International Diagnosis and Management Practices for CTEPH Inadequate, Study Finds

An international study to assess clinical practices in the diagnosis and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) found several differences in CTEPH management among regions, but also a gap between guideline procedures and real-life practice of diagnosis and treatment.

The study, “An International Physician Survey of Chronic Thromboembolic Pulmonary Hypertension Management,” was published in the journal Pulmonary Circulation.

The study was conducted in five European countries (France, Germany, Italy, Spain, and the United Kingdom), the United States, Argentina, and Japan. A total of 496 physicians participated in the survey, including specialists from PH centers as well as non-PH centers who provided medical records of 1,748 patients with CTEPH.

Across all regions, physicians reported that at diagnosis, the majority of patients had significant functional limitations and were referred to them by pulmonologists, cardiologists, or internal medicine physicians for evaluation or treatment.

During diagnosis assessment, 81 to 98 percent of patients underwent echocardiography, the most commonly used technique. Other methods, such as right heart catheterization, computed tomography (CT) angiography, lung function test, and chest x-ray, were used less frequently and varied widely among regions. Only 33 to 54 percent of patients underwent a ventilation/perfusion (V/Q) scan during their diagnostic workup, even though this is recommended for all patients with pulmonary hypertension to screen for CTEPH.

Pulmonary endarterectomy (PEA), a surgical method to clear the pulmonary blood vessels, is the standard treatment for CTEPH patients and is potentially curative. However, it is not always considered in the management of the disease.

The study found that Japan had the lowest rate of PEA assessment, with only 25 percent of patients evaluated for it. In Europe, 44 percent of the patients were evaluated for PEA, while in the U.S. and Argentina, one-third of patients were evaluated.

In all regions, these low PEA evaluation rates were mainly due to patient refusal and low referral for PEA treatment, with surgery only considered if drug therapy failed. Additionally, the authors reported that evaluation for PEA was more common in PH centers than in non-PH centers.

Single drug therapy was the most common therapeutic strategy, with 58 to 73 percent of CTEPH patients receiving it. Phosphodiesterase 5 inhibitors, such as Adcirca or Revatio, were commonly used in the U.S. and Europe as a monotherapy, but also in combination with other drugs, such as endothelin receptor antagonists (Volibris, Tracleer, or Opsumit) and prostaglandins (Flolan, Ventavis, or Remodulin).

Overall, the authors considered that the major CTEPH inadequate management practices found in this study were related to the low usage of V/Q scans during diagnosis and the low rate of PEA evaluation and referral.

“Results from this study, which includes a global aspect of CTEPH care, demonstrate not only regional differences in CTEPH management but, more importantly, considerable nonadherence to the diagnosis and treatment guidelines for CTEPH, even in PH centers,” the authors wrote.

“These findings indicate that while algorithms for the management of CTEPH exist, they are not closely followed and that there is a need for more education of physicians managing patients with possible CTEPH.”