Improved prognosis for chronic thromboembolic pulmonary hypertension over recent decades

Suita, Japan – Chronic thromboembolic pulmonary hypertension (CTEPH) is the clinical term for high blood pressure in the lungs caused by blockages, such as clots, in the blood vessels. Although CTEPH therapies have improved over recent decades, trends in patient prognosis remain unclear. Now, Japanese researchers have analyzed patient data from 1980 to 2023 to reveal that the long-term prognosis of patients has significantly improved in recent years. 

Patients with CTEPH have historically had poor outcomes because of limited treatment options. However, recent advances in surgical techniques and drug development mean that there are now a range of treatments available. For example, in pulmonary endarterectomy, blood clots are surgically removed from the lungs. If this technique is ineffective or cannot be performed, patients can undergo balloon pulmonary angioplasty, where balloons are used to open or widen the blood vessels. In addition, multimodal therapies (combining these surgical techniques, balloon pulmonary angioplasty, and medical therapy) are now commonly tailored to individual patients and may improve prognosis. However, the prognosis of patients with CTEPH over time has not been studied—until now.

In a study published recently in European Respiratory Journal, researchers from the National Cerebral and Cardiovascular Center in Japan retrospectively studied 834 patients with CTEPH. They categorized the patients into early (1980–1999), middle (2000–2010), and current (late 2010–2023) eras. The middle and current eras were characterized by the introduction of pulmonary endarterectomy and balloon pulmonary angioplasty, respectively.

“In these three eras, we were particularly interested in the long-term prognosis of patients,” explains lead author of the study Takatoyo Kiko. “We also looked at the proportion of patients from each era who did not receive CTEPH therapy, and assessed trends in the adoption of multimodal treatment.”

The research team found that the long-term prognosis of patients with CTEPH significantly improved over time, with 68% of patients surviving to 5 years in the early group, 85% in the middle group, and 93% in the current group. Over the same time period, the proportion of patients who did not receive therapy decreased from 65% in the early group to 3% in the current group, and multimodal therapy adoption increased from 0% to 58%.

“Together, our findings indicate that there has been an increase in treatment rates and multimodal therapy adoption from 1980 to the present, with associated improvements in patient prognosis,” says Takeshi Ogo, senior author of the study. “This is great news for patients with CTEPH.”

Although these results come from a single healthcare center in Japan, they are very promising for the future of CTEPH. If treatment advances continue, patient prognosis may keep improving into the next decades.