Pulmonary Hypertension Treatment at Columbia

There is no cure for pulmonary hypertension but an extensive range of treatments developed over the past decade have greatly improved quality of life for patients with the disease. Columbia's Pulmonary Hypertension Comprehensive Care Center has been involved in nearly every clinical trial involving medications for pulmonary hypertension patients, and we are developing some of the most novel and advanced therapies available for children, adolescents, and adults with this disease.

As part of a world-renowned medical center, we are able to bring together a team with extraordinary expertise in the treatment and management of this disease. Our team includes some of the best heart and lung transplant surgeons in the world. 

Your treatment plan will depend upon the type and severity of your or your child’s disease. Once we have determined an accurate diagnosis, we will develop a comprehensive treatment plan that may address the underlying cause of your pulmonary hypertension as well as the disease itself. Treatment is complicated and may change over time, so monitoring and follow-up care is a critical part of every patient's plan.

Treatments can range from medication alone, to novel surgeries, to lung transplant. We will involve you every step of the way.

Medications

Your treatment may include one or more medications. Your physician will discuss the medications that are part of your treatment and any potential side effects.

• Anticoagulants (blood thinners): Blood thinners help prevent the formation of blood clots in the small pulmonary arteries.
• Diuretics (water pills): Diuretics help eliminate excess fluid from your body's tissues and arteries, reducing swelling and fluid buildup in your lungs and helping your heart do less work.
• Blood vessel dilators (vasodilators): Vasodilators open narrowed blood vessels. There are many different types of these medications, which can be given as an oral medication, through an inhaler device, or continuously through an intravenous (IV) catheter connected to a small pump that fits on your belt or subcutaneous infusion pump into the skin. Different types of vasodilators have different side effects.
• Prostanoids: These medications are vasodilators that can be delivered orally, by inhalation or through a subcutaneous or intravenous pump to relax the lung   blood vessels. 
• Endothelin receptor antagonists (ERA): These medications help prevent blood     vessels from narrowing. 
• Phosphodiesterase Inhibitors (PDE 5 Inhibitors): These medications open the      blood vessels in the lungs to allow better blood flow.
• Soluble guanylate cyclase (SGC) stimulator: This medication interacts with nitric oxide to help relax and lower pressure in the blood vessels of the lungs. 
• Oxygen: Oxygen therapy involves breathing pure oxygen to treat low oxygen in your blood. In severe cases of pulmonary hypertension continuous oxygen therapy may be necessary. 
• Calcium channel blockers: These drugs help decrease blood pressure by relaxing the muscles in the walls of your blood vessels. Calcium channel blockers are effective in a small number of people with pulmonary hypertension. 
• Digoxin: Digoxin can help strengthen your heartbeat and control your heart rate.

Surgery

When medication alone is no longer effective, there are interventional procedures and surgical treatments for certain forms of pulmonary hypertension. We are one of a few programs in the country that offer curative surgery for chronic thromboembolic pulmonary hypertension (CTEPH) and a novel modified Potts shunt for advanced pulmonary arterial hypertension.

• Balloon pulmonary angioplasty: A minimally invasive interventional procedure in which the lung arteries, blocked by a clot, can be opened up by inserting a tiny balloon and inflating it inside the artery. This surgery is performed using X-ray imaging to guide a long thin tube called a catheter, which is inserted through a tiny incision in the neck or leg vessel, to the artery. The balloon opens up the artery, lowering pressure and reducing strain on the right side of the heart. 
• Unidirectional valved shunt (reverse Potts shunt): A surgery in which a tube connection is established from the main pulmonary artery to the descending aorta.  This diverts blood to the body without having to go through the damaged lungs. It takes off strain from the right ventricle in cases of severe pulmonary hypertension.
• Atrial septostomy: An interventional procedure in which one of our cardiology experts creates an opening between the upper left and right chambers of your heart (atria) to relieve the pressure on the right side of your heart with a catheter.
• Lung or heart-lung transplant: When other treatments fail, some people with pulmonary hypertension are candidates for a lung or heart-lung transplant. 
• Pulmonary thromboendarterectomy (PTE): PTE surgery can reverse pulmonary hypertension in some patients with chronic thromboembolic pulmonary hypertension, a rare form of pulmonary hypertension caused by clotting in the lungs. A surgeon removes all clots from the arteries in the lung enabling the lung pressure to normalize. 

• Angioplasty or stenting: These interventional procedures can be helpful for patients who have pulmonary hypertension due to pulmonary vein stenosis. (We also start many of these patients on medications to slow the progression of disease and to decrease chances of the veins narrowing again.)

About Pulmonary Hypertension