scholarly journals Current strategies for managing CTEPH: results of the worldwide prospective CTEPH Registry

2021 ◽  
pp. 00850-2020
Author(s):  
Stefan Guth ◽  
Andrea M. D'Armini ◽  
Marion Delcroix ◽  
Kazuhiko Nakayama ◽  
Elie Fadel ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Patient Characteristics ◽  
Pulmonary Angiography ◽  
Right Heart Catheterisation ◽  
First Choice ◽  
Lung Scan ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of ◽  
And Gender

BackgroundPulmonary endarterectomy (PEA), pulmonary arterial hypertension (PAH) therapy, and balloon pulmonary angioplasty (BPA) are currently accepted therapies for chronic thromboembolic pulmonary hypertension (CTEPH). This international CTEPH registry identifies clinical characteristics of patients, diagnostic algorithms, and treatment decisions in a global context.Methods1010 newly diagnosed consecutive patients were included into the registry between February 2015 and September 2016. Diagnosis was confirmed by right heart catheterisation, ventilation-perfusion lung scan, computerised pulmonary angiography, and/or invasive pulmonary angiography after at least 3 months on anticoagulation.ResultsOverall, 649 patients (64.3%) were considered for PEA, 193 (19.1%) for BPA, 20 (2.0%) for both PEA and BPA and 148 (14.7%) for PAH therapy only. Reasons for PEA inoperability: technical inaccessibility (n=235), co-morbidities (n=63), and patient refusal (n=44). In Europe, America and other countries (AAO), 72% of patients were deemed suitable for PEA whereas in Japan, 70% of patients were offered BPA as first choice. Gender was evenly balanced, except in Japan where 75% of patients were female. A history of acute pulmonary embolism was reported for 65.6% of patients. At least one PAH therapy was initiated in 35.8% of patients (26.2% of PEA candidates, 54.5% of BPA candidates, and 54.1% of those not eligible for either PEA or BPA). At the time of analysis, 39 patients (3.9%) had died of PH-related causes (3.5% after PEA and 1.8% after BPA).ConclusionsThe registry revealed noticeable differences in patient characteristics (rates of pulmonary embolism and gender) and therapeutic approaches in Japan compared with Europe and AAO.

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

2020 ◽  
Vol 49 (5) ◽  
pp. 320-330
Author(s):  
Wen Ruan ◽  
Jonathan JL Yap ◽  
Kevin KH Quah ◽  
Foong Koon Cheah ◽  
Ghee Chee Phuah ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Residual Disease ◽  
Treatment Options ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Pulmonary Endarterectomy ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Cardinal Symptom

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a known sequela after acute pulmonary embolism (PE). It is a debilitating disease, and potentially fatal if left untreated. This review provides a clinically relevant overview of the disease and discusses the usefulness and limitations of the various investigational and treatment options. Methods: A PubMed search on articles relevant to pulmonary embolism, pulmonary hypertension, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, and balloon pulmonary angioplasty were performed. A total of 68 articles were found to be relevant and were reviewed. Results: CTEPH occurs as a result of non-resolution of thrombotic material, with subsequent fibrosis and scarring of the pulmonary arteries. Risk factors have been identified, but the underlying mechanisms have yet to be fully elucidated. The cardinal symptom of CTEPH is dyspnoea on exertion, but the diagnosis is often challenging due to lack of awareness. The ventilation/perfusion scan is recommended for screening for CTEPH, with other modalities (eg. dual energy computed tomography pulmonary angiography) also being utilised in expert centres. Conventional pulmonary angiography with right heart catherisation is important in the final diagnosis of CTEPH. Conclusion: Operability assessment by a multidisciplinary team is crucial for the management of CTEPH, as pulmonary endarterectomy (PEA) remains the guideline recommended treatment and has the best chance of cure. For inoperable patients or those with residual disease post-PEA, medical therapy or balloon pulmonary angioplasty are potential treatment options. Keywords: Balloon pulmonary angioplasty, Chronic thromboembolic pulmonary hypertension, Pulmonary embolism, Pulmonary endarterectomy, Pulmonary hypertension

Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension

2005 ◽  
Vol 93 (03) ◽  
pp. 512-516 ◽  
Author(s):  
Diana Bonderman ◽  
Johannes Jakowitsch ◽  
Christopher Adlbrecht ◽  
Michael Schemper ◽  
Paul Kyrle ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Odds Ratio ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Medical Conditions ◽  
Symptomatic Pulmonary Embolism ◽  
Increased Risk ◽  
Lung Scan ◽  
Thromboembolic Pulmonary Hypertension ◽  
Inflammatory Bowel

SummaryChronic thromboembolic pulmonary hypertension (CTEPH) is characterized by organized thromboemboli that obstruct the pulmonary vascular bed. Although CTEPH is a serious complication of acute symptomatic pulmonary embolism in 4% of cases, signs, symptoms and classical risk factors for venous thromboembolism are lacking. The aim of the present study was to identify medical conditions conferring an increased risk of CTEPH. We performed a case-control-study comparing 109 consecutive CTEPH patients to 187 patients with acute pulmonary embolism that was confirmed by a high probability lung scan. Splenectomy (odds ratio=13, 95% CI 2.7–127), ventriculoatrial (VA-) shunt for the treatment of hydrocephalus (odds ratio=13, 95% CI 2.5–129) and chronic inflammatory disorders, such as osteomyelitis and inflammatory bowel disease (IBD, odds ratio=67, 95% CI 7.9–8832) were associated with an increased risk of CTEPH.

Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

2014 ◽  
Vol 112 (09) ◽  
pp. 598-605 ◽  
Author(s):  
Laurent Guérin ◽  
Francis Couturaud ◽  
Florence Parent ◽  
Marie-Pierre Revel ◽  
Florence Gillaizeau ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Systolic Pulmonary Artery Pressure ◽  
Prospective Multicentre Study ◽  
Right Heart Catheterisation ◽  
Venous Thromboembolic Events ◽  
Thromboembolic Pulmonary Hypertension ◽  
Acute Pe

SummaryChronic thromboembolic pulmonary hypertension (CTEPH) has been estimated to occur in 0.1–0.5% of patients who survive a pulmonary embolism (PE), but more recent prospective studies suggest that its incidence may be much higher. The absence of initial haemodynamic evaluation at the time of PE should explain this discrepancy. We performed a prospective multicentre study including patients with PE in order to assess the prevalence and to describe risk factors of CTEPH. Follow-up every year included an evaluation of dyspnea and echocardiography using a predefined algorithm. In case of suspected CTEPH, the diagnosis was confirmed using right heart catheterisation (RHC). Signs of CTEPH were searched on the multidetector computed tomography (CT) and echocardiography performed at the time of PE. Of the 146 patients analysed, eight patients (5.4%) had suspected CTEPH during a median follow-up of 26 months. CTEPH was confirmed using RHC in seven cases (4.8%; 95%CI, 2.3 – 9.6) and ruled-out in one. Patients with CTEPH were older, had more frequently previous venous thromboembolic events and more proximal PE than those without CTEPH. At the time of PE diagnosis, patients with CTEPH had a higher systolic pulmonary artery pressure and at least two signs of CTEPH on the initial CT. After acute PE, the prevalence of CTEPH appears high. However, initial echocardiography and CT data at the time of the index PE suggest that a majority of patients with CTEPH had previously unknown pulmonary hypertension, indicating that a first clinical presentation of CTEPH may mimic acute PE.

scholarly journals Chronic thromboembolic pulmonary hypertension in young woman with history of caesarian section

2015 ◽  
Vol 23 (4) ◽  
pp. 232-8 ◽  
Author(s):  
Nitia A. Asbarinsyah ◽  
Rarsari S. Soerarso ◽  
Nani Hersunarti ◽  
Bambang B. Siswanto
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Condition ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Caesarian Section ◽  
Pulmonary Angiography ◽  
Diagnostic Tools ◽  
Vein Thrombosis ◽  
Pulmonary Vasodilator ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of subgroups of pulmonary hypertension. This is a serious medical condition that severely under diagnosed. CTEPH is commonly underdiagnosed due to non specific symptoms and lack of diagnostic tools. The aim of this presentation is to discuss the etiology, risk factors, diagnosis and management of CTEPH. A 36-year-old woman presented with easily fatigue and dyspneu on effort since two years ago. The symptom occured about three months after she gave birth with caesarian section due to preeclampsia. Further history taking, physical examination, electrocardiography (ECG) and echocardiography were highly suggestive of pulmonary hypertension. No deep vein thrombosis (DVT) was found on vascular femoral sonography. It was found after the lung perfusion scintigraphy performed that she actually had CTEPH. This patient was categorized as inoperable because CT pulmonary angiography showed no thrombus. The patient got pulmonary vasodilator and oral anticoagulant for lifelong.

scholarly journals Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature

2017 ◽  
Vol 49 (2) ◽  
pp. 1601792 ◽  
Author(s):  
Yvonne M. Ende-Verhaar ◽  
Suzanne C. Cannegieter ◽  
Anton Vonk Noordegraaf ◽  
Marion Delcroix ◽  
Piotr Pruszczyk ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Meta Analysis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Population Level ◽  
Right Heart ◽  
Random Effects Models ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is relevant for management decisions but is currently unknown.We performed a meta-analysis of studies including consecutive PE patients followed for CTEPH. Study cohorts were predefined as “all comers”, “survivors” or “survivors without major comorbidities”. CTEPH incidences were calculated using random effects models.We selected 16 studies totalling 4047 PE patients who were mostly followed up for >2-years. In 1186 all comers (two studies), the pooled CTEPH incidence was 0.56% (95% CI 0.1–1.0). In 999 survivors (four studies) CTEPH incidence was 3.2% (95% CI 2.0–4.4). In 1775 survivors without major comorbidities (nine studies), CTEPH incidence was 2.8% (95% CI 1.5–4.1). Both recurrent venous thromboembolism and unprovoked PE were significantly associated with a higher risk of CTEPH, with odds ratios of 3.2 (95% CI 1.7–5.9) and 4.1 (95% CI 2.1–8.2) respectively. The pooled CTEPH incidence in 12 studies that did not use right heart catheterisation as the diagnostic standard was 6.3% (95% CI 4.1–8.4).The 0.56% incidence in the all-comer group probably provides the best reflection of the incidence of CTEPH after PE on the population level. The ∼3% incidences in the survivor categories may be more relevant for daily clinical practice. Studies that assessed CTEPH diagnosis by tests other than right heart catheterisation provide overestimated CTEPH incidences.

scholarly journals Recurrent chronic thromboembolic disease despite optimal anticoagulation in setting of recent COVID-19 infection

2021 ◽  
Vol 14 (1) ◽  
pp. e238733 ◽  
Author(s):  
Vittorio Romeo Terrigno ◽  
Daniel Anthony Ricketti ◽  
Pranav Patel ◽  
Satyajeet Roy
Keyword(s):  
Pulmonary Embolism ◽  
Acute Pulmonary Embolism ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
International Normalized Ratio ◽  
Thromboembolic Disease ◽  
Anticoagulation Treatment ◽  
Therapeutic Anticoagulation ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of ◽  
Chronic Thromboembolic Disease

We present a case of a 38-year-old man with a history of chronic thromboembolic pulmonary hypertension on therapeutic anticoagulation and recent hospitalisation for COVID-19 disease who was hospitalised for recurrent acute pulmonary embolism despite therapeutic anticoagulation with warfarin (International Normalized Ratio (INR) of 3.0). Our case highlights the hypercoagulable state associated with COVID-19 disease and the absence of standardised approaches to anticoagulation treatment for this population.

P4671A well-defined subgroup of chronic thromboembolic pulmonary hypertension patients are positive for antiphospholipid antibodies

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Denas ◽  
C Chen ◽  
Y Du ◽  
X Jiang ◽  
Y Zhou ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Risk Factor ◽  
Antiphospholipid Antibodies ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Thromboembolic Pulmonary Hypertension

Abstract Introduction Recurrent thromboembolism is a major risk factor for chronic thromboembolic pulmonary hypertension (CTEPH). Antiphospholipid antibodies (aPL) are a recognized risk factor for recurrent thromboembolism, however, their contribution to the development of CTEPH is currently unknown. Purpose The aim of this study was to evaluate the prevalence of antiphospholipid autoantibodies in a large cohort of consecutive patients with CTEPH. Methods Starting May 2013 to December 2018, 297 CTEPHpatients were consecutively enrolled. Diagnosis was confirmed with standardized right heart catheterization, 2D or/and 3D pulmonary angiography in all patients.All patients were also screened for thrombophilia including lupus anticoagulant IgG/IgM anticardiolipin antibodies and IgG/IgM aβ2GPI antibodies. Clinical and demographic characteristics, laboratory profile and hemodynamic data were compared between patients with aPL positive and aPL negative laboratory profiles. Results Overall, 297 consecutive patients with CTEPH were assessed for aPLs. Of these, 23 patients (7.7%) resulted positive for laboratory tests exploring aPL. Among patients with positive aPL, profile 17 (74%) were positive for all three test exploring aPL (LA+, aCL+, aB2GPI+). When compared to CTEPH patients without aPL test positivity, those positive for aPL were significantly younger (30.0 vs 55.6 years, p<0.0001), had a positive history of pulmonary embolism (95.6% vs 65.7%, p=0.003), deep venous thrombosis (78.3% vs 41.9%, p=0.0008) and were more frequently affected by other autoimmune diseases (43.5% vs 2.9%, p<0.0001). Moreover, positive aPL patients showed better hemodynamics on right heart catheterization, had more proximal lesions, and had more frequently level I and II surgical classification (82.6% vs 40.5%, p=0.0002) on pulmonary angiography. Conclusions These findings suggest that young patients with pulmonary embolism should be assessed for aPLs to guide anticoagulation therapy and prevent recurrences. Additionally, younger age, more proximal lesions and less severe hemodynamic profiles make majority patients with APS good candidates for PEA surgery.

scholarly journals Prediction of chronic thromboembolic pulmonary hypertension with standardised evaluation of initial computed tomography pulmonary angiography performed for suspected acute pulmonary embolism

2021 ◽  
Author(s):  
Gudula J. A. M. Boon ◽  
Yvonne M. Ende-Verhaar ◽  
Ludo F. M. Beenen ◽  
Johan Coolen ◽  
Marion Delcroix ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
High Risk ◽  
Acute Pulmonary Embolism ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Expert Judgment ◽  
Pulmonary Angiography ◽  
Computed Tomography Pulmonary Angiography ◽  
Thromboembolic Pulmonary Hypertension

Abstract Objectives Closer reading of computed tomography pulmonary angiography (CTPA) scans of patients presenting with acute pulmonary embolism (PE) may identify those at high risk of developing chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to validate the predictive value of six radiological predictors that were previously proposed. Methods Three hundred forty-one patients with acute PE were prospectively followed for development of CTEPH in six European hospitals. Index CTPAs were analysed post hoc by expert chest radiologists blinded to the final diagnosis. The accuracy of the predictors using a predefined threshold for ‘high risk’ (≥ 3 predictors) and the expert overall judgment on the presence of CTEPH were assessed. Results CTEPH was confirmed in nine patients (2.6%) during 2-year follow-up. Any sign of chronic thrombi was already present in 74/341 patients (22%) on the index CTPA, which was associated with CTEPH (OR 7.8, 95%CI 1.9–32); 37 patients (11%) had ≥ 3 of 6 radiological predictors, of whom 4 (11%) were diagnosed with CTEPH (sensitivity 44%, 95%CI 14–79; specificity 90%, 95%CI 86–93). Expert judgment raised suspicion of CTEPH in 27 patients, which was confirmed in 8 (30%; sensitivity 89%, 95%CI 52–100; specificity 94%, 95%CI 91–97). Conclusions The presence of ≥ 3 of 6 predefined radiological predictors was highly specific for a future CTEPH diagnosis, comparable to overall expert judgment, while the latter was associated with higher sensitivity. Dedicated CTPA reading for signs of CTEPH may therefore help in early detection of CTEPH after PE, although in our cohort this strategy would not have detected all cases. Key Points • Three expert chest radiologists re-assessed CTPA scans performed at the moment of acute pulmonary embolism diagnosis and observed a high prevalence of chronic thrombi and signs of pulmonary hypertension. • On these index scans, the presence of ≥ 3 of 6 predefined radiological predictors was highly specific for a future diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), comparable to overall expert judgment. • Dedicated CTPA reading for signs of CTEPH may help in early detection of CTEPH after acute pulmonary embolism.

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