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 ERS Statement on Chronic Thromboembolic Pulmonary Hypertension

2020 ◽  
pp. 2002828
Author(s):  
Marion Delcroix ◽  
Adam Torbicki ◽  
Deepa Gopalan ◽  
Olivier Sitbon ◽  
Frederikus A. Klok ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Acute Pulmonary Embolism ◽  
Current Knowledge ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Treatment Modalities ◽  
Reference Network ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with a secondary microvasculopathy of vessels less than 500 µm, leads to increased pulmonary vascular resistance and progressive right heart failure. The mechanism responsible for the transformation of red clots into fibrotic material remnants has not yet been elucidated. In patients with pulmonary hypertension, the diagnosis is suspected when a ventilation/perfusion lung scan shows mismatched perfusion defects and confirmed by right heart catheterisation and vascular imaging. Today, in addition to lifelong anticoagulation, treatment modalities include surgery, angioplasty and medical treatment according to the localisation and characteristics of the lesions.This Statement outlines a review of the literature and current practice concerning diagnosis and management of CTEPH. It covers the definitions, diagnosis, epidemiology, follow up after acute pulmonary embolism, pathophysiology, treatment by pulmonary endarterectomy, balloon pulmonary angioplasty, drugs and their combination, rehabilitation and new lines of research in CTEPH.It represents the first collaboration of the European Respiratory Society (ERS), the International CTEPH Association (ICA) and the European Reference Network (ERN)-Lung in the pulmonary hypertension domain. The Statement summarises current knowledge but does not make formal recommendations for clinical practice.

Improvement after bariatric surgery in chronic thromboembolic pulmonary hypertension: a novel treatment target

2021 ◽  
Vol 14 (3) ◽  
pp. e228358
Author(s):  
Anika Vaidy ◽  
Paul Forfia ◽  
Jeremy Mazurek ◽  
Anjali Vaidya
Keyword(s):  
Bariatric Surgery ◽  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Walk Distance ◽  
Right Heart ◽  
Exertional Dyspnea ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

A 66-year-old man with a medical history significant for multiple pulmonary embolism and morbid obesity was evaluated for chronic thromboembolic pulmonary hypertension. Echocardiogram and right heart catheterisation were significant for severe pulmonary hypertension. Therefore, he was started on pulmonary hypertension medical therapy with riociguat and ambrisentan, in addition to anticoagulation. He experienced a dramatic clinical response to medical therapy. Despite haemodynamic improvement, the patient remained symptomatic with significant fatigue, exertional dyspnea and poor functional status as highlighted by a 6 min walk distance of only 128 m. Patient was referred for bariatric surgery with a gastric sleeve, after which he successfully lost 95 lbs in 6 months. Postoperative right heart catheterisation demonstrated normal pulmonary  vascular resistance and cardiac output. His echocardiogram revealed normal right ventricular size and function. His 6 min walk distance also nearly quadrupled from 128 to 512 m, consistent with WHO Functional Class I.

scholarly journals Multicentre observational screening survey for the detection of CTEPH following pulmonary embolism

2018 ◽  
Vol 51 (4) ◽  
pp. 1702505 ◽  
Author(s):  
Nicolas Coquoz ◽  
Daniel Weilenmann ◽  
Daiana Stolz ◽  
Vladimir Popov ◽  
Andrea Azzola ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Observational Study ◽  
Acute Pulmonary Embolism ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Second Step ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension ◽  
Screening Survey

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe complication of pulmonary embolism. Its incidence following pulmonary embolism is debated. Active screening for CTEPH in patients with acute pulmonary embolism is yet to be recommended.This prospective, multicentre, observational study (Multicentre Observational Screening Survey for the Detection of Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Following Pulmonary Embolism (INPUT on PE); ISRCTN61417303) included patients with acute pulmonary embolism from 11 centres in Switzerland from March 2009 to November 2016. Screening for possible CTEPH was performed at 6, 12 and 24 months using a stepwise algorithm that included a dyspnoea phone-based survey, transthoracic echocardiography, right heart catheterisation and radiological confirmation of CTEPH.Out of 1699 patients with pulmonary embolism, 508 patients were assessed for CTEPH screening over 2 years. CTEPH incidence following pulmonary embolism was 3.7 per 1000 patient-years, with a 2-year cumulative incidence of 0.79%. The Swiss pulmonary hypertension registry consulted in December 2016 did not report additional CTEPH cases in these patients. The survey yielded 100% sensitivity and 81.6% specificity. The second step echocardiography in newly dyspnoeic patients showed a negative predictive value of 100%.CTEPH is a rare but treatable disease. A simple and sensitive way for CTEPH screening in patients with acute pulmonary embolism is recommended.

scholarly journals Chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 53 (1) ◽  
pp. 1801915 ◽  
Author(s):  
Nick H. Kim ◽  
Marion Delcroix ◽  
Xavier Jais ◽  
Michael M. Madani ◽  
Hiromi Matsubara ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Anticoagulation Therapy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Treatment Decision ◽  
Right Heart Failure ◽  
Vascular Involvement ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Pulmonary Angiogram ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.

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.

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 Thrombotic Lesion of the Pulmonary Vessels in Patients with Pulmonary Embolism

2020 ◽  
Vol 16 (6) ◽  
pp. 1002-1008
Author(s):  
A. A. Klimenko ◽  
N. A. Demidova ◽  
N. A. Shostak ◽  
M. O. Anischenko
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Disease ◽  
Combined Therapy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart ◽  
Pulmonary Thrombosis ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Right

After suffering pulmonary embolism (PE), doctors are confronted with various consequences of the disease, from asymptomatic residual pulmonary thrombosis to the formation of chronic thromboembolic pulmonary hypertension (CTEPH). There is also a subgroup of patients who have undergone pulmonary embolism, who experience shortness of breath during physical exertion, absent before pulmonary embolism, or shortened dyspnea preceding PE, combined with residual thrombosis of pulmonary artery (PA) and normal average pressure in PA at rest during catheterization of the right heart (CRH). This condition is defined as chronic thromboembolic pulmonary disease or post thromboembolic syndrome. Pathogenetic aspects of this condition are not fully investigated. It is important to predict the development of postembolic syndrome and to develop algorithms for the diagnosis, treatment and rehabilitation of patients with symptoms and residual pulmonary thrombosis. In case of the development of pulmonary vasculopathy in some patients who have undergone pulmonary embolism, a severe life-threatening condition forms - CTEPH, characterized by an increase in pressure in the pulmonary artery, right heart failure due to the presence of organized blood clots that have entered the pulmonary vascular bed during PE. The volume of thrombotic masses does not always correlate with clinical symptoms, which indicates the importance of microvascular remodeling. If CTEPH is suspected, a diagnostic algorithm is required, including ventilation-perfusion scintigraphy, CT angiopulmonography and catheterization of the right heart. Treating a patient with CTEPH is a difficult task fora doctor. The timely referral of the patient to the center where they are involved in treatment, including surgery and CTEPH is extremely important. Timely performed thrombendarterectomy in some cases allows to completely cure the patient. In the case of inoperable CTEPH or residual pulmonary hypertension after thrombendarterectomy, balloon angioplasty of the PA is used as well as drug treatment with specific drugs that reduce the pressure in the PA (riociguat), endothelin receptor antagonists (bosentan, macitentan), prostanoids (inhalant illoprost) phosphodiesterase-5 inhibitor and combined therapy. In this article we considered some consequences directly related to PE: asymptomatic residual pulmonary thrombosis, chronic thromboembolic pulmonary disease, chronic thromboembolic pulmonary hypertension.

scholarly journals Higher Incidence of Chronic Thromboembolic Pulmonary Hypertension After Acute Pulmonary Embolism in Asians Than in Europeans: A Meta-Analysis

2021 ◽  
Vol 8 ◽  
Author(s):  
Wenyi Pang ◽  
Zhu Zhang ◽  
Zenghui Wang ◽  
Kaiyuan Zhen ◽  
Meng Zhang ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Cohort Studies ◽  
Sample Size ◽  
Acute Pulmonary Embolism ◽  
Meta Analysis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Venous Thromboembolic Events ◽  
Thromboembolic Pulmonary Hypertension ◽  
Acute Pe

Aim: To summarize the incidence of right heart catheter diagnosed chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary embolism (PE) in a meta-analysis.Methods: Cohort studies reporting the incidence of CTEPH after acute PE were identified via search of Medline, Embase, China National Knowledge Infrastructure and WanFang databases.Results: Twenty-two cohort studies with 5,834 acute PE patients were included. Pooled results showed that the overall incidence of CTEPH was 2.82% (95% CI: 2.11–3.53%). Subgroup analyses showed higher incidence of CTEPH in Asians than Europeans (5.08 vs. 1.96%, p = 0.01), in retrospective cohorts than prospective cohorts (4.75 vs. 2.47%, p = 0.02), and in studies with smaller sample size than those with larger sample size (4.57 vs. 1.71%, p &lt; 0.001). Stratified analyses showed previous venous thromboembolic events and unprovoked PE were both significantly associated with increased risk of CTEPH (OR = 2.57 and 2.71, respectively; both p &lt; 0.01).Conclusions: The incidence of CTEPH after acute PE is ~3% and the incidence is higher in Asians than Europeans. Efforts should be made for the early diagnosis and treatment of CTEPH in PE patients, particularly for high-risk population.

scholarly journals EXPRESS: Chronic thromboembolic pulmonary hypertension following pulmonary embolism with a right ventricular thrombus: A report of two cases

2021 ◽  
pp. 204589402110136
Author(s):  
Tailong Zhang ◽  
Weitao Liang ◽  
Longrong Bian ◽  
Zhong Wu
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Imaging Features ◽  
Pulmonary Endarterectomy ◽  
Right Heart ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Right ◽  
Cardiac Masses

Right heart thrombus (RHT) accompanied by chronic thromboembolic pulmonary hypertension (CTEPH) is a rare entity. RHT may develop in the peripheral veins or in situ within the right heart chambers. The diagnosis of RHT is challenging, since its symptoms are typically non-specific and its imaging features resemble those of cardiac masses. Here, we report two cases of RHT with CTEPH that presented as right ventricular masses initially. Both patients underwent simultaneous pulmonary endarterectomy (PEA) and resection of the ventricular thrombi. Thus, when mass-like features are confirmed by imaging, RHT should be suspected in patients with CTEPH, and simultaneous RHT resection is required along with PEA.

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