CTEPH or chronic thromboembolic pulmonary hypertension is a potentially curable form of pulmonary hypertension. The possible cure is an intense surgery called PTE or pulmonary thromboendarterectomy. The goal of PTE is to remove blood clots from arteries in the lungs. Removing the clots allows for blood to flow from the right side of the heart through the lungs and back to the left side of the heart without increased pressure. The results are improved oxygenation of the entire body and decreased work load on the right side of the heart.
What Should I Expect During Evaluation for PTE Surgery?
The first step of treating CTEPH is to receive an appropriate diagnosis by a Pulmonary Hypertension or CTEPH expert. A PH Center of Excellence will be able to screen for and diagnose CTEPH via VQ scan and or pulmonary angiography. Because the PTE surgery is very delicate and difficult it is important to work with your PAH provider to carefully choose which center to go to. Centers of Excellence have been recognized for their programs that promote best practices in patient care, quality, and safety. These centers have years of experience performing hundreds of PTEs per year. Their outcomes are superior to centers with less experience. We refer our patients to UC San Diego.
Once the referral has been made to the PTE specialty center a coordinator from that program will reach out to the patient and give instructions on filling out appropriate paperwork that they will require. The physician making the referral will have already sent medical records to the PTE center. The PTE team will inform the patient and their physician if any additional tests are necessary to determine candidacy for surgery. Depending on the location of the center and the cost/convenience of visiting the center an appointment may be set up to meet the PTE team and discuss the surgery, other patients will meet the PTE team just prior to surgery. It is important to work with the center to plan the approximate 3 week stay that will be required for surgery and recovery.
Not all patients will be good candidates for PTE. Patients with comorbidities such as pulmonary fibrosis may not qualify. Other patients have inoperable CTEPH meaning the clots are too numerous and possibly in the smaller blood vessels of the lungs that cannot be adequately accessed by surgery.
How is PTE Surgery Performed?
The pulmonary thromboendarterectomy is an 8-10 hour surgery. The patient’s chest is opened through their sternum. The right and left pulmonary arteries are addressed one at a time. A small incision is made in the pulmonary artery and the old blood clots that are compressed against the wall of the blood vessel are carefully removed. The fibrotic old clotted material is meticulously dissected as completely as possible. At times during the procedure, the patient’s heart is stopped, and the patient is placed on a heart-lung bypass machine. They are cooled to a hypothermic level to protect their organs during the periods when the heart is stopped and the brain and body are supported by the heart-lung bypass machine. You can watch a video of Dr. Michael Madani of UC San Diego performing PTE surgery by clicking here.
What Happens After PTE Surgery?
Following the surgery the patient will be transported from the operating room to the cardiac intensive care unit. They will be there for 3-10 days before transferring to a medical floor of the hospital. After a few more days the patient will be allowed to leave the hospital but will be required to come back for daily exams and blood tests to make sure their blood thinners are at the appropriate level. Generally patients are allowed to return home after about 3 weeks.
CTEPH patients with a successful PTE will often return home off of PAH therapies. They will require blood thinners for the rest of their life to prevent chronic clots from reforming.
What If The PTE Surgery Doesn’t Work?
For some CTEPH patients PTE surgery improves symptoms but doesn’t cure their pulmonary hypertension. These patients have persistent CTEPH. Other patients develop new chronic clots and have recurrent CTEPH after surgery. These patients like all CTEPH patients will be treated with blood thinners. They may also be placed on Adempas, the only FDA approved medication for CTEPH as well as other PAH treatments not specifically designed for CTEPH.