Rare Form of Pulmonary Hypertension May Be Underdiagnosed and Undertreated

Chronic thromboembolic pulmonary hypertension (CTEPH) may be underdiagnosed and undertreated, according to a literature review.

This rare condition occurs when a pulmonary artery obstruction leads to pulmonary vascular resistance. Pulmonary hypertension then worsens, and the individual with CTEPH may experience right ventricular failure. The pathogenesis of CTEPH is unknown, but one theory suggests it may be a complication of acute pulmonary embolism subsequent to venuous thromboembolism.

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However, CTEPH is difficult to diagnose because many patients do not have a history of pulmonary embolism, and the early signs and symptoms of CTEPH are nonspecific. As a result, referral to a specialist and diagnosis of CTEPH may be delayed. These factors means that CTEPH is likely underreported and that data on the worldwide burden of the condition are limited.

To better understand the incidence of CTEPH, researchers evaluated quantitative epidemiologic data from 25 publications and 14 databases across the United States, Europe, and Japan. The data were used to estimate future incidence and diagnosis rates in specific countries. In the US and Europe, annual incidence of CTEPH was 3 to 5 cases per 100,000 people, while the rate in Japan was 1.9 cases per 100,000 people.

In addition, the literature review suggested that only a small amount of CTEPH cases in the US and Europe (7% to 29%) were diagnosed.

“These data suggest that CTEPH is underdiagnosed and undertreated, and there is an urgent need to increase awareness of CTEPH. High-quality epidemiological studies are required to increase understanding of CTEPH,” the study’s authors concluded.

—Lauren LeBano

Reference:

Gail H, Hoeper MM, Richter MJ, Cacheris , Hinzmann B, Mayer E. An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe, and Japan. Eur Respir Rev. 2017;26:160121.