26 April 2025, Boston—Chronic thromboembolic pulmonary hypertension (CTEPH) often goes undiagnosed for years, is frequently caught late, and primarily affects young, otherwise healthy individuals. Even when diagnosed, finding the right treatment can be challenging.
At this year’s Annual Meeting and Scientific Sessions of the International Society for Heart and Lung Transplantation (ISHLT), a day-long CTEPH conference—co-sponsored by ISHLT and the International CTEPH Association (ICA) —brought together worldwide experts who presented ground-breaking outcomes research on treatments for the disease.
“In the past, patients with CTEPH were, on average, misdiagnosed for several years,” said presenter Michael M. Madani, MD, FACS, Executive Director-Surgery, UC San Diego Health Cardiovascular Institute and co-chair of the CTEPH conference. “Due to educational events like this, it’s down to about 12-18 months.
“Unfortunately, we still see patients who’ve been treated for asthma, COPD, and obesity for years before someone considers CTEPH,” he said.
The session covered new medications, refined treatment techniques, innovative treatment approaches, and case-based clinical decision-making.
Key takeaways
The Cause of CTEPH
- Old blood clots, or pulmonary embolisms (PEs), get trapped in the blood vessels of the lung. Some resolve on their own while large PEs may be lethal.
- In patients with CTEPH, chronic unresolved PEs form scar tissue inside the vessels.
CTEPH Diagnosis
- The average length of misdiagnosis has decreased from years to approximately 12-18 months
- The overall incidence rate is about 2-5 percent but may be increasing due to better recognition. It’s critically important for physicians to consider other factors leading to clot formation in the lungs
- About 50 percent of patients who experience CTEPH have no history of deep vein thrombosis or acute PE
- Patients with pacemakers and other intravenous catheters or devices may be prone to clots in the lung vessels
- Radiologists must be trained to detect small chronic clots in the lung vessels on CT scans
CTEPH by the Numbers
- Accounts for up to 15 percent of pulmonary hypertension patients
- Affects men and women equally
- The median age of patients is 50. Dr. Madani has operated on patients as young as six and as old as 90
- The number of CTEPH surgeries (to remove scar tissue from the lung vessels, called a pulmonary endarterectomy) performed in the US has roughly doubled in the past decade. Most CTEPH patients who undergo successful surgery can live a normal lifestyle.
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About ISHLT
The International Society for Heart and Lung Transplantation (ISHLT) is a not-for-profit, multidisciplinary, professional organization dedicated to improving the care of patients with advanced heart or lung disease through transplantation, mechanical support, and innovative therapies via research, education, and advocacy. ISHLT members focus on transplantation and a range of interventions and therapies related to advanced heart and lung disease.
The ISHLT Annual Meeting and Scientific Sessions will be held from 27-30 April at the John B. Hynes Veterans Memorial Convention Center in Boston.
Contact:
Jess Burke, Director of Marketing and Communications
+1.312.224.0015
jess.burke@ishlt.org