Incidence Of Chronic Thromboembolic Pulmonary Hypertension Research Articles

Diagnosis of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: data from a practice-based longitudinal cohort

BackgroundA large prospective multicenter cohort study with systematic follow-up recently reported a 2.3% 2-year cumulative incidence of chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary embolism (PE). ObjectivesThe present investigation aimed to determine the reported prevalence and incidence of CTEPH diagnosis after acute PE in real-world practice over a 12-year period. MethodsThis study was based on nationwide ambulatory billing claims and drug prescription data of all residents with public health insurance in Germany from 2010 to 2021. ResultsA total of 573 972 patients with acute PE (median age, 71 years; 57.4% women) were identified between 2010 and 2021. Prevalence of CTEPH among patients with history of PE increased during the period from 0.4% in 2010 to 0.9% in 2021. CTEPH was diagnosed in 2556 patients after acute PE, with most (17.6%) diagnoses reported within the first 3 months after the index PE event. The cumulative incidence rate after 3 months (first quarter) was calculated at 0.08% and after the first 2 years (eighth quarter) at 0.36%; it was 0.75% over the entire (90-month) follow-up period. Patients with CTEPH diagnosis during follow-up more often had right ventricular dysfunction at the index acute PE (14.9% vs 8.3%; P < .001). ConclusionThe low CTEPH incidence rate after acute PE in the present analysis suggests low awareness of CTEPH. It further suggests a lack of systematic follow-up protocols for acute PE survivors in the real world. Improved implementation of existing recommendations on follow-up strategies after PE is warranted.

Chronic Thromboembolic Pulmonary Hypertension after Pulmonary Embolism in SARS-CoV-2

Introduction: Chronic thromboembolic pulmonary disease (CTEPD) consists of persistent pulmonary vascular obstruction on imaging and involves long-term functional limitations, with or without chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to evaluate the incidence and risk factors of both persistent pulmonary vascular defects and CTEPH after hospitalization in patients with COVID-19 and PE during a 2-year follow-up. Methods: A prospective observational study was carried out in a tertiary hospital center. Patients were hospitalized between March 2020 and December 2021 with a diagnosis of PE during SARS-CoV-2 infection. Patients received anticoagulant treatment for at least 3 months and were followed up for 2 years. Between the third and fourth months after discharge, all patients were evaluated for the presence of residual thrombotic defects by CTPA and/or perfusion pulmonary scintigraphy. Clinical findings, lung function tests with DLCO, exercise capacity, and echocardiograms were also assessed. Results: Of the 133 patients included, 18% had persistent thrombotic defects on lung imaging at follow-up. The incidence of CTEPD was 0.75% at 2 years of follow-up. Patients with persistent defects were significantly older, had a higher prevalence of systemic arterial hypertension, higher D-dimer and NT-proBNP levels, and more severe PE at diagnosis. Furthermore, there was a higher prevalence of right ventricular dysfunction on echocardiogram at diagnosis of PE (25.0% vs. 2.7%, p = 0.006). This was the only variable independently related to persistent defects in multivariate analyses (OR: 8.13 [95% CI: 1.82–36.32], p = 0.006). Conclusion: The persistence of thrombotic defects after PE is a common finding after SARS-CoV-2 infection, affecting 18% of the population. However, the incidence of CTEPH appears to be lower (0.75%) in COVID-19-related PE compared to that previously observed in PE unrelated to COVID-19.

Long‐term sequelae following acute pulmonary embolism: A nationwide follow‐up study regarding the incidence of CTEPH, dyspnea, echocardiographic and V/Q scan abnormalities

AbstractWe aimed to follow a nationwide cohort of patients with pulmonary embolism (PE) without any exclusions to generate information regarding long‐term symptoms, investigational findings and to determine the prevalence of chronic thromboembolic pulmonary hypertension (CTEPH). We hypothesized that this approach would yield generalizable estimates of CTEPH prevalence and incidence. All individuals diagnosed with acute PE in Sweden in 2005 were identified using the National Patient Register. In 2007, survivors were asked to complete a questionnaire regarding current symptoms. Those with dyspnea were referred for further examinations with laboratory tests, electrocardiogram (ECG), and a ventilation/perfusion scan (V/Q scan). If CTEPH was suspected, a referral to the nearest pulmonary arterial hypertension‐center was recommended. Of 5793 unique individuals with PE diagnosis in 2005, 3510 were alive at the beginning of 2007. Altogether 53% reported dyspnea at some degree whereof a large proportion had V/Q scans indicating mismatched defects. Further investigation revealed 6 cases of CTEPH and in parallel 18 cases were diagnosed outside this study. The overall prevalence of CTEPH was 0.4% (95% confidence interval [CI]: 0.2%–0.6%) and 0.7% (95% CI: 0.4%–1.0%) among the survivors. The cumulative incidence of CTEPH in the group of patients who underwent a V/Q scan was 1.1% (95% CI: 0.2%–2.0%). There was a high mortality following an acute PE, a high proportion of persistent dyspnea among survivors, whereof several had pathological findings on V/Q scans and echocardiography. Only a minority developed CTEPH, indicating that CTEPH is the tip of the iceberg of post‐PE disturbances.

Feasibility of a screening algorithm for chronic thromboembolic pulmonary hypertension: The OSIRIS study

IntroductionChronic thromboembolic pulmonary hypertension (CTEPH) is a long-term sequel to pulmonary embolism (PE) whose incidence varies according to different published studies. We have carried out this study to determine its incidence within 2 years after index pulmonary embolism and to study limitations to an early diagnosis. Material and methodsOSIRIS is a multicentre, longitudinal cohort study. Patients were followed for 3, 6, 12, and 24 months after pulmonary embolism using a structured three-step algorithm. A physician-centered questionnaire at least one positive response in a screening proceeded to the second step, transthoracic echocardiography. The third step consisted of ventilation/perfusion lung scintigraphy and right heart catheterisation. A transthoracic echocardiography was performed in patients without positive response in the screening questionnaire after 2 years. CTEPH diagnosis required haemodynamic confirmation by right heart catheterisation and mismatched perfusion defects on lung scintigraphy. ResultsA total of 1191 patients were enrolled in 18 Spanish hospitals. Cumulative CTEPH incidence after 2-years PE was: 2.49 % (95 % CI: 1.68–3.56) and the incidence rate of CTEPH was 1.1 cases per 1000 person-months (95 % CI: 0.725; 1.60). The CTEPH algorithm presented a lack of adherence of 29 %; patient and physician preferences posed barriers to the triage algorithm The screening questionnaire, in patients who completed the follow-up, shows a specificity of 91.3 % (89.0–93.2 %) and negative predictive value of 99.4 % (98.4–99.8 %).. ConclusionsOSIRIS provides practiced clinical based data on the chronic thromboembolic pulmonary hypertension incidence and identified barriers to the implementation of a 3-step triage algorithm for its detection. Clinical trial registrationclinicaltrials.gov identifier: NCT03134898.

Frequency and characterization of CTEPH and CTEPD according to the mPAP threshold > 20 mm Hg: Retrospective analysis from data of a prospective PE aftercare program

BackgroundThe influence of the new pulmonary hypertension (PH) definition on the incidence of chronic thromboembolic PH (CTEPH) is unclear. The incidence of chronic thromboembolic pulmonary disease without PH (CTEPD) is unknown. ObjectivesTo determine the frequency of CTEPH and CTEPD using the new mPAP cut-off >20 mmHg for PH in patients who have suffered an incidence of pulmonary embolism (PE) and were recruited into an aftercare program. MethodsIn a prospective two-year observational study based on telephone calls, echocardiography and cardiopulmonary exercise tests, patients with findings suspicious for PH received an invasive work-up. Data from right heart catheterization were used to identify patients with or without CTEPH/CTEPD. ResultsTwo years after acute PE (n = 400) we found an incidence of 5.25% for CTEPH (n = 21) and 5.75% for CTEPD (n = 23) according to the new mPAP threshold >20 mmHg. Five of 21 patients with CTEPH and 13 of 23 patients with CTEPD showed no signs of PH in echocardiography. CTEPH and CTEPD subjects showed a reduced VO₂ peak and work rate in cardiopulmonary exercise testing (CPET). The capillary end-tidal CO2 gradient was comparably elevated in CTEPH and CTEPD, but it was normal in the Non-CTEPD-Non-PH group. According to the PH definition provided by the former guidelines, only 17 (4.25%) patients have been diagnosed with CTEPH and 27 individuals (6.75%) were classified having CTEPD. ConclusionsUsing mPAP >20 mmHg for diagnosis of CTEPH leads to an increase of 23.5% of CTEPH diagnosis. CPET may help to detect CTEPD and CTEPH.

Keep minds opening for chronic thromboembolic pulmonary hypertension: More data, less clarity

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequelae of pulmonary embolism (PE), resulting from persistent obstruction of pulmonary arteries with non‐resolving thromboemboli and consecutive pulmonary small‐vessel remodeling.1,2 From the landmark research by Pengo et al.3 reporting that the cumulative incidence of CTEPH after acute episode of PE was 3.8% (95% confidence interval [CI] 1.1–6.5) at 2 years, subsequent studies reported a notable wide range of incidences, ranging from 0.1% to 11.8%,4,5 which keep the rationale of routine screening for CTEPH after an episode of PE in intense debate.

Dyspnea and perfusion defects (V/Q scan) are common after an acute pulmonary embolus whereas chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequele

Abstract Introduction Acute pulmonary embolism (PE), a common disease with substantial morbidity and mortality. Many previous studies have focused on the short-term consequences after an acute PE, but less attention has been directed to the long-term effects of a PE. The most feared complication is chronic thromboembolic pulmonary hypertension (CTEPH), with reported incidence rates ranging from 0.6 to 4.8%. Even though CTEPH is a rare complication after PE, dyspnea and functional impairment are common complaints, and a syndrome named PPES (post-PE-syndrome) has been suggested. The aim of this study was to follow a large national-wide cohort of post-PE-patients and to gather information regarding long-term symptoms and to determine the prevalence of CTEPH. Patients and methods We identified all patients diagnosed with acute PE in 2005 (ICD-10-SE: I26.0 and I26.9) that were registered in the Swedish National Patient Register (NPR), which has nearly 100% coverage. In 2007, all surviving individuals were invited to participate by letter. They were asked to complete a questionnaire regarding symptoms and risk factors for long-term sequele including CTEPH. Individuals who reported dyspnea or had known risk factors for CTEPH were invited to a follow-up with blood tests (NT-pro-BNP) and pulmonary scintigraphy. If any perfusion defects were detected, an echocardiography was performed. If signs of pulmonary hypertension, a referral was done to the regional PAH-center. Results In 2005, 5793 unique patients were admitted with a PE diagnosis in Sweden. Of those, 3510 were still alive in 2007, and 3226 individuals were contacted and asked to complete the questionnaire. Altogether 2105 (65%) individuals returned the questionnaire, and 53% reported dyspnea and 15% were asymptomatic but had risk factors for CTEPH. Altogether 944 individuals were referred for further follow-up including a recommendation to perform a V/Q-scan. Altogether 530 V/Q-scans were performed and 44% showed perfusion defects, whereof 85% had an echocardiography. Twenty-seven % of them registered a systolic right ventricular pressure of at least 30 mmHg. Referrals resulted in 6 cases with proved CTEPH. In parallel, 10 patients with CTEPH diagnosed before 2005 were detected, and 8 patients were diagnosed after 2005 and outside the study.Altogether 24 patients with CTEPH were thus identified and the cumulative incidence of CTEPH ranged between 0.7 and 1.2%. Conclusion Dyspnea is common after an acute PE and over 40% of them have perfusion defects on a V/Q scan, suggesting the presence of a post PE-syndrome (PPES). The incidence of CTEPH is however low. More research is needed to better define PPES. Funding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Actelion PharamceuticalsSwedish Heart and Lung Foundation

Imaging of the right ventricle in predicting the development of chronic thromboembolic pulmonary hypertension (CTEPH)

There is increasing evidence in the literature emphasizing the importance of right ventricular (RV) imaging in the prognosis of pulmonary hypertension. We aimed to investigate the predictive role of RV dysfunction parameters assessed by echocardiography (ECHO) and thorax computed tomography (CT) in developing CTEPH. We included prospectively patients diagnosed with pulmonary embolism (PE). All patients underwent ECHO and CT within 24 hours after admission. We repeated CT and ECHO after six months and one year to assess the incidence of CTEPH and the predictive role of RV dysfunction factors in the development of CTEPH. We included twenty-two patients (seven male) with a mean age of 53.9±17.9 years; CTEPH developed in two patients during the follow-up. Baseline PO2 levels were significantly lower in patients with CTEPH (61.5±11.4 vs 77.8±25.2, p&lt;0.05). The baseline RV diameter, RV EF, and systolic PAP levels evaluated by ECHO differed significantly in two patients who developed CTEPH. Two patients that developed CTEPH had the lowest RVS (-10.3% and -11.7%). This study claims that hypoxemia, decreased RV EF, RVS, increased systolic PAP values in ECHO, and increased RV/LV ratio evaluated in thorax CT indicate the severity of RV dysfunction in acute PE and may predict CTEPH development.

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation on screening for chronic thromboembolic pulmonary hypertension patients after acute pulmonary embolism: Update

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism (APE). Both pharmacological and invasive treatments for CTEPH are available in Poland, and awareness of the disease among physicians is growing. It has been suggested that the COVID-19 pandemic may increase the incidence of CTEPH and facilitate disease detection during more advanced stages of the illness. Thus, the Polish Cardiac Society's Working Group on Pulmonary Circulation, in cooperation with independent experts in this field, launched the updated statement on the algorithm to guide a CTEPH diagnosis in patients with previous APE. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months of effective anticoagulation, particularly when specific risk factors are present. Echocardiography is the main screening tool for CTEPH. A diagnostic workup of patients with significant clinical suspicion of CTEPH and right ventricular overload evident on echocardiography should be performed in reference centers. Pulmonary scintigraphy is a safe and highly sensitive screening test for CTEPH. Computed tomography pulmonary angiography with precise detection of thromboembolic residues in the pulmonary circulation is important for the planning of a pulmonary thromboendarterectomy. Right heart catheterization definitively confirms the presence of pulmonary hypertension and direct pulmonary angiography allows for the identification of lesions suitable for thromboendarterectomy or balloon pulmonary angioplasty. In this document, we propose a diagnostic algorithm for patients with suspected CTEPH. With an individualized and sequential diagnostic strategy, each patient can be provided with suitable and tailored therapy provided by a dedicated CTEPH Heart Team.

Incidence of Chronic Thrombo-embolic Pulmonary Hypertension Following Acute Pulmonary Thrombo-embolism: An Indian Perspective

Chronic thrombo-embolic pulmonary hypertension (CTEPH) remains a severe disabling disease causing a significant amount of mortality and morbidity worldwide. The incidence and severity of this condition is quite obscure. The initial inciting event, the reason of progression, the natural history of the disease and the predictors of adverse outcomes are not yet adequately clarified. From the Indian subcontinent, data regarding this disease is limited. But with the advent of the multi-detector computed tomography, the understanding of this disease is gradually improving. As most of the available data suggests, acute pulmonary embolism (PE) as the main initial trigger leading to CTEPH, we prospectively analysed all patients being admitted in our hospital with acute PE and followed them over a period of one-and-a-half years to determine the incidence of CTEPH in this group. This is just an attempt to increase the awareness about the disease pattern and determine the rate of progression, risk factors of poor outcome, so that early detection and prompt treatment can benefit the patient care.

Chronic thromboembolic pulmonary hypertension and impairment after pulmonary embolism: the FOCUS study.

AimsTo systematically assess late outcomes of acute pulmonary embolism (PE) and to investigate the clinical implications of post-PE impairment (PPEI) fulfilling prospectively defined criteria.Methods and resultsA prospective multicentre observational cohort study was conducted in 17 large-volume centres across Germany. Adult consecutive patients with confirmed acute symptomatic PE were followed with a standardized assessment plan and pre-defined visits at 3, 12, and 24 months. The co-primary outcomes were (i) diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), and (ii) PPEI, a combination of persistent or worsening clinical, functional, biochemical, and imaging parameters during follow-up. A total of 1017 patients (45% women, median age 64 years) were included in the primary analysis. They were followed for a median duration of 732 days after PE diagnosis. The CTEPH was diagnosed in 16 (1.6%) patients, after a median of 129 days; the estimated 2-year cumulative incidence was 2.3% (1.2–4.4%). Overall, 880 patients were evaluable for PPEI; the 2-year cumulative incidence was 16.0% (95% confidence interval 12.8–20.8%). The PPEI helped to identify 15 of the 16 patients diagnosed with CTEPH during follow-up (hazard ratio for CTEPH vs. no CTEPH 393; 95% confidence interval 73–2119). Patients with PPEI had a higher risk of re-hospitalization and death as well as worse quality of life compared with those without PPEI.ConclusionIn this prospective study, the cumulative 2-year incidence of CTEPH was 2.3%, but PPEI diagnosed by standardized criteria was frequent. Our findings support systematic follow-up of patients after acute PE and may help to optimize guideline recommendations and algorithms for post-PE care.

Chronic thromboembolic pulmonary hypertension: diagnosis, operability assessment and patient selection for pulmonary endarterectomy

Healthcare providers outside pulmonary hypertension (PH) centers having misinformation or insufficient education, and a general lack of treatment awareness contribute to a massive underdiagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), diagnostic delay and refusal of surgery by patients. Together with the subjective operability assessment, this leads to too few patients undergoing pulmonary endarterectomy (PEA); even though this surgery results in improved survival and exercise capacity. Acute pulmonary embolism (PE) survivors should undergo a CTEPH screening strategy. Patients screened positive and those with CTEPH symptoms (with or without history of PE), should undergo transthoracic echocardiography (TTE) to determine the probability of PH. High PH probability patients should undergo a ventilation/perfusion (V/Q) scan. A negative scan rules out CTEPH. Patients with a positive V/Q scan, but also patients with findings suggestive for CTEPH on computed tomography pulmonary angiography (CTPA) to diagnose acute PE, should be referred to a CTEPH center. Further diagnostic work-up currently consists of catheter based pulmonary angiography, CTPA and right heart catheterization. However, new imaging technologies might replace them in the near future, with one single imaging tool to screen, diagnose and assess operability as the ultimate goal. Operability assessment should be performed by a multidisciplinary CTEPH team. PEA surgery should be organized in a single center per country or for each forty to fifty million inhabitants in order to offer the highest level of expertise. Informing patients about PEA should preferably be done by the treating surgeon. Based on the estimated incidence of CTEPH and with a better education of patients and healthcare providers, despite the advent of new interventional and medical therapies for CTEPH, the number of PEA surgeries performed should still have the potential to grow significantly.

Epidemiology of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic.

This study investigated the epidemiology and survival outcomes of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic, wherein pulmonary endarterectomy (PEA) surgery was the only targeted treatment option until 2015. This study included all consecutive adults newly diagnosed with CTEPH in the Czech Republic between 2003 and 2016. Incidence/prevalence rates were calculated using general population data extracted from the Institute of Health Information and Statistics of the Czech Republic. Kaplan–Meier estimates of survival from diagnosis until 2018 were calculated. Of a total of 453 patients observed, 236 (52.1%) underwent PEA (median time from diagnosis to PEA: 2.9 months) and 71 (34.1%) had residual pulmonary hypertension (PH) post‐PEA. CTEPH incidence rate (95% confidence interval [CI]) between 2006 and 2016 was 4.47 (4.05; 4.91) patients per million (ppm) per year, and the prevalence (95% CI) was 37.43 (33.46; 41.73) ppm in 2016. The rate of CTEPH‐related hospitalizations (95% CI) per 100 person‐years was 24.4 (22.1; 26.9) for operated patients and 34.2 (30.9; 37.7) for not‐operated patients. Median overall survival (95% CI) for all patients from CTEPH diagnosis was 11.2 (9.4; not reached) years. Five‐year survival probability (95% CI) was 95.3% (89.9; 97.9) for operated patients without residual PH, 86.3% (75.3; 92.7) for operated patients with residual PH and 61.2% (54.0; 67.6) for not‐operated patients. This study reported epidemiological estimates of CTEPH in the Czech Republic consistent with estimates from other national systematic registries; and indicates an unmet medical need in not‐operated patients and operated patients with residual PH.

Incidence and predictors of chronic thromboembolic pulmonary hypertension following acute pulmonary embolism: An echocardiography guided approach

There is significant variability in the worldwide epidemiology of chronic thromboembolic pulmonary hypertension (CTEPH). We thereby aim to determine the incidence and predictors of CTEPH, following an episode of acute pulmonary embolism (PE), using non-invasive modalities. Patients with acute PE were prospectively followed-up and after receiving at least 3 months of effective anticoagulation, persistently symptomatic patients with echocardiographic evidence of persistent pulmonary hypertension, were investigated further for CTEPH. Incidence of CTEPH was 8.19%. Delayed presentation, higher pulmonary artery pressures at presentation and discharge, and greater thrombotic burden were significant predictors for the development of CTEPH following acute PE.

Incidence of chronic thromboembolic pulmonary hypertension in Taiwan

The diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is complex, and the modality of treatment is surgery and targeted medication. Patients with CTEPH could have a poor prognosis if their diagnosis or treatment is delayed. The incidence of CTEPH and its clinical features are largely unknown in Taiwan, even among other Asian populations. In this study, we aimed to investigate the geodemographics of CTEPH in Taiwan and describe the practical management and treatment outcomes in patients with CTEPH. This study retrospectively enrolled patients in the Taiwan cohort - Registry of CTEPH. The study was conducted over 2 years inclusive of follow-up. The enrolment criteria depended on the current global guideline. From January 2018 to March 2020, 107 CTEPH patients enrolled in the Taiwan registry. All patients received right heart catheterisation examinations. The overall median age was 61.4±16.5 years, and the cohort was dominated by female patients (75/107). Risk factors included pulmonary embolism (81.3%), deep vein thrombosis (22.4%), and previous major surgery (20.6%). Twenty-one (19.6%) patients underwent pulmonary endarterectomy operation alone, and 38 (35.5%) patients underwent balloon pulmonary angioplasty alone. To our knowledge, this is the first national cohort study that demonstrated the raw CTEPH incidence in Taiwan. It also showed the CTEPH incidence between male and female patients in the Asian population was different from the Caucasian population.

Non-invasive early exclusion of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: the InShape II study

BackgroundThe current diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is unacceptably long, causing loss of quality-adjusted life years and excess mortality. Validated screening strategies for...

Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review.

This systematic review of literature and online reports critically appraised incidence and prevalence estimates of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension to identify the most accurate estimates. Medline® and Embase® databases were searched for articles published between 1 January 2003 and 31 August 2020. Studies were grouped according to whether they were registries (population-based estimates), clinical databases (hospital-based estimates) or claims/administrative databases. Registries were classified into systematic and non-systematic registries, according to whether every national centre participated. Of 7309 publications identified, 5414 were screened after removal of duplicates and 33 were included. Inclusion was based on study type, availability of a clear numerator (diagnosed population) and a population- or hospital-based denominator, or all primary data required to calculate estimates. Only the most recent publication from a database was included. Most studies were based on European data and very few included children. In adults, the range of estimates per million was approximately 20-fold for pulmonary arterial hypertension incidence (1.5–32) and prevalence (12.4–268) and of similar magnitude for chronic thromboembolic pulmonary hypertension incidence (0.9–39) and prevalence (14.5–144). Recent (≤5 years) national systematic registry data from centralised healthcare systems provided the following ranges in adult estimates per million: approximately 5.8 for pulmonary arterial hypertension incidence, 47.6–54.7 for pulmonary arterial hypertension prevalence, 3.1–6.0 for chronic thromboembolic pulmonary hypertension incidence and 25.8–38.4 for chronic thromboembolic pulmonary hypertension prevalence. These estimates were considered the most reliable and consistent for the scientific community to plan for resource allocation and improve detection rates.

The preliminary diagnostic and therapeutic outcomes of chronic thromboembolic pulmonary hypertension registry rajaie cardiovascular medical and research center

Introduction: We herein present the preliminary results of our center's chronic thromboembolic pulmonary hypertension (CTEPH) registry of the tertiary outcomes of patients diagnosed with CTEPH and treated with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), or medical treatment. Methods: The present retrospective cross-sectional study was conducted on patients who received treatment for CTEPH in Rajaie Cardiovascular Medical and Research Center over the past four years, from 2016 to 2020. The data were obtained from routine hospital admission and outpatient visit records. The patients were divided into PEA, BPA, and medical treatment groups, and the outcomes and complications of the procedures were reported. Results: Twenty-eight patients, at a median age of 40 (33–59) years were included in our study and were followed for approximately 20.0 (16.2–23.7) months. PEA was performed on 13 (46%) patients, BPA on 10 (35%), and medical therapy only on 5 (17%). Death occurred in 4 (30%) patients after surgery, and 10 (77%) had a surgical complication. There were no deaths or recorded complications in the other two groups, but the rates of postprocedural pulmonary hypertension (30% vs. 91%; P = 0.006) and rehospitalization (7% vs. 73%; P < 0.001) were significantly lower in the operated patients. The 1-year survival rate among the entire study population was 85.7%. Conclusions: The possibility of CTEPH incidence, especially in high-risk patients with dyspnea, should always be borne in mind given its large morbidity and mortality rate and its chances of curability with early diagnosis and proper treatment.

Accurate and efficient non-invasive strategy for early identification of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism (InShape II study)

Abstract Background The current diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary embolism (PE) is unacceptably long exceeding 1 year, causing loss of quality-adjusted life years and excess mortality. Validated screening strategies to diagnose CTEPH earlier are lacking. Importantly, performing echocardiography in all PE patients for this purpose has a low diagnostic yield, is associated with overdiagnosis and is not cost-effective. Moreover, expertise in performing high-quality PH-dedicated echocardiograms may not be available outside expert centers. Aim To validate a simple screening strategy aimed at identifying CTEPH early in the course after acute PE, avoiding echocardiography if possible (Figure 1). Methods In this prospective, international, multicenter management study, consecutive PE survivors were managed according to the predefined algorithm starting three months after acute PE. All were followed for a total period of two years. The study protocol was approved by all local IRBs and all patients provided informed consent. Results 424 patients were included across three European countries (Table 1). Following the algorithm, CTEPH was considered excluded in 343 (81%) patients based on clinical pre-test probability assessment by the “CTEPH prediction score”, evaluation of symptoms and application of the “CTEPH rule-out criteria” (Figure 1); only 19% was subjected to echocardiography. Only 1 of 343 patients managed without echocardiography was diagnosed with CTEPH, 10 months after initial PE, for a failure rate of 0.29% (95% CI 0–1.6%). Overall, 13 patients were diagnosed with CTEPH (incidence 3.1%), of whom 10 within 4 months after PE diagnosis. Conclusions The algorithm accurately ruled out CTEPH and avoided echocardiography in 81% of patients. The vast majority of CTEPH cases were identified early in the course of acute PE which is a considerable improvement compared to current clinical practice with an economic use of healthcare resources. Figure 1. Study flowchart Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): This study was supported by unrestricted grants from Bayer/Merck Sharp &amp; Dohme (MSD) and Actelion Pharmaceuticals Ltd. F.A. Klok and G.J.A.M. Boon were supported by the Dutch Heart Foundation (2017T064).

Quality of initial anticoagulant treatment and risk of CTEPH after acute pulmonary embolism.

BackgroundThe pathophysiology of chronic thromboembolic pulmonary hypertension (CTEPH) is not fully understood. Poor-quality anticoagulation may contribute to a higher risk of CTEPH after acute pulmonary embolism (PE), partly explaining the transition from acute PE to CTEPH. We assessed the association between the time in therapeutic range (TTR) of vitamin-K antagonist (VKA) treatment and incidence of CTEPH after a PE diagnosis.MethodsCase-control study in which the time spent in, under and above therapeutic range was calculated in 44 PE patients who were subsequently diagnosed with CTEPH (cases). Controls comprised 150 consecutive PE patients in whom echocardiograms two years later did not show pulmonary hypertension. All patients were treated with VKA for at least 6 months after the PE diagnosis. Time in (TTR), under and above range were calculated. Mean differences between cases and controls were estimated by linear regression.ResultsMean TTR during the initial 6-month treatment period was 72% in cases versus 78% in controls (mean difference -6%, 95%CI -12 to -0.1), mainly explained by more time above the therapeutic range in the cases. Mean difference of time under range was 0% (95%CI -6 to 7) and 2% (95CI% -3 to 7) during the first 3 and 6 months, respectively. In a multivariable model, adjusted odds ratios (ORs) for CTEPH were around unity considering different thresholds for ‘poor anticoagulation’, i.e. TTR <50%, <60% and <70%.ConclusionSubtherapeutic initial anticoagulation was not more prevalent among PE patients diagnosed with CTEPH than in those who did not develop CTEPH.