shown to block the harmful “endothelin pathway,” a narrowing of blood vessel walls in the lungs caused by a substance in the body called endothelin.
- Phosphodiesterase-5 inhibitors, used to open the blood vessels in the lungs by enhancing what is called the “nitric oxide pathway” in the body.
- Calcium channel blockers, also open blood vessels in the lungs but only in a small subset of patients who show a favorable response during heart catheterization testing.
Infusion therapies use more potent medications to treat severe cases of PH. They are essential for patients with the most severe forms of the disease. These therapies act by enhancing the “prostacyclin pathway” in body, and may include:
Epoprostenol — This medication is given intravenously 24 hours a day for an extended period of time through a permanent catheter inserted in one of the larger neck or chest veins. Patient understanding of and involvement in the therapy is required to make sure the treatment is delivered safely. This was the first therapy approved for treatment of Group 1 pulmonary artery hypertension patients and the most potent so far.
Treprostinil — This medication can be delivered on a long-term basis intravenously using the same catheter used for epoprostenol (above) or through a very small plastic needle that is inserted into the skin of the abdomen. A small external device clipped to a person’s clothing pumps medication underneath the skin through the needle.
Inhaled therapies also use potent medications to treat severe cases of PH. This group of treatments also acts by enhancing the “prostacyclin pathway” in the body. This treatment approach requires patients to inhale medications many times a day through a customized inhalation device.
Careful combinations of treatments are often required to alleviate the complex group of conditions that may be present for pulmonary hypertension patients. Specialized training and experience among the Pulmonary Hypertension Program physicians help them craft the most effective treatment approach for each patient.
When other treatment options do not provide effective symptom control for pulmonary hypertension patients, surgical options may be carefully recommended in consultation with both pulmonary and cardiothoracic surgery specialists.
- Lung transplant — Lung transplantation is recommended for patients with advanced PH who are not responding to medical treatments. While the use of the available therapies has led to a decline in the need for transplantation, it is possible that your physician will discuss this with you during the initial visit(s).
- Pulmonary thromboendarterectomy (PTE) — A pulmonary thromboendarterectomy (PTE) is a highly specialized and complex procedure, which removes blood clots from the arteries in the lungs of patients with chronic thromboembolic pulmonary hypertension or CTEPH. When PTE is appropriate, it can be a cure for patients with CTEPH. Given the complexity of the procedure, and the experience needed by the physicians, PTE is only performed at select hospitals across the country.
During a PTE procedure, blood clots caused by CTEPH are skillfully removed from arteries in the lungs, one pulmonary artery at a time. As these clots are removed, healthy blood flow is restored from the right side of the heart through the lungs and back to the left side of the heart. This results in improved oxygenation and decreased work load on the right side of the heart. After recovery from the procedure most patients will be symptom free and be able to return to physical activity without becoming short of breath. For some patients, pulmonary rehabilitation may be needed.
Additional Interventions for PH Treatment
Depending on the type and severity of your pulmonary hypertension and the presence of other conditions (e.g., sleep apnea, emphysema or heart failure) the PH team may recommend additional interventions as part of a multidisciplinary approach, such as referral to:
- One of our cardiologists to arrange for a diagnostic heart catheterization. The cardiologist may also address any specific cardiac issues such as heart arrhythmias (abnormal heart beats) or blockage of coronary vessels (for those patients who don’t already have a primary cardiologist).
- A rheumatologist, a specialist with expertise in managing conditions in which the immune system is overactive, leading to inflammation of organs such as the heart, lungs, muscles and joints. Conditions that fit in this category include lupus, scleroderma and rheumatoid arthritis.
- Pulmonary rehabilitation, a special type of physical therapy that concentrates on improving the strength of your breathing muscles and overall endurance and stamina. Many patients who go through pulmonary rehabilitation sessions enjoy a noticeable improvement in exercise tolerance.