Aftermath of a blood clot brings deadly pressure in the lungs

Aftermath of a blood clot brings deadly pressure in the lungs


Source: Pittsburgh Post Gazette

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 and a pulmonary function test.

“After the tests, they called me up and told me to go straight to the hospital and don’t drive,” he said.

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 these patients, a series of tests can determine if they are eligible for surgery to remove these clots from within the artery — offering a chance of curing this otherwise life-threatening problem.

This surgery, called pulmonary thromboendarterectomy, or PTE, is offered in very 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 considered as 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 underdiagnosed 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 Hypertension Program, the surgery is not performed at this time, according to pulmonologist Belinda Rivera-Lebron.

“It’s much more difficult technically than heart surgery for coronary disease,” she said. “The clot is not easily accessible.”

Few doctors are trained in the specialty, first developed in 1977 at the University of 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 mid-Atlantic 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 Pittsburgh area 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 open-heart surgery — and shutting off the blood supply.

A video of the surgery used in Dr. Moraca’s presentations shows how then the pulmonary artery is opened up. Slowly and carefully the fibrous tissue inside is separated from the vessel wall (“it’s like super glue,” Dr. Moraca said). The surgeon’s hands methodically pull the vessel to reach the farthest part possible and then continue to peel away the blockage with small forceps.

Dr. Moraca recalled a patient he operated on in 2014: a 19-year-old woman flown in from Virginia, unable to breathe. “Her pulmonary pressure was so high, she had horrible [right heart failure from her CTEPH],” he said. “Now she’s back to a normal life.”

Five days after his surgery, which was followed by time in the ICU and a step-down unit, Mr. Faix said he was feeling much better and felt ready to start tying flies again.

His pulmonary pressure was back to normal.

“I just know how lucky I got,” he said. 

Referred to Allegheny General in February by his doctors at the University of Rochester was Greg Young, 50, of Rochester. Originally, he had been diagnosed with a pulmonary embolism and was hospitalized in his hometown for a week in December.

“They gave me a clot buster and that seemed to make it a little better,” he said. But after leaving the hospital, he had a scan that showed blockages in the lung arteries.

“It revealed the extent of the clotting and scar tissue,” he said. It was CTEPH and he was told by his pulmonologist it would take surgery to fix.

Mr. Young drove down to Pittsburgh for preoperative testing, then had the surgery and 11 days later was sent home.

In about a month, he said, he felt much better, and he’s back playing basketball with his kids.

Across all the centers that do the surgery, some blockage remains in the smallest, farthest vessels for about 30 percent of patients. Dr. Moraca said Allegheny General’s average is 16 percent. He said he’s traveling to Italy to study new techniques for reaching blockages in vessels far within the lungs.

Dr. Moraca, surgical director of the CTEPH program, and cardiologist Manreet Kanwar, its associate medical director, lead a collaboration of specialists — in cardiac surgery, cardiology, anesthesiology, critical care, pulmonology, radiology and nursing.

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.

“Not every patient is a candidate for surgery; not every patient wants surgery,” Dr. Rivera-Lebron said. UPMC Presbyterian has about 10 to 15 CTEPH patients each year, she said. 

“The only curative is the surgery as far as we know. There are patients who have the the angioplasty and then have normal pressure. It might resolve the pulmonary hypertension, improve your symptoms, your breathing, the echocardiogram. But we cannot say you’re technically cured. The clot is still embedded in the wall.”

Dr. Rivera-Lebron said she foresees a new focus on following up with patients who have had a pulmonary embolism.

“Normally they just follow up with their primary care doctor,” she said. “They don’t have a followup echocardiogram that would show if the heart is under strain or if the clot has resolved. … For about 2½ years, we’ve had a rise in patients who had an embolism and have chronic disease.”

She said patients may be on medication for pulmonary hypertension, but haven’t tried Riociguat.

“Doctors in the community might not know it exists,” Dr. Rivera-Lebron said. “They might come in for angioplasty. We have a increase in referrals from local pulmonologists and cardiologists for patients who don’t want surgery.”

Jill Daly: or 412-263-1596.


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