Pulmonary Hypertension Program
The physicians and team members of the Pulmonary Hypertension (PH) Program at Froedtert & The Medical College of Wisconsin 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.
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 DiseaseTreatments 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 TreatmentMost 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 medicationsOral 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 therapiesInfusion 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 basisi 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 therapiesInhaled 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.
Combination therapyCareful 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.
SurgeryWhen 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 at Froedert & The Medical College of Wisconsin.
Lung transplantation – Used in cases in which other forms of treatment are not effective. In the most severe cases, heart and lung transplantation may be advised. Learn more about lung transplantation at Froedert & The Medical College of Wisconsin.
Pulmonary thrombo endarterectomy – A highly specialized and complex surgery in which the blood vessels of the lungs are cleared of chronic clot and scar material to reopen the blocked blood vessels in the lungs.
See our FAQ list to learn more about what to expect during follow up patient care visits to the Pulmonary Hypertension Program.
Date: July 18, 2012
Online Editor(s): Richard Petre