Chronic Thromboembolic Pulmonary Hypertension

Table of Contents

1. What the Condition Is
2. How an Old Clot Becomes Scar Tissue
3. How Often It Follows a Pulmonary Embolism
4. Who Appears to Be at Higher Risk
5. The Symptoms People Report
6. How It Is Found
7. Why It Is Missed
8. Pulmonary Endarterectomy Results
9. Balloon Pulmonary Angioplasty Results
10. Riociguat Trials
11. Macitentan Trials
12. Anticoagulation Findings
13. Survival Across Treatments and Decades
14. What the 2022 ESC/ERS Guideline States
15. What Is Not Known
16. Key Research Papers
17. Connections
18. Featured Videos

What the Condition Is

The right side of the heart sends blood through the lung arteries to pick up oxygen. Pulmonary hypertension means the pressure in those arteries is too high. Under the 2022 European guideline definition, that is a mean pressure above 20 millimeters of mercury (mmHg), measured at rest with a catheter (Simeone 2024).

In chronic thromboembolic pulmonary hypertension the raised pressure has a specific cause. A 2021 European Respiratory Society statement describes it as a rare complication of acute pulmonary embolism, "either symptomatic or not". The large lung arteries are blocked by fibrous material, a second disease develops in vessels smaller than 500 micrometers, resistance to blood flow rises, and the result is progressive right heart failure (Delcroix 2021). The strain on the right heart is the same process described on the Cor Pulmonale page, although the cause there is lung disease.

It is separate from pulmonary arterial hypertension, in which the small arteries narrow without a clot blockage. The two are treated differently, which is why telling them apart matters.

How an Old Clot Becomes Scar Tissue

A fresh clot in the lung is normally broken down by the body's own clot-dissolving system, helped by blood thinners that stop it from growing. In this condition, that clearing fails. A 2017 review describes the disease as beginning with persistent obstruction of large and middle-sized lung arteries by organized clots, meaning clots that have been converted into solid tissue rather than removed (Simonneau 2017).

Researchers have proposed several reasons why the clot stays:

• Abnormal clot breakdown. Failure to resolve may be related to abnormal fibrinolysis, the process that dissolves clots, or to underlying blood or autoimmune disorders (Simonneau 2017).
• Altered clot structure and repair. A 2024 task force paper lists abnormalities in the structure and function of fibrinogen (the protein that forms a clot's mesh), inflammatory clotting, and failure of new blood vessels to grow into the clot. It also notes that some clotting may take place in the artery itself (Kim 2024).

The blockage is only part of the story. Small-vessel disease can develop in the obstructed areas, possibly triggered by the unresolved material. It can also develop downstream of the blockages in arteries that remain open, possibly because of excess blood flow from high-pressure bronchial and systemic arteries (Simonneau 2017). The degree of this small-vessel disease has a substantial effect on how severe the condition is and how people do after surgery (Simonneau 2017). This two-part picture explains why treatment has several arms: surgery and balloons act on the blockages, and medicines act mainly on the small vessels.

The European Respiratory Society statement is plain that the central step is still unexplained: the mechanism that transforms red clots into fibrotic remnants "has not yet been elucidated" (Delcroix 2021).

How Often It Follows a Pulmonary Embolism

The figure depends heavily on who is counted and how the diagnosis was confirmed.

• A prospective follow-up study. Among people with a first pulmonary embolism and no earlier clots, the cumulative rate of symptomatic disease was 1.0% at six months, 3.1% at one year and 3.8% at two years. No cases occurred after two years among those followed for longer (Pengo 2004).
• A 2017 meta-analysis of 16 studies and 4,047 patients. The pooled rate was 0.56% among all patients with pulmonary embolism, 3.2% among survivors, and 2.8% among survivors without major other illnesses. The authors judged 0.56% the best reflection of the rate at population level, and about 3% more relevant to daily clinical practice (Ende-Verhaar 2017).
• The effect of the test used. In 12 studies that did not use right heart catheterization as the standard, the pooled rate was 6.3%. The meta-analysis authors concluded that studies relying on other tests overestimate how common the condition is (Ende-Verhaar 2017).

The relationship also runs the other way. Not everyone with the condition remembers or was ever told of a clot. In a 2011 international registry of 679 newly diagnosed patients, 74.8% reported a history of acute pulmonary embolism (Pepke-Zaba 2011). In a later worldwide registry of 1,010 patients the figure was 65.6% (Guth 2021). So roughly a quarter to a third of patients had no recorded pulmonary embolism at all.

Who Appears to Be at Higher Risk

These are associations from observational studies. They do not show that any one factor causes the disease.

• Features of the clot. In that 2004 follow-up study, risk rose with a previous pulmonary embolism (odds ratio 19.0), a pulmonary embolism with no identifiable trigger (odds ratio 5.70), a larger perfusion defect (2.22 per decile drop in perfusion), and younger age (1.79 per decade) (Pengo 2004). The 2017 meta-analysis found higher risk with recurrent clots in the veins (odds ratio 3.2) and with unprovoked pulmonary embolism (odds ratio 4.1) (Ende-Verhaar 2017).
• Other conditions. A European database study of 687 patients linked the disease with infected pacemakers and ventriculo-atrial shunts, splenectomy, earlier and recurrent clots in the veins, blood groups other than O, and lupus anticoagulant or antiphospholipid antibodies. It also identified thyroid replacement therapy and a history of cancer as new associations. Several of the confidence intervals were very wide (Bonderman 2009).
• Clotting disorders. A thrombophilic disorder was recorded in 31.9% of the 2011 registry patients, and a removed spleen in 3.4% (Pepke-Zaba 2011). Related pages cover antiphospholipid syndrome and factor V Leiden.

The worldwide registry found sex evenly balanced except in Japan, where 75% of patients were female (Guth 2021). Patients in Japan also differed from others in how often a pulmonary embolism had been recorded, and the reasons for these regional differences are not settled by the registry.

The Symptoms People Report

A 2024 review of diagnosis calls the presentation "often nonspecific", with symptoms that can be subtle and easily mistaken for other conditions (Simeone 2024). The review describes the following:

• Breathlessness on exertion. This is the most frequently reported symptom, and it limits exercise and ordinary activities.
• Leg and foot swelling. This reflects fluid retention and a weakening right ventricle.
• Lightheadedness, dizziness or fainting during exertion. The review ties these to reduced heart output and links them to more severe disease.

Hospitals treat sudden breathlessness, chest pain and collapse as urgent, because they can signal a new pulmonary embolism. The warning signs are described on the Pulmonary Embolism and Deep Vein Thrombosis pages.

How It Is Found

Diagnosis usually proceeds in steps, and the diagnosis in the worldwide registry was made only after at least 3 months on blood thinners (Guth 2021). That waiting period separates clots that have failed to clear from clots still dissolving.

1. Heart ultrasound. A 2024 review reports that the European pulmonary embolism guideline advises an echocardiogram as a first evaluation for people with persistent breathlessness, functional limits or risk factors (Simeone 2024).
2. Ventilation-perfusion scan. This nuclear scan compares where air reaches in the lungs with where blood reaches. The condition is suspected when the scan shows areas that receive air but not blood, known as mismatched perfusion defects (Delcroix 2021). The acute pulmonary embolism guidelines support it as the first-line test when breathlessness is present and an ultrasound suggests a high probability of pulmonary hypertension (Kim 2024). Conditions affecting the smallest lung vessels generally give normal or patchy scans rather than segment-shaped defects, with one exception, pulmonary veno-occlusive disease (Simeone 2024).
3. Right heart catheterization. A thin tube passed through a vein into the heart and lung artery measures the pressures directly. This, together with imaging of the vessels, confirms the diagnosis (Delcroix 2021). Measuring the wedge pressure is needed to exclude pulmonary hypertension coming from the left side of the heart (Kim 2024).
4. Vessel imaging. Computed tomography pulmonary angiography or invasive pulmonary angiography maps the blockages, which feeds into the decision on treatment (Guth 2021; Kim 2024).

The scan comparison that shaped this order comes from a single British center. Among 227 patients referred for pulmonary hypertension between 2000 and 2005, the ventilation-perfusion scan had a sensitivity of 96%–97.4% and a specificity of 90%–95%. Computed tomography pulmonary angiography had a sensitivity of 51% and a specificity of 99%. So the angiogram missed about half of the true cases in that series (Tunariu 2007). That study used the scanners of its time, and it does not test newer machines.

A 2024 review also reports that testing for antiphospholipid syndrome is recommended, and that this guidance does not extend to wider panels for inherited clotting disorders (Simeone 2024).

Why It Is Missed

A 2024 task force paper states that accurate diagnosis "is often delayed" (Kim 2024). In the 2011 international registry, a median of 14.1 months had passed between first symptoms and diagnosis (Pepke-Zaba 2011). The published findings point to several reasons:

• The symptoms are common ones. Breathlessness on exertion has many causes, and the disease's early signs are easily attributed to something else (Simeone 2024).
• Many patients have no clot on record. With roughly a quarter to a third of patients reporting no prior pulmonary embolism, there is often nothing in the history that points to the diagnosis (Pepke-Zaba 2011; Guth 2021).
• The common scan can look clear. In the British series, computed tomography pulmonary angiography was negative in 38 of 78 people who did have the disease, while the ventilation-perfusion scan was low probability in only 2 of them (Tunariu 2007).
• Routine checks are not universal. A 2024 review reports that routine screening for pulmonary hypertension after pulmonary embolism is not universally recommended. Evaluation is reserved for people with persistent symptoms (Simeone 2024).

Pulmonary Endarterectomy Results

Pulmonary endarterectomy is open surgery that removes the scar-like material from inside the lung arteries. It is a different operation from removing a fresh clot. A report from one large American center describes opening the breastbone, a heart-lung machine, deep cooling of the body, and periods when circulation is stopped completely so that the surgeon can see the small branches (Jamieson 2003). The authors of that report consider the operation curative.

Deaths around the operation.

• The same center reported 4.4% mortality (22 of its last 500 patients) in 2003 (Jamieson 2003). A later report from the center gave in-hospital mortality of 5.2% among 1,000 patients and 2.2% among the next 500, and no deaths in the last 260 consecutive patients having this operation alone (Madani 2012).
• Across the 2011 international registry, documented mortality was 4.7% (Pepke-Zaba 2011). High-volume results from specialist centers may not apply everywhere.

Changes in pressure and walking.

• In the most recent 500 patients in the American report, mean lung artery pressure fell from 45.5 to 26.0 mmHg (Madani 2012).
• A meta-analysis of 4,868 patients found average falls of about 21 mmHg in mean pressure and 561 dyn·s/cm⁵ in vascular resistance, along with a 96-meter gain in six-minute walking distance (Hsieh 2018).

Long-term survival. In the United Kingdom national cohort of 880 patients, survival was 86%, 84%, 79% and 72% at 1, 3, 5 and 10 years. In the more recent half of the cohort, it was 91% and 90% at 1 and 3 years. Most deaths after the period around surgery were not due to right ventricular failure (Cannon 2016).

Not everyone is left with normal pressure. The same meta-analysis found persistent pulmonary hypertension in 25% of patients after surgery (Hsieh 2018). In the United Kingdom cohort, a mean pressure of 38 mmHg or more, together with resistance of 425 dynes·s^-1·cm^-5 or more at reassessment, correlated with worse long-term survival. A mean pressure of 30 mmHg or more correlated with starting a lung-vessel drug afterward (Cannon 2016).

Who has it. In the worldwide registry, 649 of 1,010 patients (64.3%) were considered for this operation. Reasons people could not have it were technically unreachable disease (235 patients), other illnesses (63) and the patient declining (44) (Guth 2021). Operability varied widely by country: in the earlier registry the share having surgery ranged from 12.0% to 60.9% across countries (Pepke-Zaba 2011). In Europe, America and other countries outside Japan, 72% were judged suitable for surgery, while in Japan 70% were offered the balloon procedure as first choice (Guth 2021).

Balloon Pulmonary Angioplasty Results

In balloon pulmonary angioplasty, a catheter is passed through the blood vessels into the lung arteries, and a small balloon opens narrowed branches. The work is staged over several sessions. Patients in a Japanese randomized trial averaged 4.7 procedures (Kawakami 2022). By 2023, more than 1,700 treated patients had been reported in the literature (Lang 2023).

Registries.

• Japan, 308 patients and 1,408 procedures. Mean lung artery pressure fell from 43.2 to 24.3 mmHg after the final session and was 22.5 mmHg at follow-up. Survival was 96.8% at 1 and 2 years and 94.5% at 3 years. Complications occurred in 36.3% of procedures, including lung injury (17.8%), coughing up blood (14.0%) and artery perforation (2.9%). Twelve patients (3.9%) died during follow-up, 8 of them within 30 days of a procedure (Ogawa 2017).
• France, 184 patients and 1,006 sessions. Mean pressure fell by 26% and vascular resistance by 43%, and six-minute walking distance rose by a mean of 45 meters. Lung injury occurred in 9.1% of sessions, falling from 13.3% in the early period to 5.9% in the recent period. Three-year survival was 95.1% (Brenot 2019). The authors describe an "unavoidable learning curve" for the procedure.

Two randomized trials against riociguat in inoperable disease.

• RACE, France, 105 patients. At 26 weeks, vascular resistance had fallen to 39.9% of its starting level with the balloon procedure and to 66.7% with riociguat. Treatment-related serious adverse events occurred in 42% and 9% respectively, and lung injury in 35% of the balloon group. There were no treatment-related deaths. At 52 weeks, after patients with continuing problems could add the other treatment, the two sequences gave a similar reduction. Procedure-related serious events were less frequent in those given riociguat first (14% against 42%) (Jaïs 2022).
• MR BPA, Japan, 61 patients. At 12 months, mean lung artery pressure had improved by 16.3 mmHg with the balloon procedure and by 7.0 mmHg with riociguat. Coughing up blood or lung bleeding affected 44% of the balloon group and 4% of the riociguat group. Complications occurred in 12% of the 147 procedures (Kawakami 2022).

Both trials were open-label, so patients and doctors knew which treatment was given. A 2025 systematic review of five studies covered 184 patients on both treatments, 94 on the balloon procedure alone and 79 on riociguat alone. Its authors concluded that combination appeared more effective than either alone. Even so, the fall in mean pressure was greatest in the balloon-only group, at about 17.6 mmHg against 12.2 mmHg with both and 5.4 mmHg with riociguat alone (Araújo 2025). The review is small and combines trials with observational studies.

Riociguat Trials

Riociguat is a tablet that stimulates soluble guanylate cyclase, an enzyme involved in relaxing blood vessels. It was tested for people whose disease could not be operated on, or whose high pressure persisted or returned after surgery.

• CHEST-1. This double-blind trial randomized 261 patients to riociguat or placebo for 16 weeks. Six-minute walking distance rose by 39 meters with riociguat and fell by 6 meters with placebo, a difference of 46 meters. Vascular resistance fell by 226 dyn·s/cm⁵ with riociguat and rose by 23 with placebo. Right ventricular failure was the most common serious adverse event, at 3% in each group, and fainting occurred in 2% with riociguat and 3% with placebo (Ghofrani 2013).
• CHEST-2. In this open-label extension, 237 patients were given riociguat adjusted up to a maximum of 2.5 mg three times daily. At one year, walking distance was 51 meters above the original baseline among those observed, and functional class had improved, stabilized or worsened in 47%, 50% and 3%. There was no placebo comparison at this stage (Simonneau 2015).

Side effects listed in the 2025 systematic review were dizziness, headache, reflux, vomiting and nausea, reported in 15%–23% of patients (Araújo 2025). In the RACE trial, the most frequent treatment-related serious event with riociguat was severe low blood pressure with fainting, in 4% (Jaïs 2022). CHEST-1 ran for 16 weeks and its main measure was walking distance, so it does not show whether riociguat lengthens life.

Macitentan Trials

Macitentan blocks receptors for endothelin, a substance that narrows blood vessels. Its results in this condition are mixed.

• MERIT-1 (phase 2). This trial randomized 80 patients with inoperable disease, giving macitentan 10 mg once daily or placebo. At week 16, vascular resistance had fallen to 71.5% of its starting level with macitentan and to 87.6% with placebo. The most common adverse events on macitentan were swelling of the limbs (23%) and decreased hemoglobin (15%). The journal retracted the original 2017 version of this paper and republished it in 2024. The figures here come from the 2024 version (Ghofrani 2024).
• MERIT-2 (open-label extension). Of the 80 patients, 76 continued for a median of 45.5 months. Fourteen (18.4%) died, and none of the deaths were assessed as related to macitentan. With no control group, the extension cannot show how much of the course is due to the drug (Kim 2025).
• MACiTEPH (phase 3). This double-blind trial randomized 127 patients to a higher dose, macitentan 75 mg once daily, or placebo. Most (81.9%) were already on a pulmonary hypertension drug. It was stopped early for futility. The difference in change in six-minute walking distance at week 28 was 16.1 meters in the placebo group's favor (95% confidence limits -32.3 to 0.16), so the main goal was not met. Clinical worsening occurred in 6.3% on macitentan and 14.3% on placebo. More patients on macitentan stopped treatment because of an adverse event (21.9% against 7.9%) (Jenkins 2026).

The two controlled trials differ in dose, patient mix, background treatment and main measure, so they do not answer the same question. CHEST-1 and MERIT-1 were funded by the makers of the drugs tested.

Anticoagulation Findings

A 2024 task force paper describes lifelong therapeutic anticoagulation as recommended in this condition to prevent further clots (Kim 2024). The open question has been which type. Vitamin K antagonists such as warfarin have long been used, and direct oral anticoagulants are newer. The way warfarin works is shown in the vitamin K cycle animation.

• After surgery, United Kingdom. A retrospective study followed 794 patients on vitamin K antagonists and 206 on direct oral anticoagulants. Major bleeding was the same (0.67% against 0.68% per person-year). Recurrent clots in the veins were more frequent with direct oral anticoagulants (4.62% against 0.76% per person-year). Survival did not differ (Bunclark 2020).
• Japan, prospective registry. This study followed 481 patients on direct oral anticoagulants and 446 on warfarin. Three-year rates of the combined morbidity and mortality outcome were 4.2% and 5.9%, which were comparable. Three-year clinically relevant bleeding was lower with direct oral anticoagulants (2.4% against 6.4%), with a hazard ratio of 0.35 (Hosokawa 2023).
• Worldwide registry. No survival difference was seen between vitamin K antagonists and non-vitamin K oral anticoagulants (Delcroix 2024).
• 2023 meta-analysis of 2,969 patients. This combined one randomized trial and six observational studies. Direct oral anticoagulants were linked with lower mortality (relative risk 0.54) and a higher risk of recurrent pulmonary embolism (relative risk 3.80), with no significant difference in major bleeding (relative risk 0.59, confidence interval 0.34–1.02). Its authors caution that, because most studies were observational, biases and confounding factors may be at work (Ishisaka 2023).

In the observational studies, the drug each patient received was not assigned by chance, so the groups may have differed from the start. The findings pull in different directions. For people who also have antiphospholipid syndrome, the 2024 paper reports that vitamin K antagonists are particularly recommended (Kim 2024).

Survival Across Treatments and Decades

The largest modern picture comes from a worldwide registry of 1,009 newly diagnosed patients at 34 centers in 20 countries. Of these, 60% had surgery, 18% had the balloon procedure, and the rest had neither. Three-year survival was 94%, 92% and 71% in those three groups. Deaths during follow-up were 7%, 11% and 27% (Delcroix 2024).

That comparison needs care. Patients were not randomly assigned. Median age was 60 in the surgery group, 66 in the balloon group and 69 in the group with no mechanical treatment, so the groups started out different. Within the group having no procedure, use of pulmonary hypertension drugs and oxygen therapy, lower right atrial pressure and functional class were associated with lower mortality.

A retrospective study from Japan compared eras in 834 patients. The share having no specific treatment fell from 65% to 3%, and combined treatment rose from 0% to 58%. Five-year survival rose from 68% (1980–1999) to 85% (2000–2010) and 93% (2010–2023) (Kiko 2025). Better detection of milder disease could contribute to a rise like this, so the trend cannot be credited to any single treatment.

A 2024 task force paper summarizes the current position as 3-year survival above 90% with combined treatment. It also states that the best improvement in symptoms and survival comes from the two mechanical treatments (Kim 2024).

What the 2022 ESC/ERS Guideline States

The European Society of Cardiology and European Respiratory Society (ESC/ERS) issued joint pulmonary hypertension guidelines in 2022 (Humbert 2022). The positions below are given as described in three published papers that summarize the guideline: an ESC working group consensus statement (Lang 2023), a 2023 review (Banaszkiewicz 2023) and the 2024 task force paper (Kim 2024). Class I is the strongest grade, and class IIa means the weight of evidence favors considering a treatment.

• Team review. The guideline recommends review of all patients by a multidisciplinary team capable of offering every treatment. That team includes surgeons, balloon specialists, pulmonary hypertension doctors and chest radiologists trained in high-volume centers (Lang 2023; Kim 2024).
• Surgery. Pulmonary endarterectomy is the recommended first-line treatment for operable patients (Kim 2024; Lang 2023).
• Balloon procedure. The recommendation was upgraded from class IIb, level C in 2015 to class I, level B in 2022, for people technically inoperable or left with pulmonary hypertension after surgery (Lang 2023; Kim 2024). The guideline treats it as not replacing surgery (Banaszkiewicz 2023).
• Medicine before the balloon procedure. Class IIa. The guideline describes considering medical therapy first when vascular resistance is above 4 Wood units, though the task force paper notes the quality of the evidence is low (Kim 2024; Banaszkiewicz 2023).
• Blood thinners with antiphospholipid syndrome. Vitamin K antagonists are particularly recommended in this group (Kim 2024).
• Clot disease without high pressure. Class IIa, level C. The guideline describes considering either pulmonary endarterectomy or the balloon procedure for selected people with symptoms of chronic thromboembolic pulmonary disease but no pulmonary hypertension (Banaszkiewicz 2023; Lang 2023).
• Follow-up. Long-term follow-up is recommended, with right heart catheterization and imaging at 3–6 months after surgery or the balloon procedure (Kim 2024; Lang 2023).

Guideline grades record the panel's judgment of the evidence at the time, and later trials such as MACiTEPH were published after this guideline.

What Is Not Known

• Why some clots turn to scar. The mechanism has not yet been worked out (Delcroix 2021), and the molecular processes behind the small-vessel disease are not completely understood (Simonneau 2017).
• How to catch it early. Routine screening after pulmonary embolism is not universal (Simeone 2024), and rates change with the population and the test chosen (Ende-Verhaar 2017).
• The long-term record of the balloon procedure. Early prognosis looks similar to surgery, while longer-term prognosis remains unclear (Banaszkiewicz 2023). No trial reviewed here randomized patients between the balloon procedure and surgery.
• The best order and combination. The RACE authors state that more studies are needed on sequences combining one or two medicines with the balloon procedure (Jaïs 2022).
• Which blood thinner is best. The evidence is mostly observational, and bleeding and recurrence results differ between studies (Bunclark 2020; Hosokawa 2023; Ishisaka 2023).
• The role of macitentan. A positive phase 2 result on vascular resistance was followed by a phase 3 trial stopped for futility on walking distance, using a different dose and patient group (Ghofrani 2024; Jenkins 2026).
• Why regions differ so much. Registries show large differences by country in operability, in the choice of treatment, and in the sex of patients (Pepke-Zaba 2011; Guth 2021).

Decisions about medicines and procedures belong with a clinician, usually at a specialist center for this disease.

Key Research Papers

1. Pengo V, Lensing AW, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350(22):2257-64. PubMed PMID: 15163775
2. Ende-Verhaar YM, Cannegieter SC, Vonk Noordegraaf A, et al. Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature. Eur Respir J. 2017;49(2):1601792. PubMed PMID: 28232411
3. Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124(18):1973-81. PubMed PMID: 21969018
4. Guth S, D'Armini AM, Delcroix M, et al. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry. ERJ Open Res. 2021;7(3):00850-2020. PubMed PMID: 34409094
5. Delcroix M, Pepke-Zaba J, D'Armini AM, et al. Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy. Circulation. 2024;150(17):1354-1365. PubMed PMID: 39286890
6. Delcroix M, Torbicki A, Gopalan D, et al. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J. 2021;57(6):2002828. PubMed PMID: 33334946
7. Simonneau G, Torbicki A, Dorfmüller P, Kim N. The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017;26(143):160112. PubMed PMID: 28356405
8. Kim NH, D'Armini AM, Delcroix M, et al. Chronic thromboembolic pulmonary disease. Eur Respir J. 2024;64(4):2401294. PubMed PMID: 39209473
9. Bonderman D, Wilkens H, Wakounig S, et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33(2):325-31. PubMed PMID: 18799507
10. Tunariu N, Gibbs SJ, Win Z, et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007;48(5):680-4. PubMed PMID: 17475953
11. Simeone B, Maggio E, Schirone L, et al. Chronic thromboembolic pulmonary hypertension: the diagnostic assessment. Front Cardiovasc Med. 2024;11:1439402. PubMed PMID: 39309600
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13. Madani MM, Auger WR, Pretorius V, et al. Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2,700 patients. Ann Thorac Surg. 2012;94(1):97-103. PubMed PMID: 22626752
14. Cannon JE, Su L, Kiely DG, et al. Dynamic Risk Stratification of Patient Long-Term Outcome After Pulmonary Endarterectomy: Results From the United Kingdom National Cohort. Circulation. 2016;133(18):1761-71. PubMed PMID: 27052413
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18. Jaïs X, Brenot P, Bouvaist H, et al. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study. Lancet Respir Med. 2022;10(10):961-971. PubMed PMID: 35926542
19. Kawakami T, Matsubara H, Shinke T, et al. Balloon pulmonary angioplasty versus riociguat in inoperable chronic thromboembolic pulmonary hypertension (MR BPA): an open-label, randomised controlled trial. Lancet Respir Med. 2022;10(10):949-960. PubMed PMID: 35926544
20. Araújo P, Calé R, Pereira E, et al. Balloon pulmonary angioplasty and riociguat in the management of chronic thromboembolic pulmonary hypertension: a systematic review. Respir Med. 2025;244:108154. PubMed PMID: 40412446
21. Lang IM, Andreassen AK, Andersen A, et al. Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function. Eur Heart J. 2023;44(29):2659-2671. PubMed PMID: 37470202
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23. Ghofrani HA, D'Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369(4):319-29. PubMed PMID: 23883377
24. Simonneau G, D'Armini AM, Ghofrani HA, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015;45(5):1293-302. PubMed PMID: 25395036
25. Ghofrani HA, Simonneau G, D'Armini AM, et al. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med. 2024;12(4):e21-e30. PubMed PMID: 38548406
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PubMed Topic Searches

1. PubMed: chronic thromboembolic pulmonary hypertension
2. PubMed: pulmonary endarterectomy outcomes
3. PubMed: balloon pulmonary angioplasty
4. PubMed: direct oral anticoagulants in this condition

Connections

• Pulmonology
• Pulmonary Hypertension
• Pulmonary Embolism
• Pulmonary Arterial Hypertension
• Cor Pulmonale
• Deep Vein Thrombosis
• Antiphospholipid Syndrome
• Factor V Leiden
• Vitamin K, Clotting & How Warfarin Works

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