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Aims: Despite refinements in balloon pulmonary angioplasty (BPA), total occlusion remains a challenge in chronic thromboembolic pulmonary hypertension (CTEPH). Owing to their low success and high complication rates, most interventional cardiologists are reluctant to address total occlusion, and there is a paucity of literature on BPA performance in total occlusion. We aimed to classify total occlusion according to morphology and present an illustrative approach for devising a tailored treatment strategy for each distinct type of total occlusion., Methods and Results: All patients diagnosed with CTEPH who underwent BPA between May 2018 and May 2022 at Fuwai Hospital in Beijing, China, were included retrospectively. A total of 204 patients with CTEPH who underwent BPA were included in this study. Among these, 38 occluded lesions were addressed in 33 patients. Based on the morphology, we categorized the lesions into three groups: pointed-head, round-head, and orifice occlusions. Pointed-head occlusion could be successfully addressed using soft-tip wire, round-head occlusion warranted hard-tip wire and stronger backup, and orifice occlusion warranted the strongest backup force. The success rates for each group were as follows: pointed-head (95.45%), round-head (46.15%), and orifice occlusion (33.33%), with orifice occlusion having the highest complication rate (50%). The classification of occlusion was associated with BPA success (round-head occlusion vs. pointed-head occlusion, OR 24.500, 95% CI 2.498-240.318, P = 0.006; orifice occlusion vs. pointed-head occlusion, OR 42.000, 95% CI 3.034-581.434, P = 0.005)., Conclusions: Occlusion morphology has a significant impact on BPA success and complication rates. A treatment strategy tailored to each specific occlusive lesion, as outlined in the present study, has the potential to serve as a valuable guide for clinical practitioners., (© 2023 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Balloon pulmonary angioplasty (BPA) is typically performed in a sequential manner., Objectives: This study aimed to determine the lowest frequency of BPA for patients who could not reach treatment goals in a short period., Design: Retrospective cohort., Methods: We retrospectively enrolled 186 BPA-treated patients diagnosed with chronic thromboembolic pulmonary hypertension. According to the accumulative number of performed BPA sessions or treated pulmonary vessels or the ratio of the number of treated pulmonary vessels/the number of baseline lesions (T/P) prior to the initial occurrence of clinical outcome or censored date, we divided patients into different groups. The principal outcome was clinical worsening., Results: After stratifying patients by the number of performed BPA sessions, most baseline parameters were comparable among groups. During follow-up, 31 (16.7%) of 186 patients experienced clinical worsening. The 6-month cumulative clinical worsening-free survival rates of ⩾2 performed sessions group were significantly higher than that of 1 performed session group. The 12-month cumulative rates of clinical worsening-free survival exhibited a declining pattern in the subsequent sequence: ⩾3, 2, and 1 performed BPA sessions, and this trend persisted when follow-up time exceeded 12 months. The 6-, 12-, and 24-month cumulative clinical worsening-free survival rates were comparable between patients with 3 and ⩾4 performed BPA sessions. Similar results were also observed when stratifying patients by the accumulative number of treated pulmonary vessels (⩽8, 9-16, ⩾17) and T/P (⩽0.789, 0.790-1.263, ⩾1.264)., Conclusion: To achieve optimal short-term outcomes, patients might need to undergo ⩾2 BPA sessions or have ⩾9 pulmonary vessels treated or have T/P ⩾0.790 within 6 months, and undergo ⩾3 BPA sessions or have ⩾17 pulmonary vessels treated or have T/P ⩾1.264 within 12 months.

Background: Balloon pulmonary angioplasty (BPA) can effectively alleviate pulmonary hypertension in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Identifying predictors of unfavorable hemodynamic response to BPA is essential to guide clinical practice. Therefore, our objective was to construct and validate a nomogram to facilitate clinicians predicting hemodynamic response to BPA., Methods: Patients with CTEPH and underwent BPA from May 2018 to April 2022 were retrospectively collected. Favorable hemodynamic response to BPA was defined as a mean pulmonary arterial pressure ≤30 mmHg and/or a reduction in pulmonary vascular resistance ≥30 % at follow-up., Results: A total of 155 patients were included. At baseline, patients with favorable hemodynamic response had significantly lower proportion of occlusive lesions (11.11 % vs. 26.32 %, P = 0.017), higher diffusing capacity of the lungs for carbon monoxide (63.77 % ± 14.10 % vs. 59.11 % ± 11.78 %, P = 0.039), and better cardiac morphology than counterparts. LASSO regression and random forest were used to construct prediction models respectively. The LASSO regression model demonstrated better predictive ability and accuracy than the random forest model, as evidenced by higher area under curve (0.745 vs. 0.740) and lower Brier score (0.192 vs. 0.195). A nomogram was constructed based on the LASSO regression model, consisting of right ventricular end-diastolic diameter/left ventricular end-diastolic diameter, number of treated pulmonary vessels and proportion of occlusion lesions. High predictive ability of the LASSO model was preserved in validation (C index 0.744)., Conclusions: The current study constructed a nomogram with high accuracy in predicting BPA hemodynamic outcome, which could facilitate decision-making in clinical practice., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: The COMPERA 2.0 4-stratum (4-S) risk score has been demonstrated superior over the 3-stratum (3-S) one in patients with pulmonary arterial hypertension and medically managed patients with chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to determine the prognostic value of the original 4-S and 3-S COMPERA 2.0 risk score and two new derivative versions in CTEPH patients who underwent balloon pulmonary angioplasty (BPA)., Methods: We retrospectively enrolled 175 BPA-treated patients with CTEPH. We assessed the risk stratification before and after each BPA session of CTEPH patients by the original 4-S and 3-S COMPERA 2.0 risk score (by rounding decimal to the nearest integer) and two new proposed derivative versions: the modified version (by rounding decimal to the next integer) and a hybrid version that fuses the original and modified versions. The primary endpoint was clinical worsening events. The secondary outcomes were achieving low-risk profile and mean pulmonary arterial pressure (mPAP) < 30 mmHg at follow-up. We used the Kaplan-Meier curve analysis to assess the survival differences between stratified patients. The comparative model's performance was evaluated in terms of discrimination by Harrell's C-index., Results: All versions of COMPERA 2.0 4-S model outperformed the 3-S one in discriminating the differences in echocardiographic and hemodynamic parameters and clinical worsening-free survival rates. The original and hybrid 4-S model could independently predict the primary and secondary endpoints, and the hybrid version seemed to perform better. The first BPA session could significantly improve risk profiles, and these changes were associated with the likelihood of experiencing clinical worsening events, achieving a low-risk profile and mPAP < 30 mmHg at follow-up. The number of BPA sessions required to achieve low risk/mPAP < 30 mmHg increased as the baseline risk score escalated., Conclusions: The COMPERA 2.0 4-S model outperformed the 3-S one in BPA-treated patients with CTEPH. The 4-S model, especially its hybrid version, could be used to predict clinical outcome before the initiation of BPA and monitor treatment response., (© 2022. The Author(s).)

Background: Attenuated heart rate recovery at 1 min (HRR1) was demonstrated to correlate with poor prognosis in patients with pulmonary arterial hypertension, whereas its role in patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains unclear., Objectives: The aim of this study was to investigate the correlations between HRR1 and functional status, echocardiography, hemodynamics, and prognosis of CTEPH., Methods: We retrospectively enrolled patients with CTEPH who underwent right heart catheterization and cardiopulmonary exercise test between June 2014 to October 2020 in Fuwai hospital. The primary outcome was clinical worsening. Linear regression was performed to assess the association between HRR1 and established markers of CTEPH severity. Receiver operating characteristic curve analysis was performed to determine the optimal cutoff of HRR1. Cox regression models were used to assess the association between HRR1 and clinical worsening., Results: A total of 211 patients with CTEPH were included in the study. After adjusting for confounders, HRR1 positively correlated with 6-min walk distance, mixed venous oxygen saturation, and peak oxygen consumption, and negatively correlated with NT-proBNP, pulmonary vascular resistance, and ventilatory equivalent of carbon dioxide. Compared with patients with HRR1 ≥16 beats, patients with HRR1 <16 beats had approximately a 3-fold risk of experiencing clinical worsening and the risk escalated with time., Conclusion: HRR1 could reflect disease severity and was independently associated with prognosis in patients with CTEPH., (© 2021 The Author(s). Published by S. Karger AG, Basel.)

Background: To achieve favorable hemodynamics, the number of balloon pulmonary angioplasty (BPA) sessions varied significantly among patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Increased BPA sessions burdened patients financially and psychologically. We aim to identify baseline characteristics that could predict early BPA response., Methods: Consecutive patients who were diagnosed with inoperable CTEPH and received BPA between May 2018 and October 2021 at Fuwai Hospital were retrospectively collected. Patients were categorized into 'Early BPA responders' or 'Non-early BPA responders' according to the hemodynamic outcome within the first three BPA sessions., Results: In total, 101 patients were included into analysis. At baseline, non-early BPA responders had lower female proportion, longer disease duration, and poorer laboratory test results compared with early responders, whereas hemodynamics were comparable. After the first three BPA sessions, hemodynamic improvement was more significant in early responders. Incidence of complication was comparable between the two groups. Multivariable logistic analysis identified that female sex (odds ratio [OR]: 7.155, 95% confidence interval [CI]: 1.323-38.692, p  = 0.022), disease duration (OR: 0.851, 95% CI: 0.727-0.995, p  = 0.043), baseline total bilirubin (OR: 0.934, 95% CI: 0.875-0.996, p  = 0.038), and baseline NT-proBNP (OR: 0.473, 95% CI: 0.255-0.879, p  = 0.018) were independently associated with early BPA response. Combination of these four parameters could predict 90% early BPA response., Conclusions: Patients with shorter disease duration, female sex, lower baseline NT-proBNP, and lower baseline total bilirubin are more likely to achieve early hemodynamic response to BPA. Moreover, early hemodynamic response was not accompanied with increased incidence of procedure-related complications.

Background: Both acute myocardial infarction and acute pulmonary embolism are distinct medical urgencies while they may conincide. Leriche's syndrome is a relatively rare aortoiliac occlusive disease characterized by claudication, decreased femoral pulses, and impotence. We present the first case of concomitant acute pulmonary embolism, acute myocardial infarction, and Leriche syndrome., Case Presentation: A 56-year-old male with a history of intermittent claudication was admitted for evaluating the sudden onset of chest pain. Elevated serum troponin level, sustained high D-dimer level, ST-T wave changes on electrocardiogram, and segmental wall motion abnormality of the left ventricle on transthoracic echocardiography were noted. Pulmonary Computed Tomography Angiogram revealed multiple acute emboli. Aortic Computed Tomography Angiogram spotted complete obstructions of the subrenal aorta and bilateral common iliac arteries with collateral circulation, maintaining the vascularization of internal and external iliac arteries. We stated the diagnosis of acute pulmonary embolism and Leriche syndrome and initiated oral anticoagulation. However, Q waves on electrocardiogram and wall motion abnormality on echocardiography persisted after embolus dissolved successfully. Coronary computed tomography angiogram found coronary arterial plaques while myocardial Positron Emission Tomography detected decreased viable myocardium of the left ventricle. We subsequently ratified the diagnosis of concurrent acute pulmonary embolism, acute myocardial infarction, and Leriche syndrome. The patient was discharged and has been followed up at our center., Conclusion: We described the first concurrence of acute pulmonary embolism, acute myocardial infarction, and Leriche syndrome.

Background: Balloon pulmonary angioplasty (BPA) has been demonstrated to improve cardiac function and exercise capacity in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but its instant impact on cardiopulmonary function has seldom been evaluated. This study aims to determine the safety and efficacy of BPA and its immediate and lasting effects on cardiopulmonary function among CTEPH patients., Methods: From May 2018 to January 2019, patients with inoperable CTEPH who underwent BPA sessions were consecutively enrolled. Hemodynamics were measured by right heart catheterization, selective pulmonary angiography and BPA were successively conducted. Hemodynamic variables, WHO functional class (WHO-FC), 6-min walk distance (6MWD) and serum NT-proBNP were evaluated before and after BPA sessions during hospitalization. Pulmonary function testing (PFT) and cardiopulmonary exercise testing (CPET) were performed within 1-3 days pre and post BPA to evaluate the effect of BPA on cardiopulmonary function., Results: Twenty-five patients with inoperable CTEPH who underwent a total of forty BPA sessions were consecutively enrolled. A total of 183 segmental or subsegmental vessels (4.6 ± 1.9 vessels per session) in 137 segments (3.4 ± 1.6 segments per session) were dilated. No procedure-related complications occurred. Instant hemodynamics, WHO-FC, 6MWD and NT-proBNP were all significantly improved after a single BPA session. Significant improvement in cardiopulmonary function was also evident as assessed by PFT indexes (forced vital capacity, forced expiratory volume in the first second, maximal voluntary ventilation) and CPET parameters (peak work rate, peak VO 2 , oxygen uptake efficiency slope). Further analysis among ten CTEPH patients receiving multiple BPA sessions (2-4 sessions) indicated BPA resulted in lasting improvements in hemodynamics and cardiopulmonary function., Conclusions: BPA, a safe and effective approach, can bring instant improvements after a single session and lasting benefits after multiple sessions to hemodynamics and cardiopulmonary function for patients with inoperable CTEPH.

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by unresolved thrombi in the pulmonary arteries and microvasculopathy in nonoccluded areas. If left untreated, progressive pulmonary hypertension will induce right heart failure and, finally, death. Currently, pulmonary endarterectomy (PEA) remains the only method that has the potential to cure CTEPH. Unfortunately, up to 40% of patients are ineligible for this procedure for various reasons. In recent years, refined balloon pulmonary angioplasty (BPA) has become an alternative option for inoperable CTEPH patients, and it may be another curative treatment in the future, particularly in combination with prior PEA. Nevertheless, 23% of patients still suffer from persistent PH after BPA. Given that CTEPH shares many similarities with idiopathic pulmonary arterial hypertension (PAH), targeted drugs developed for PAH are also attractive options for CTEPH, especially for inoperable or persistent/recurrent CTEPH patients. To date, riociguat, macitentan, and subcutaneous treprostinil are the only drugs proven by randomized control trials to be capable of improving the exercise capacity (6-min walking distance) of CTEPH patients. In this review, we summarize the achievements and unresolved problems of PAH-targeted therapy for CTEPH over the last decade.

Chronic thromboembolic pulmonary hypertension (CTEPH) is an established long-term complication of pulmonary thromboembolism (PTE). However, studies have shown that many patients with a definitive CTEPH diagnosis have no history of symptomatic PTE, suggesting that PTE is not the only cause of CTEPH. Despite extensive progress in research on pulmonary hypertension in recent years, due to a lack of relevant studies on the pathophysiology of CTEPH, implementing pulmonary endarterectomy (PEA) in patients has many challenges, and the prognosis of patients with CTEPH is still not optimistic. Therefore, revealing the pathogenesis of CTEPH would be of great significance for understanding the occurrence and development of CTEPH, developing relevant drug treatment studies and formulating intervention strategies, and may provide new preventive measures. This article summarizes the current research progress in CTEPH pathogenesis from the perspective of risk factors related to medical history, abnormal coagulation and fibrinolytic mechanisms, inflammatory mechanisms, genetic susceptibility factors, angiogenesis, in situ thrombosis, vascular remodeling, and other aspects.

Background: Takotsubo syndrome (TTS) is an acute cardiac condition with reversible heart failure which is often triggered by psychological and physical stressful events. Although pulmonary embolism (PE) was reported as a trigger for TTS, the concurrence of TTS and PE has been rarely reported, let alone that triggered by PE. Here we describe a case of a postmenopausal female presenting with symptoms similar to myocardial ischemia, which may be caused by PE, and review the available literature that may help clinicians with their practice to similar situations since no published guidelines are available., Case Presentation: An 86-year-old female was referred to the emergency department for unrelieved chest tightness, shortness of breath and back pain. Cardiac biomarkers were mildly elevated and electrocardiogram displayed pathologic Q-waves, ST-segment elevation and inverted T-waves. Unexpectedly, coronary angiography was in absence of obstructed coronary atherosclerosis or acute plaque rupture. Chest computed tomography illustrated multiple pulmonary emboli in bilateral pulmonary arteries. She had suffered from long-term right lower extremity pain and experienced a long railway journey with less activity. Both echocardiogram and cardiac magnetic resonance demonstrated regional hypokinesia of left ventricle. She received anticoagulant and diuretic therapies, three-month follow up after discharge revealed uneventful recovery without any pulmonary emboli or regional motion abnormalities, thus she was retrospectively diagnosed with TTS caused by PE., Conclusion: TTS and PE are scarcely concurrent and PE can exert as a potential trigger for TTS. TTS is easily misdiagnosed, actively seeking possible risk factors of TTS is in favor of early diagnosis and timely intervention. TTS with PE is reversible, timely and effective treatments ensure the best possible outcome.

The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Histopathologic studies revealed that pulmonary vasculature lesions similar to idiopathic pulmonary arterial hypertension (PAH) existed in CTEPH patients as well. It's well-known that genetic predisposition plays an important role in the mechanism of PAH. So we hypothesized that PAH-causing gene mutation might exist in some CTEPH patients and act as a background to facilitate the development of CTEPH. In this study, we analyzed 7 PAH-causing genes including BMPR2, ACVRL1, ENG, SMAD9, CAV1, KCNK3, and CBLN2 in 49 CTEPH patients and 17 patients recovered from pulmonary embolism (PE) but without pulmonary hypertension(PH). The results showed that the nonsynonymous mutation rate in CTEPH patients is significantly higher than that in PE without PH patients (25 out of 49 (51%) CTEPH patients vs. 3 out of 17 PE without PH patients (18%); p = 0.022). Four CTEPH patients had the same point mutation in ACVRL1 exon 10 (c.1450C>G), a mutation approved to be associated with PH in a previous study. In addition, we identified two CTEPH associated SNPs (rs3739817 and rs55805125). Our results suggest that PAH-causing gene mutation might play an important role in the development of CTEPH.

Objective: To evaluate the clinical features and reasons of misdiagnosis of connective tissue disease plus pulmonary embolism (PE)., Methods: The clinical data were reviewed retrospectively for 22 hospitalized patients with connective tissue disease and PE from February 2006 to March 2014. And the features of onset, clinical and laboratory characteristics, imaging tests and causes of misdiagnosis were analyzed., Results: There were 12 males and 10 females with an average age of (36.2 ± 14.2) years. And 12 (54.5%) PE patients had concurrent deep venous thrombosis. Antiphospholipid syndrome and Behcet's disease were the major causes of connective tissue disease. Chest distress, palpitation and extremity swelling were initial symptoms. There were elevated erythrocyte sedimentation rate (n = 11, 50.0%), elevated C-reactive protein (n = 8, 36.4%) and abnormal autoantibody (n = 15, 68.2%) respectively. Computed tomography showed pulmonary arteries stenosis, occlusion and aneurismal dilation. Eighteen (81.8%) patients had pulmonary hypertension with a systolic pulmonary pressure of (72 ± 20) mmHg (1 mmHg = 0.133 kPa). And 3 of them presented severity of pulmonary pressure non-matched to the degree of pulmonary vascular involvement. And 18 (81.8%) patients with connective tissue disease were missed due to a lack of typical symptoms and physician understanding of disease before admission. Three (13.6%) patients had a misdiagnosis of idiopathic pulmonary arterial hypertension. The median time of diagnosis was 12 months., Conclusions: Connective tissue disease may develop PE as the initial symptom. And some patients lack typical clinical manifestations of connective tissue disease. It should raise an alert with the physicians.

Introduction: The natural history of acute pulmonary embolism (PE) under treatment is about a gradual resolution of the thrombi, and uncommonly, the development of chronic thromboembolic pulmonary hypertension (CTEPH). We hypothesized that ventilatory efficiency parameters during cardiopulmonary exercise testing (CPET) may be able to monitor the process and predict CTEPH., Methods: 15 patients rehabilitated from acute PE (total resolution of thrombi), 44 patients with chronic PE (with residual thrombi), 66 patients with CTEPH, and 36 sedentary healthy controls performed incremental CPET., Results: The lowest VE/VCO₂ was higher in CTEPH patients than that in chronic PE and rehabilitated patients (43.4 L/min vs 29.9 L/min vs 27.1 L/min, p<0.005). The VE/VCO₂ slope (48.4 L/min/L/min vs 29.9 L/min/L/min vs 28.0 L/min/L/min, p<0.005) and oxygen uptake efficiency plateau (OUEP) (37.1 L/min vs 27.0 L/min vs 25.2L/min, p<0.005) had the similar changes. In logistic regression analysis, the lowest VE/VCO₂ ≥ 34.35 L/min was the best predictor of CTEPH (OR 159.0, 95% CI 36.0-702.3, p<0.001). The lowest VE/VCO₂ was higher in chronic PE patients compared with the controls (29.9 L/min vs 26.5 L/min, p<0.05), but there was no difference between the rehabilitated patients and the controls. In multiple linear regression analysis, the percentage of vascular obstruction by ventilation-perfusion lung scanning (PVO) was the most significant independent predictor for indices of ventilatory efficiency in chronic PE and rehabilitated patients., Conclusions: CTEPH is associated with weakened ventilatory efficiency. The lowest VE/VCO₂ ratio has the best capability to predict CTEPH. Ventilatory inefficiency improves along with recovery of acute PE., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized as the incomplete resolution of emboli after pulmonary embolism (PE) and the subsequent fibrotic organization and remodeling of pulmonary vascular bed. It has been reported that abnormal fibrin probably contributes to the incomplete resolution of emboli. And there is evidence that free iron could convert fibrinogen into fibrin which is remarkably resistant to lysis. Thus, we hypothesized that persistent iron overload might participate in the development of CTEPH. Materials and methods A case-control study was conducted. Forty-five CTEPH patients were enrolled as cases, and 36 age and sex frequency-matched chronic PE patients without pulmonary hypertension were selected as controls. Levels of free iron, soluble transferrin receptor (sTfR), ferritin, sTfR/ferritin ratio, hepcidin-25, tumor necrosis factor-alpha (TNF-α), interleukin (IL)-6, and malondialdehyde (MDA) were compared between the two groups. Logistic regression analysis was carried out to estimate odds ratios. Results There was no difference of the levels of free iron, hepcidin-25, sTfR, ferritin, sTfR/ferritin ratio, TNF-α, and MDA between CTEPH patients and the controls. Levels of sTfR and ferritin in both groups were within the normal limits. Levels of IL-6 in CTEPH patients were significantly higher than that in the controls. A negative correlation was observed between hepcidin-25 and sTfR (Spearman's r =−0.438, P < .001), and a positive correlation was observed between hepcidin-25 and ferritin (Spearman's r = 0.503, P < .001). In the univariate logistic regression model, there was no association observed between CTEPH and free iron, hepcidin-25, sTfR, ferritin, sTfR/ferritin ratio, TNF-α, IL-6, and MDA. Conclusions CTEPH has no association with iron overload. The iron status evaluated by sTfR and ferritin is within the normal limits in this CTEPH population. [ABSTRACT FROM AUTHOR]