A number of patients who were suffering from a type of pulmonary hypertension known as CTEPH, or chronic thromboembolic pulmonary hypertension, have gotten their normal life back after undergoing a breakthrough operation at the Vanderbilt Heart & Vascular Institute (VHVI). CTEPH develops in a small percentage of people who have a history of pulmonary embolisms, and without proper treatment, can lead to heart failure. Today’s CTEPH treatment options are few, but the VHVI is offering a highly effective surgical way to address the obstructive blood clots, and this information comes in light of the first ever CTEPH Awareness Day, observed November 18.
The surgical procedure is called a pulmonary thromboendarterectomy, and is currently available in less than 10 health institutions in the US, including VHVI. Dr. Michael Petracek, the chair of the Department of Cardiac Surgery at Vanderbilt, said it is not unusual for a CTEPH patient to seek medical attention and be told there is nothing that can be done about their condition, but this novel, life-saving procedure can change that.
However, the surgery comes with high risks, and is recommended only for a certain type of CTEPH patient. “They have no blood supply to the brain while I go in and take out scar tissue from their lungs. You have a short period of time to do it,” Dr. Petracek said. “I do both sides in 20-minute intervals and relieve their pulmonary hypertension in order to get their heart function back to normal.” During the procedure, patients are hooked to a heart-lung machine and cooled to 64.4 degrees Fahrenheit to lower the body’s metabolic rate, before a median sternotomy is performed to open the chest.
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Dr. Ivan Robbins, a professor of Medicine, said that accurately diagnosing CTEPH can be a challenge as patients would come presenting vague respiratory symptoms, which would prompt an echocardiogram to first determine the presence of PH. A suspected CTEPH case then requires confirmation via pulmonary angiography to visualize the blood clots. “Surgery can be curative and the results can be dramatic. We’ve had people with severe pulmonary hypertension, who, at the conclusion of surgery, have normal or near normal pressures in their lungs. Of all the forms of pulmonary hypertension, CTEPH is the only one that can be cured.” While doctors recommend that CTEPH patients undergo this surgery before symptoms of heart failure begin to manifest, those who have already incurred damage to the heart can can still benefit from a pulmonary thromboendarterectomy.
Because this procedure is relatively new, and only a few medical centers offer it, a large majority of patients with CTEPH are unaware it exists. PH Awareness Month is a good time to spread more information about less known treatment options.