Department of Medicine
Pulmonary Hypertension & Thromboendarterectomy Program
Pulmonary Hypertension and Thromboendarterectomy Program
Explore This Treatment Program
At the Pulmonary Hypertension and Thromboendarterectomy Program at Massachusetts General Hospital, we use a multidisciplinary approach to pulmonary hypertension (PH) that allows us to develop a plan of care that is individualized to your needs. All approved medical and surgical treatments for pulmonary hypertension are available to you. Our involvement in numerous research trials allows us to provide you and your family with unique treatment options and the most up-to-date therapies.
We understand that being diagnosed with PH can be scary and confusing for many patients, which is why our team is dedicated to supporting and educating patients and their families on the disease. We encourage you to learn as much as possible about your disease, medications and therapies. We run an active Patient Support Group, which is composed of doctors, nurses, family and friends who share their personal stories about PH to help educate each other in a welcoming environment.
The care you receive from us extends beyond your office visit. We make every effort to ensure that you continue receiving the best possible care when you return home and make ourselves available to you for any questions and concerns you may have.
Your First Appointment
If you or your doctor think that you have pulmonary hypertension, please call our office at (617) 724-3705. Our coordinators will take your information and ask that you send your records to our office (if completed outside the Mass General Brigham network). Our team of nurses and doctors will review your records to determine the best course of action. Sometimes, more testing will be ordered before your appointment. These tests may include:
- Echocardiogram: an ultrasound of your heart that can estimate the pulmonary pressures and heart function
- Pulmonary function test: these tests measure your lung function and how well your lungs work
- Ventilation perfusion scan: a test to see if you have blood clots in your lungs
- CT chest: a detailed picture of your lungs, more detailed than a chest x-ray
- Blood work
At the time of your first appointment, you and your doctor will go over your symptoms and review the results of your testing. A right heart catherization might be recommended to directly measure the pressure in the right side of your heart and the pulmonary artery. We utilize a collaborative, multi-disciplinary approach which brings together pulmonary and critical care doctors, interventional cardiologists, cardiothoracic surgeons, pulmonary therapy and nursing to provide the best care possible.
About This Program
What is Pulmonary Hypertension?
Pulmonary hypertension is a serious disease, defined as high blood pressure in the blood vessels of the lungs, and can result in right heart failure. The right side of the heart receives deoxygenated blood from the body and then pumps this blood into the lungs via blood vessels called pulmonary arteries, where the blood is filled with oxygen again. Pulmonary hypertension occurs when the pulmonary arteries become thickened and narrowed, causing the pressure in this system to become elevated. The high pressure causes the right side of the heart to work harder to pump blood to the lungs. As a result, the right heart can become weak and enlarged. This can lead to shortness of breath, swelling of the legs or a decreased ability to exercise.
There are many reasons for developing pulmonary hypertension. The first type of pulmonary hypertension is also called pulmonary arterial hypertension (PAH). PAH can be idiopathic (meaning that the cause is unknown), heritable (due to a genetic mutation that is passed on in families) or due to an underlying disease such as HIV, connective tissue disease (e.g. scleroderma, lupus) or some types of liver disease.
Another cause of pulmonary hypertension is heart disease. In this case, the left side of the heart is damaged and causes a back up of pressure to the right side of the heart.
Other causes of pulmonary hypertension include lung disease (such as COPD, pulmonary fibrosis, sleep apnea), pulmonary emboli (blood clots in the lungs) and other rare causes.
Some people may have no symptoms of pulmonary hypertension in its early stages. However, other people may experience the following:
- Shortness of breath with movement or exertion
- Feeling tired or fatigued
- Inability to exercise
- Lightheadedness or dizziness when walking or going up stairs
- Swelling in your feet, ankles or abdomen
- Chest pain
Pulmonary hypertension can be a treatable disease. All available therapies for pulmonary hypertension are offered by our program. The goals of treatment are to improve your symptoms and slow the progression of the disease.
Pulmonary Hypertension Medications
There are three categories of pulmonary hypertension medications:
- Nitric Oxide Pathway Agents: These medications are oral therapies that increase the beneficial effects of nitric oxide on the pulmonary blood vessels. These medications work to relax the pulmonary arteries to allow the blood vessels to dilate. This reduces the pressure in the pulmonary arteries and allows blood to flow more easily. Examples of these medications include: sildenafil (Revatio), tadalafil (Adcirca) and riociguat (Adempas)
- Endothelin Receptor Antagonists: These medications are oral therapies that block the harmful effects of endothelin on the blood vessels. These medications help prevent blood vessels from narrowing. Examples of these medications are: bosentan (Tracleer), ambrisentan (Letairis) and macitentan (Opsumit)
- Prostacyclin Pathway Agents: This type of medication works to increase vasodilation (widen the blood vessels) in the pulmonary circulation and decrease pressure in the blood vessels supplying blood to the lungs. These medications can be given in different forms: either orally, intravenously, inhaled or subcutaneously (under the skin). These include: epoprostenol (Veletri, Flolan), treprostinil (Orenitram, Tyvaso, Remodulin), iloprost (Ventavis), selexipag (Uptravi)
- Diuretics (Lasix, Bumex, Torsemide, Spironolactone, Metolazone): Also called “water pills,” these medications help remove excess fluid that builds up in the body due to heart failure
- Anticoagulation: Also known as “blood thinners,” these medications are used to treat blood clots in the lungs
Lung transplantation: In advanced disease, and depending on your response to other treatments, you may be referred to our lung transplantation program.
Other Types of PH
Chronic thromboembolic pulmonary hypertension (CTEPH) is another type of pulmonary hypertension. It develops from reoccurring or unresolved pulmonary emboli (blood clots in the pulmonary arteries). A pulmonary embolism is a common condition that can be treated with medication to help break up the clot, usually a blood thinner. However, when the pulmonary embolism does not break up with medication, scar tissue may form inside the arteries. This chronic clot, or scar tissue, may cause blockages which lead to pulmonary hypertension.
In addition to medications, our team offers both pulmonary thromboendarterectomy (PTE surgery) and balloon pulmonary angioplasty (BPA) as treatments for CTEPH. The standard evaluation includes a thorough visit with a specialist in pulmonary hypertension and often additional testing, to decide on the best treatment option. A multidisciplinary team of pulmonologists and surgeons decide if a patient is an operative candidate for PTE, BPA or for continued medical therapy.
Standard Testing for CTEPH Evaluation
- Echocardiogram - to assess heart function and degree of pulmonary hypertension
- Chest CT (CTA) - to assess lung anatomy, other lung disease and central involvement of pulmonary arteries with clot and scar
- Ventilation Perfusion Scan (V/Q scan) - to assess the amount and location of obstruction of blood flow to the lung
- Pulmonary Function Tests (PFTs) - to screen for other lung diseases
- Right Heart Catheterization (RHC) - to assess the blood pressure in the lungs, the degree of obstruction in the lung blood vessels, and the function of the heart
- Pulmonary Angiogram (PA gram) - a dye study of the pulmonary arteries to assess the anatomy of the pulmonary arteries and the location of the obstructions
Pulmonary Thromboendarterectomy (PTE)
PTE is a highly specialized procedure in which chronic clots and scar that cause pulmonary hypertension are removed from the pulmonary arteries in the lungs. Removing these obstructions from the pulmonary artery increases blood flow to the lungs which improves both lung and heart function. PTE can be a curative procedure for this type of pulmonary hypertension. Patients are candidates for a PTE procedure if they are symptomatic from CTEPH, are otherwise in reasonable health, and have obstructions that are surgically accessible. Patients with more distal obstructions (in the outer portions of the lung) are often better candidates for a balloon angioplasty procedure.
The surgery usually takes about 6 hours and is done under general anesthesia. The incision for the surgery is similar to a typical heart surgery incision which involves dividing the breast bone to fully expose the heart and pulmonary arteries. The patient is placed on the heart-lung machine and cooled to about 20°C. Each pulmonary artery is opened and cleared of obstructing chronic clots and scar tissue. The patient is returned to the intensive care unit asleep and cared for by a nurse and intensivist. Typically, the patient is fully awake and weaned from the breathing machine over the next 12-24 hours, and most patients stay in the hospital for 7-10 days after the operation. Most patients notice an immediate improvement in the breathing after surgery. Our results were recently reported in the literature ( PMID 29284512, J Cardiothorac Surgery, 2017;12:121) and we published our technique in a book (Selke F and Ruel M, eds. Atlas of Cardiac Surgical Techniques, 2nd ed. Elsevier, 2018).
Balloon Pulmonary Angioplasty (BPA)
This procedure can be an option for patients who are not candidates for PTE surgery. BPA uses balloons to open blood vessels that are narrowed or blocked from chronic blood clots. This not only restores blood flow, but also reduces the pressure in the pulmonary artery and improves symptoms of shortness of breath. BPA is done by interventional cardiologists or interventional radiologists. The pulmonary arteries are accessed by placing a catheter in the large vein of the leg (femoral vein). The catheter is floated up toward the lungs through the large veins in the body. Each BPA procedure takes approximate 1.5-2 hours and following the procedure, the patient will spend the night in the hospital for observation. Patients usually need multiple procedures, on average 4-6, to get the full beneficial effect.
Why Choose Mass General For Treatment of CTEPH?
Failure to recognize CTEPH or misdiagnosis can be common. At Mass General, you will receive high quality, expert evaluation from multidisciplinary specialists including pulmonologists, cardiac and thoracic surgeons, and interventional cardiologists. Mass General is a leader in treating patients with CTEPH, with the largest CTEPH program in the region, and we are proud to report a high rate of successful outcomes.
In attempts to better understand PH and develop new, effective treatments, we are involved in many research studies. You may have the opportunity to participate in one of these cutting-edge studies. Your participation is, of course, completely voluntary and your decision regarding enrolling in a study will, in no way, affect the care you receive from us.
Meet Our Team
Pulmonologists Who Specialize in Pulmonary Hypertension
Josanna Rodriguez-Lopez, MD
Alison Witkin, MD
Surgeons in the PTE Program
Cameron Wright, MD
Surgical Director of PTE Program
Kenny Rosenfield, MD
BPA Interventional cardiologist
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