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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Thoracic Surgery

Treatment Strategy and Your Care Team for CTEPH

At a Glance

CTEPH treatment requires a specialized multidisciplinary team. Pulmonary Endarterectomy (PEA) surgery is the gold standard and potential cure. For those who cannot undergo surgery, Balloon Pulmonary Angioplasty (BPA) or medications are effective options. All patients need lifelong blood thinners.

Because CTEPH is complex, your treatment isn’t decided by just one doctor. Instead, it requires a Multidisciplinary Team (MDT)—a group of specialized surgeons, cardiologists, and pulmonologists—to review your scans and determine which of the three main treatment “pillars” is right for you [1][2].

Determining “Operability”: The Jamieson Classification

The first and most important question the team asks is: “Can we physically remove these scars?” To answer this, they use the Jamieson Classification, which ranks how reachable the blockages are [3][4]:

  • Type 1 & 2: The scars are in the main or large branches of the pulmonary arteries. These are the most “operable” and have the best long-term outcomes after surgery [4][5].
  • Type 3: The scars are deeper, in the segmental branches. These can still be operated on, but the surgery is more complex, and patients might need additional treatments later [4].
  • Type 4: The disease is primarily in the tiny, microscopic vessels. Surgery is generally not possible for this type [3].

Pillar 1: Pulmonary Endarterectomy (PEA)

PEA is the gold standard treatment because it is the only one that is potentially curative [6]. However, patients must be prepared for the reality that PEA is a massive, highly complex open-heart surgery.

During the operation, the patient is placed on a heart-lung bypass machine, and their body temperature is cooled significantly. The surgeon then temporarily stops blood circulation entirely (Deep Hypothermic Circulatory Arrest) in brief intervals so they can see inside the tiny lung arteries without blood obscuring the view [6][7]. The surgeon then meticulously peels the scar tissue away from the artery walls. Because of the magnitude of this surgery, it carries significant risks, including reperfusion pulmonary edema (fluid flooding the lungs as blood flow returns) and a challenging, multi-week recovery. However, for eligible patients treated at expert centers, the payoff is immense: a chance to return to near-normal heart function [8][9].

Pillar 2: Balloon Pulmonary Angioplasty (BPA)

If your scars are too deep for surgery (Type 3 or 4), or if you are not a candidate for open-heart surgery due to other health issues, BPA is a highly effective alternative [10]. A cardiologist uses a catheter to thread tiny balloons into the lung arteries, inflating them to push the scars aside and restore blood flow.

While BPA is minimally invasive and does not require opening the chest, it is not without risk. Potential complications include vascular injury (tearing a blood vessel) or coughing up blood (hemoptysis) [11]. It usually takes several separate sessions to clear the lungs, but BPA has revolutionized CTEPH care, showing excellent survival rates—over 90% at five years in many cases [11][12].

Pillar 3: Medical Therapy

Medications are used to relax the blood vessels and improve blood flow. Riociguat (Adempas) is the primary medication specifically approved for CTEPH [13]. However, it is not the only option. Doctors frequently use other pulmonary arterial hypertension (PAH) medications—such as PDE-5 inhibitors (like sildenafil), ERAs, or prostacyclins—off-label or in combination depending on the patient’s specific needs [14][15]. Medications are primarily used for patients who cannot have surgery, or those who still have high pressure after their procedures.

The “Lifeline”: Anticoagulation

Regardless of which treatment you receive, you will need lifelong anticoagulation (blood thinners) to prevent new clots from forming [16]. Crucial Rule: NEVER stop or pause your blood thinners for dental work or minor procedures without consulting your CTEPH specialist.

There is an ongoing medical discussion about which type of blood thinner is best. While DOACs (like Eliquis or Xarelto) are more convenient, many expert centers still prefer Warfarin (Coumadin), especially in the months leading up to surgery [17]. Some evidence suggests that patients on DOACs might have “fresher” or more persistent clots at the time of surgery compared to those on Warfarin, which can make the PEA surgery more difficult [18][19]. Always follow your surgical team’s specific protocol.

Common questions in this guide

What is the Jamieson Classification for CTEPH?
The Jamieson Classification helps doctors rank how reachable the scar tissue is within your pulmonary arteries. Types 1 and 2 are in larger vessels and are highly operable, while Type 3 is deeper and more complex. Type 4 involves tiny vessels where surgery is generally not possible.
What is pulmonary endarterectomy (PEA) surgery?
PEA is an open-heart surgery and the gold standard treatment for CTEPH because it is the only potentially curative option. During the procedure, a surgeon meticulously peels scar tissue out of the lung arteries while you are on a heart-lung bypass machine.
What are my options if I cannot have PEA surgery?
If your blockages are too deep or you cannot have open-heart surgery, Balloon Pulmonary Angioplasty (BPA) is a highly effective, minimally invasive alternative. A cardiologist uses tiny balloons threaded through a catheter to push scars aside and restore blood flow.
When is medication used to treat CTEPH?
Medications are primarily used to relax blood vessels and improve blood flow. They are usually prescribed for patients who cannot undergo surgery, or for those who still have high blood pressure in their lungs after their procedures.
Will I need to take blood thinners after CTEPH surgery?
Yes, you will need lifelong blood thinners (anticoagulation) regardless of which treatment you receive. This is a crucial step to prevent new blood clots from forming in your lungs, and you should never stop taking them without consulting your specialist.
Which blood thinner is best for CTEPH patients?
While newer blood thinners like DOACs are convenient, many expert CTEPH centers prefer Warfarin, especially leading up to surgery. Always follow your specific surgical team's protocol, as some evidence suggests DOACs can make PEA surgery more difficult.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Which Jamieson Type (1-4) is my disease, and how does that affect my chance of a full cure through surgery?
  2. 2.Has my case been reviewed by a specialized multidisciplinary team (MDT) specifically for operability?
  3. 3.If I am currently on a DOAC (like Eliquis or Xarelto), do you recommend switching to Warfarin before my surgery or procedure?
  4. 4.Am I a candidate for 'multimodal' therapy, such as a combination of medication and BPA?
  5. 5.What are this center's specific survival and complication rates for PEA and BPA?

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

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This page explains CTEPH treatment options, including surgical and medical therapies, for educational purposes. Always consult your multidisciplinary care team to determine the safest treatment plan for your specific condition.

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