Aftermath of blood clot brings deadly pressure in lungs
When he couldn’t trek through woods and hills near a family cabin in Allegheny National Forest to get to the best spots for fly-fishing, Richard Faix of Monroeville said he knew his shortness of breath was serious.
“I was tired of people telling me I had asthma,” said Mr. Faix, 63, an ultrasound imaging specialist. He got a new pulmonologist, who ordered a CAT scan anda pulmonary function test.
“After the tests, they called me up and told me to go straight to the hospital and don’t drive,” hesaid.
The blood pressure in his lungs was very high, an uncommon disorder known as pulmonary hypertension. At Forbes Hospital, radiologists reviewed the scans and found signs of an even rarer condition known as chronic thromboembolic pulmonary hypertension — scar tissue left by a clot in the lung blood vessel.
Although Mr. Faix doesn’t recall having a clot, CTEPH is caused by either an existing clot or fibrous scar tissue blocking the vessel. Most blood clots start in the legs, and if treated correctly with blood thinners, they typically dissolve. But if they don’t go away, patients are left with residual clots that can become lodged in their lungs, and over time, these patients can develop pulmonary hypertension. For such patients, a series of tests can determine whether they are eligible for surgery to remove the clots from within the artery — offering a chance of curing an otherwise life-threatening problem.
The surgery, called pulmonary th rom bo end arte rec to my, or PTE, is offered at a few centers in the U.S., including at Allegheny General Hospital. When surgery is not an option, there are medications and a procedure called balloon pulmonary angioplasty to lower the pressure. Rarely, a lung transplant is consideredas a last option.
Untreated,cases with the highest pulmonary pressures have a poor two-year survival rate, according to Robert Moraca, cardiovascular and thoracic surgeon at Allegheny General. Unfortunately, he said, “It’s a highly under diagnosed condition .”
He has been doing the delicate PTE procedure there since 2012, and Mr. Faix was his 52nd patient. Dr. Moraca’s first
patient, Jean Magazzu of Mt. Lebanon, interviewed back then by the Post-Gazette, is still
doing well, her doctor reported.
Although CTEPH is also treated in UPMC’s Comprehensive Pulmonary HypertensionProgram, the surgery is not performed at this time, according to pulmonologist BelindaRivera-Lebron.
“It’s much more difficult technically than heart surgery for coronary disease,” she said. “The clot is not easily accessible.”
Dr. Rivera-Lebron said UPMC has turned from doing surgery to treat CTEPH and now focuses on the best use of balloon angioplasty and Riociguat, a recently approved drug to lower pulmonary pressure in patients who can’t undergo surgery or who continue to have pulmonary hypertension after surgery.
Fewdoctors are trained in the PTE surgery, first developed in 1977 at the Universityof California, San Diego.
Dr. Moraca said there are about 2,500 new cases of CTEPH diagnosed in the U.S. each year. Patients from throughout the MidAtlantic region who seek the surgery are referred to Allegheny General.
Together with a team of specialists, Dr. Moraca reviews the referrals. In a recent weekly review, he said, nine were considered: two from Inova Hospital in Fairfax, Va., two from the University of Virginia, one from the University of Rochester, one from Johns Hopkins University and three from Pittsburgharea hospitals.
Dr. Moraca said the San Diego surgeons now treat more than 200 patients a year, and other busy centers have 20-40 patients. The overall mortality rate is about 5 percent, he said.
The right side of a patient’s heart overworks if CTEPH is not treated, said Raymond Benza, who leads Allegheny Health Network’s pulmonary hypertension program along with fellow cardiologist Srinivas Murali. Dr. Benza explained that the right ventricle fights against the high pressure in the lungs as it strains to pump blood into the pulmonary arteries.
“If the right heart is then stretched out, it can’t go back,” he said. “That’s how people die of this disease. It’s the only form of pulmonary hypertension we can cure; being able to peel away the blockage.” If pressure is normal after surgery, the heart will return to its normal shape, he said.
The surgical procedure involves cooling the patient’s body to about 61-64 degrees — as with openheart surgery — and shutting off the blood supply.
Dr. Moraca recalled a patient he operated on in 2014: a 19-year-old woman flown in from Virginia, unable to breathe.“Herpulmonarypressure was so high, she had horrible [right heart failure from her CTEPH],” he said. “Now she’sbacktoanormallife.”
Five days after his surgery, which was followed by time in the ICU and a stepdown unit, Mr. Faix said he was feeling much better. His pulmonary pressure was back to normal.
“I just know how lucky I got,” he said.