A recent survey found that many primary care physicians are unfamiliar with chronic thromboembolic pulmonary hypertension (CTEPH) and slow to refer CTEPH patients to specialists, although the disease is treatable and potentially curable. The survey was sponsored by Bayer to raise awareness about the condition and the need for more education to improve  diagnosis and treatment.

CTEPH is a rare and life-threatening form of pulmonary hypertension (PH) that leads to high blood pressure in the lungs and can lead to right heart failure. Approximately 600,000 people have an acute pulmonary embolism each year in the U.S., and 1 in every 25 of these patients eventually develop CTEPH, even if treated with anticoagulants for at least three months. Possibly because the disease is poorly understood, estimates of new CTEPH cases in the U.S. range from 500 to 2,500 annually.

“Because surgery can be an effective treatment for CTEPH, it is critical that physicians suspect and diagnose the condition.  Unfortunately, as demonstrated by this survey, recognition of CTEPH among primary care physicians is inadequate,” said Richard Channick, MD, Director of Massachusetts General Hospital’s Pulmonary Hypertension and Thromboendarterectomy Program. “We very much need further education to identify and treat these patients as quickly as possible.”

The Bayer survey involved 604 primary care physicians (PCPs, or internal and family medicine physicians, and general practitioners), cardiologists, and pulmonologists questioned online about CTEPH in August 2015.

The survey’s main findings include, according to a press release:

• Further education on the preferred diagnostic test to identify CTEPH is needed. Disease symptoms include shortness of breath, fatigue, light-headedness, fainting, and chest discomfort. Because these symptoms are nonspecific, CTEPH is under-diagnosed and often misdiagnosed.  A ventilation/perfusion (VQ) scan is the preferred test for screening for chronic thromboembolic disease and should be viewed as an initial step in a diagnosis. But 38 percent of cardiologists and pulmonologists surveyed did not know that the VQ is the preferred screening test for CTEPH.

• Surgery is substantially under-recognized as a potential cure for CTEPH. The standard and most effective treatment for CTEPH is pulmonary thromboendarterectomy (PTE), in which blood vessels of the lungs are cleared of clot and scar material. More medical centers have begun performing PTE surgeries, but the number of physicians who recognize PTE as a potential cure for CTEPH remains low — 20 percent of PCPs, 43 percent of cardiologists, and 64 percent of pulmonologists surveyed. Furthermore, 50 percent of PCPs categorized it as a last resort, rather than a cure (14 percent) or treatment (33 percent).

• Awareness is lacking in the connection between pulmonary embolism and CTEPH. A pulmonary embolism, or a sudden blockage in a lung artery usually caused by a blood clot traveling from a leg vein, is known to lead to CTEPH in substantial numbers of people. Furthermore, an early and accurate CTEPH diagnosis is crucial, as it is a potentially curable form of PH. Yet, according to the survey results, while 82 percent of specialists thought primary care physicians should refer PE patients to them immediately, but only 45 percent of PCPs said they would.

• Specialists want PCPs to more quickly refer patients with symptoms of lung disease, including CTEPH. Fifty-five percent of cardiologists and pulmonologists surveyed wish that primary care physicians better understood the specific signs and symptoms of CTEPH and pulmonary arterial hypertension (PAH), and were quicker to refer those patients to specialists.

“Together with treating physicians, our goal is always to find ways to raise awareness about this misunderstood disease,” said Dario Mirski, MD, Vice President and Head, U.S. medical affairs at Bayer. “For patients with CTEPH, the benefits of surgery may be very positive, and we hope the results of this survey help drive conversations about optimal diagnostic tools and treatment among physicians and the patient community.”