CTEPH Treatment Options

Each time a patient is referred to our Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Program, physicians representing all components of our practice – surgical, interventional, and medical – come together to collectively assess the patient’s condition and determine the best course of treatment. Regardless of the treatment, the goals are the same – thoroughly remove blockages, restore blood circulation in the lung, subsequently maintain that ideal circulation, and improve the patient’s quality of life. Your therapy may include one or more of the following treatments.

Pulmonary Thromboendarterectomy (Surgery)

BWH is one of only a few medical centers in the U.S. that offers pulmonary thromboendarterectomy (PTE), an advanced surgical procedure for removing blockages in the lungs, as a treatment for CTEPH.

CTEPH involves chronic clotting of the pulmonary arteries. These clots turn into a mass of scar tissue that blocks circulation in the blood vessels. This blockage, in turn, causes blood pressure to increase dramatically and places great strain on the right ventricle of the heart, the part of the heart that pumps blood into the lungs. The goal of a PTE procedure is to remove as much of this chronic clot material as possible in order to lower blood pressure in the pulmonary arteries and relieve strain on the right ventricle.

In order for a patient to be a candidate for this surgery, the clot must occupy a significant amount of the pulmonary artery tree, and the clot must be accessible for the surgeon. Blockages that start toward the central (and largest) branches of the lung are easier for a surgeon to access and remove.

During the procedure, a patient is placed on a heart-lung machine, which temporarily takes over the duties of a patient’s heart and lungs. Critical to the successful performance of a PTE procedure is the ability to adequately visualize the inside of the pulmonary artery tree, and especially its smaller, terminal branches. To facilitate this improved visualization, the patient’s body is cooled to a very low temperature. This allows the heart-lung machine to be turned off safely for periods of up to 15 minutes or so, which creates a bloodless operating field. This helps the surgeon to better see the affected area of the lung and ensure that the clot has been thoroughly removed. The low temperatures protect the brain and other organs from damage during these periods when the heart-lung machine is turned off.

Following surgery, a patient typically remains in the hospital for one to two weeks, including two to four days in intensive care. While recovering in the hospital, the patient will have a filter placed in the major vein in the lower body, the inferior vena cava, in order to prevent subsequent blood clots from reaching the lungs. Soon after surgery, patients will commence taking blood-thinners to help prevent further clots from forming and possibly affecting the lungs.

Interventional (Catheter-Based) Treatment

We also are a leader in employing interventional techniques to successfully treat CTEPH. When blockages are isolated in the distal branches, just beyond the main branches of the lungs, a catheter-based treatment – in the hands of an experienced cardiologist – can be just as effective as surgery.

Blockages in these areas aren’t accessible with a surgeon’s hands, but an interventional cardiologist can reach them with a catheter (long, slender, flexible medical tool) equipped with a balloon at its tip. Once the catheter reaches the blockage site, the balloon is inflated to break up the blockage. A stent (short, expandable tube) also may be inserted to keep the blood vessel open.

Since this is not an invasive procedure, the interventional cardiologist may choose to treat your CTEPH over the course of several sessions. This not only helps to improve outcomes by easing the physical demands on an interventional specialist, but it also gives the physician an opportunity to periodically and carefully evaluate patients during their course of treatment.

In addition to being used as a primary treatment, this interventional approach is sometimes used after surgery to improve outcomes in patients who still have some residual distal blockage.

Medical Management

When medical management is called for, we use largely the same type of medications (anticoagulants) that would be used for any form of pulmonary hypertension. Our pulmonary medicine and vascular medicine specialists collaborate to optimize anticoagulation in CTEPH patients and also investigate the use of new agents.

In addition to being used as a primary treatment, anticoagulants will be prescribed for patients after an interventional or surgical procedure to help prevent the development of new clots and maintain the patient’s improved lung function.

We also offer medical treatment for patients who have inoperable, persistent pulmonary hypertension (PH) that doesn't involve the chronic clotting that characterizes CTEPH. Aside from anticoagulants, our medical management team offers a variety of other medications that help reduce pulmonary blood pressure in patients with PH.

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