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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Pulmonology

Understanding Your CTEPH Diagnosis: Validation and Next Steps

At a Glance

CTEPH is a rare but potentially curable condition caused by old blood clots that turn into scar tissue in the lungs. Treatment requires a multidisciplinary team and may include pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA), or targeted medications.

If you are feeling overwhelmed, frustrated, or even shocked by a diagnosis of Chronic Thromboembolic Pulmonary Hypertension (CTEPH), your reaction is completely valid. Many patients arrive at this diagnosis after a long, exhausting search for answers. CTEPH is a rare but serious condition that occurs when the body does not fully dissolve a pulmonary embolism (PE)—a blood clot in the lungs.

Understanding the Transition from Clot to Scar

In most people, the body’s natural systems dissolve blood clots over time. However, for about 2% to 7% of people who survive a PE, those clots do not disappear [1][2]. Instead, they undergo a process called organization, where the soft clot transforms into firm, fibrous scar tissue that becomes permanently stuck to the walls of the blood vessels [3].

This scar tissue creates physical blockages that:

  1. Restrict Blood Flow: The narrow or blocked “pipes” in your lungs make it harder for blood to pick up oxygen.
  2. Increase Pressure: Your heart must pump much harder to force blood through these narrowed pathways, leading to pulmonary hypertension (high blood pressure in the lung arteries).
  3. Strain the Heart: Over time, this constant extra work can cause the right side of your heart to enlarge and weaken [4].

Validating the “Long Road” to Diagnosis

It is common for CTEPH patients to feel like they haven’t been heard for a long time. Because the symptoms—like shortness of breath and fatigue—are non-specific, they are often mistaken for asthma, poor fitness, or the “normal” recovery from a PE. On average, patients experience a diagnostic delay of 14 to 21 months, and in some cases, it can take over two years to get the correct diagnosis [5][6][7].

If you felt like something was wrong even when initial tests came back “normal,” you were likely right. Recognition of CTEPH requires a high index of suspicion and specialized imaging that is not always performed in standard follow-up care [8][9].

A New Era of Treatment

The most important thing to know today is that CTEPH is unique: unlike many other forms of pulmonary hypertension, it is potentially curable. Treatment has advanced significantly in the last decade, and modern care is managed by a multidisciplinary team (MDT) [4][10]. This team—usually consisting of specialized surgeons, cardiologists, and pulmonologists—works together to determine the best path forward for your specific anatomy [11].

The big-picture journey usually involves three main pillars of care:

  • Pulmonary Endarterectomy (PEA): This is a specialized surgery to physically remove the scar tissue from the lung arteries. It is the “gold standard” treatment because it can potentially cure the condition and return heart function to near-normal levels [12][3].
  • Balloon Pulmonary Angioplasty (BPA): For patients whose scar tissue is too deep in the lungs for surgery, doctors can use tiny balloons to open the vessels [13].
  • Medical Therapy: Specialized medications (such as riociguat) can help relax the blood vessels and improve blood flow, often used in combination with other treatments [14][15].

Your journey now shifts from “searching for answers” to “evaluating options.” While the diagnosis is life-changing, the current medical consensus emphasizes that with expert care, patients can see dramatic improvements in their exercise capacity and quality of life [16][17].

Common questions in this guide

Why did it take so long to get a CTEPH diagnosis?
The symptoms of CTEPH, such as shortness of breath and fatigue, are non-specific and often mistaken for asthma or poor fitness. It requires specialized imaging and a high index of suspicion to confirm the diagnosis, which frequently leads to diagnostic delays of a year or more.
How does a blood clot turn into CTEPH?
In a small percentage of people, a pulmonary embolism does not dissolve naturally. Over time, the soft clot transforms into firm, fibrous scar tissue that permanently narrows or blocks the lung arteries, forcing the heart to work much harder.
Is CTEPH curable?
Yes, unlike many other forms of pulmonary hypertension, CTEPH is potentially curable. The gold standard treatment is a specialized surgery called pulmonary endarterectomy (PEA), which physically removes the scar tissue from the lung arteries.
What if I am not a candidate for PEA surgery?
If the scar tissue is too deep in the lungs for surgery, doctors can use balloon pulmonary angioplasty (BPA) to open the vessels with tiny balloons. Specialized medications, such as riociguat, can also be prescribed to help relax the blood vessels and improve blood flow.
What kind of doctors should treat my CTEPH?
Your condition should be managed by a multidisciplinary team (MDT). This team typically includes specialized surgeons, cardiologists, and pulmonologists who work together to determine the most effective treatment path for your specific anatomy.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Has my case been reviewed by a multidisciplinary team (MDT) that includes a specialized surgeon, a cardiologist, and a pulmonologist?
  2. 2.What specific evidence in my imaging shows that my clots have turned into scar tissue?
  3. 3.Am I considered a candidate for pulmonary endarterectomy (PEA), and if not, what are the specific reasons why?
  4. 4.How much experience does this center have in performing both PEA and balloon pulmonary angioplasty (BPA)?
  5. 5.What is the current plan to manage my heart strain while we finalize my long-term treatment strategy?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (17)
  1. 1

    Systematic pulmonary embolism follow-up: why we should all do it!

    Howard LS, Price LC

    The European respiratory journal 2024; (63(3)) doi:10.1183/13993003.00253-2024.

    PMID: 38485183
  2. 2

    Evaluation of the Incidence of Chronic Thromboembolic Pulmonary Hypertension 1 Year After First Episode of Acute Pulmonary Embolism: A Cohort Study.

    Rashidi F, Parvizi R, Bilejani E, et al.

    Lung 2020; (198(1)):59-64 doi:10.1007/s00408-019-00315-3.

    PMID: 31894412
  3. 3

    Clinical Updates on the Diagnosis and Management of Chronic Thromboembolic Pulmonary Hypertension.

    Ruan W, Yap JJ, Quah KK, et al.

    Annals of the Academy of Medicine, Singapore 2020; (49(5)):320-330.

    PMID: 32582909
  4. 4

    Chronic thromboembolic pulmonary disease.

    Kim NH, D'Armini AM, Delcroix M, et al.

    The European respiratory journal 2024; (64(4)) doi:10.1183/13993003.01294-2024.

    PMID: 39209473
  5. 5

    Healthcare utilization in chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.

    Ende-Verhaar YM, van den Hout WB, Bogaard HJ, et al.

    Journal of thrombosis and haemostasis : JTH 2018; (16(11)):2168-2174 doi:10.1111/jth.14266.

    PMID: 30099844
  6. 6

    A model for estimating the health economic impact of earlier diagnosis of chronic thromboembolic pulmonary hypertension.

    Boon GJAM, van den Hout WB, Barco S, et al.

    ERJ open research 2021; (7(3)) doi:10.1183/23120541.00719-2020.

    PMID: 34853780
  7. 7

    [Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry].

    Valieva ZS, Martynyuk TV, Nakonechnikov SN, Chazova IE

    Terapevticheskii arkhiv 2021; (93(9)):1058-1065 doi:10.26442/00403660.2021.09.201037.

    PMID: 36286865
  8. 8

    Chronic thromboembolic pulmonary hypertension: anticoagulation and beyond.

    Martin KA, Cuttica MJ

    Hematology. American Society of Hematology. Education Program 2021; (2021(1)):478-484 doi:10.1182/hematology.2021000282.

    PMID: 34889396
  9. 9

    Monitoring for Pulmonary Hypertension Following Pulmonary Embolism: The INFORM Study.

    Tapson VF, Platt DM, Xia F, et al.

    The American journal of medicine 2016; (129(9)):978-985.e2.

    PMID: 27046247
  10. 10

    [Expert consensus on approaches to risk stratification and choice of therapy in patients with inoperable and residual chronic thromboembolic pulmonary hypertension].

    Chazova IE, Martynyuk TV, Avdeev SN, et al.

    Terapevticheskii arkhiv 2025; (97(3)):279-288 doi:10.26442/00403660.2025.03.203237.

    PMID: 40327623
  11. 11

    Balloon Pulmonary Angioplasty for the Treatment of Chronic Thromboembolic Pulmonary Hypertension.

    Carlozzi LN, Lin CH, Steinberg ZL

    Methodist DeBakey cardiovascular journal 2024; (20(3)):57-64 doi:10.14797/mdcvj.1347.

    PMID: 38765209
  12. 12

    Residual pulmonary hypertension after pulmonary endarterectomy: A meta-analysis.

    Hsieh WC, Jansa P, Huang WC, et al.

    The Journal of thoracic and cardiovascular surgery 2018; (156(3)):1275-1287 doi:10.1016/j.jtcvs.2018.04.110.

    PMID: 29903403
  13. 13

    Technical Considerations for Performing Safe and Effective Balloon Pulmonary Angioplasty in Patients with Chronic Thromboembolic Pulmonary Hypertension.

    Hosokawa K, Yamasaki Y, Abe K

    Cardiac electrophysiology clinics 2025; (17(3)):355-368 doi:10.1016/j.ccep.2024.12.005.

    PMID: 40835304
  14. 14

    Operability assessment in CTEPH: Lessons from the CHEST-1 study.

    Jenkins DP, Biederman A, D'Armini AM, et al.

    The Journal of thoracic and cardiovascular surgery 2016; (152(3)):669-674.e3.

    PMID: 27083940
  15. 15

    Evolution of randomized, controlled studies of medical therapy in chronic thromboembolic pulmonary hypertension.

    Kim NH, Papamatheakis DG, Fernandes TM

    Pulmonary circulation 2021; (11(2)):20458940211007373 doi:10.1177/20458940211007373.

    PMID: 34104419
  16. 16

    EmPHasis-10 Health-Related Quality of Life and Exercise Capacity in Chronic Thromboembolic Pulmonary Hypertension After Balloon Angioplasty.

    Sakamoto H, Goda A, Tobita K, et al.

    Journal of the American Heart Association 2022; (11(17)):e026400 doi:10.1161/JAHA.122.026400.

    PMID: 36056723
  17. 17

    The Initial Experience of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in Latvia.

    Krigere A, Kalejs VR, Kaulins R, et al.

    Medicina (Kaunas, Lithuania) 2024; (60(4)) doi:10.3390/medicina60040568.

    PMID: 38674214

This page provides educational information about navigating a CTEPH diagnosis and exploring treatment options. Always consult your pulmonologist or multidisciplinary medical team for personalized medical advice regarding your specific condition.

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