A copy repairman who couldn’t do his job anymore and was forced to take medical leave has returned to work. A woman who couldn’t walk from her car to her desk job has enough energy to redecorate her house. A 42-year-old mother who was too sick to care for her children and was discharged to hospice is now back home with them and busy as ever.
They all came to Vanderbilt Heart & Vascular Institute (VHVI) with chronic thromboembolic pulmonary hypertension (CTEPH) and limited options. Patients with this condition often have a history of acute pulmonary embolism, or blood clots in the lung. For reasons that are poorly understood a small percentage of them do not resolve the clots, and they form chronic blood clots that turn into scar tissue and obstruct the pulmonary arteries. This puts strain on the right heart, which can lead to heart failure without treatment.
Nov. 18 is CTEPH Awareness Day. The Pulmonary Hypertension Association has designated this day as part of its efforts to increase awareness of this type of pulmonary hypertension, the methods for diagnosis and the available treatment options.
VHVI offers a specialized surgical approach – called pulmonary thromboendarterectomy – to treat this often fatal form of hypertension. Fewer than 10 medical centers in the country offer this approach.
“Many of these patients see a doctor who says there is nothing that can be done for them. We’re one of the few medical centers in this part of the country doing this procedure,” said Michael Petracek, M.D., chair of Vanderbilt’s Department of Cardiac Surgery.
It’s a high-risk, complex operation reserved for a specific type of patient. Patients undergoing a pulmonary endarterectomy are placed on the heart-lung machine and the body temperature is cooled to 64.4 degrees Fahrenheit. The chest is opened via median sternotomy.
“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,” 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.”
Diagnosing the problem is difficult and is confirmed by pulmonary angiography. Patients often present to pulmonologist Ivan Robbins, M.D., professor of Medicine, with shortness of breath, on oxygen and able to walk for only short distances without having to rest.
“Usually patients will come with very nondescript symptoms and an echocardiogram, showing evidence of pulmonary hypertension, is often the first clue that a patient has CTEPH,” Robbins said. “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.”
Ideally, CTEPH patients should undergo surgery before they develop heart failure, but even those with cardiac dysfunction can benefit greatly from thromboendarterectomy.
“People don’t realize there’s treatment for this type of pulmonary hypertension. This is one that is very curable, but it requires a big operation that not many medical centers do,” said Petracek, who is training his surgical colleagues on the procedure. “We’ve got a unique situation at Vanderbilt because we’ve got people here who really understand pulmonary hypertension.”
