Overview
Chronic thromboembolic pulmonary hypertension, often called CTEPH (pronounced 'see-tef'), is a serious condition where old blood clots block the arteries that carry blood from the heart to the lungs. Unlike a fresh blood clot that can dissolve with medication, these clots become hard and scar-like over time, narrowing the blood vessels and making it very hard for the heart to pump blood through the lungs. This causes high blood pressure in the lung arteries — which is what 'pulmonary hypertension' means. Because the heart has to work much harder than normal, it gradually becomes strained and weakened. People with CTEPH often feel extremely tired, short of breath, and unable to do everyday activities they used to manage easily. Some people develop CTEPH after a known pulmonary embolism (a blood clot in the lungs), but in others, the clots were never noticed before diagnosis. The good news is that CTEPH is one of the few forms of pulmonary hypertension that can potentially be cured. A surgery called pulmonary endarterectomy (PEA) removes the scar tissue from the lung arteries and can dramatically improve or even normalize blood pressure in the lungs. For people who cannot have surgery, a procedure called balloon pulmonary angioplasty (BPA) and medications such as riociguat (Adempas) are available. With the right treatment, many people with CTEPH can live longer, more active lives.
Also known as:
Key symptoms:
Shortness of breath, especially during physical activityExtreme tiredness or fatigue that does not improve with restFeeling dizzy or lightheaded, especially when activeFainting or near-fainting spellsChest pain or pressureSwelling in the legs, ankles, or feetBluish tint to the lips or fingertips (called cyanosis)Rapid or irregular heartbeatCoughing, sometimes with bloodReduced ability to exercise or do physical tasks
Clinical phenotype terms (37)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsPoitiers University Hospital
Damascus University
Kerckhoff Klinik
Rutgers, The State University of New Jersey
Beijing Chao Yang Hospital — NA
Irina E. Chazova
Suqiao Yang — PHASE3
Dr Sudarshan Rajagopal — PHASE3
Ester Ashraf Faried Beshay
Chinese University of Hong Kong — PHASE4
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
2 availableXARELTO
for reduction in the risk of recurrence of DVT or PE
Adempas
Treatment of adults with persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) WHO Group 4, after surgical treatment, or inoperable CTEPH, to improve exercise capacity and WHO fun…
Treatment of adults with persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) WHO Group 4, after surgical treatment, or inoperable CTEPH, to improve exercise capacity and WHO functional class
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Chronic thromboembolic pulmonary hypertension.
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Questions for your doctor
Bring these to your next appointment
- Q1.Am I a candidate for pulmonary endarterectomy surgery, and what would make me eligible or ineligible?,If I cannot have surgery, is balloon pulmonary angioplasty an option for me?,Which blood thinner is best for me, and how will we monitor it?,Should I be tested for inherited blood clotting disorders?,What symptoms should prompt me to go to the emergency room immediately?,How often do I need follow-up appointments and what tests will be repeated?,Are there any clinical trials I should consider?
Common questions about Chronic thromboembolic pulmonary hypertension
What is Chronic thromboembolic pulmonary hypertension?
Chronic thromboembolic pulmonary hypertension, often called CTEPH (pronounced 'see-tef'), is a serious condition where old blood clots block the arteries that carry blood from the heart to the lungs. Unlike a fresh blood clot that can dissolve with medication, these clots become hard and scar-like over time, narrowing the blood vessels and making it very hard for the heart to pump blood through the lungs. This causes high blood pressure in the lung arteries — which is what 'pulmonary hypertension' means. Because the heart has to work much harder than normal, it gradually becomes strained and
How is Chronic thromboembolic pulmonary hypertension inherited?
Chronic thromboembolic pulmonary hypertension follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Chronic thromboembolic pulmonary hypertension typically begin?
Typical onset of Chronic thromboembolic pulmonary hypertension is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Chronic thromboembolic pulmonary hypertension?
Yes — 20 recruiting clinical trials are currently listed for Chronic thromboembolic pulmonary hypertension on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Chronic thromboembolic pulmonary hypertension?
25 specialists and care centers treating Chronic thromboembolic pulmonary hypertension are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.