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Pulmonary thromboendarterectomy (PTE), also referred to as pulmonary endarterectomy, is the definitive treatment for chronic thromboembolic pulmonary hypertension (CTEPH). The true incidence of CTEPH is unknown and difficult to ascertain; however, most experts agree that approximately 4% to 5% of all patients who have an acute episode of pulmonary embolism (PE) will continue to develop CTEPH. Based on an incidence rate of about 0.1% for acute PE, this translates into a CTEPH incidence of approximately 10,000 to 15,000 annually in the United States alone. Furthermore, there are patients with CTEPH who have no history of prior PE or deep vein thrombosis, adding to the estimated number. Despite these facts, the disease remains significantly underdiagnosed, and currently there are only about 300 PTEs performed nationwide, the majority of which are done at the University of California, San Diego (UCSD) Health System. The technical aspects of the procedure can be somewhat challenging and require meticulous and complete dissection of the entire pulmonary vascular tree, with the patient under profound hypothermic circulatory arrest. However, the determination of true CTEPH patients and those who would benefit from surgery can also be challenging and relies heavily on the experience of the CTEPH team. In this article, we will highlight some key points about the disease and describe the surgical techniques of PTE.

ISHLT members have recognized the importance of a consensus statement on the evaluation and management of patients with chronic thromboembolic pulmonary hypertension. The creation of this document required multiple steps, including the engagement of the ISHLT councils, approval by the Standards and Guidelines Committee, identification and selection of experts in the field, and the development of 6 working groups. Each working group provided a separate section based on an extensive literature search. These sections were then coalesced into a single document that was circulated to all members of the working groups. Key points were summarized at the end of each section. Due to the limited number of comparative trials in this field, the document was written as a literature review with expert opinion rather than based on level of evidence. [ABSTRACT FROM AUTHOR]

Background: Cardiopulmonary exercise testing (CPET) and pulmonary function testing (PFT) are noninvasive methods to evaluate the respiratory and circulatory systems. This research aims to evaluate and monitor chronic thromboembolic pulmonary hypertension (CTEPH) noninvasively and effectively by these two methods. Moreover, the research assesses the predictive value of CPET and PFT parameters for severe CTEPH.Methods: We used data from 86 patients with CTEPH (55 for test set, and 31 for validation set) at the Shanghai Pulmonary Hospital Affiliated to Tongji University. The clinical, PFT and CPET data of CTEPH patients of different severity classified according to pulmonary artery pressure (PAP) (mm Hg) were collected and compared. Logistic regression analysis was performed to appraise the predictive value of each PFT and CPET parameter for severe CTEPH. The performance of CPET parameters for predicting severe CTEPH was determined by receiver operating characteristic (ROC) curves and calibration curves.Results: Data showed that minute ventilation at anaerobic threshold (VE @ AT) (L/min) and oxygen uptake at peak (VO2 @ peak) (mL/kg/min) were independent predictors for severe CTEPH classified according to PAP (mm Hg). Additionally, the efficacy of VE @ AT (L/min) and VO2 @ peak (mL/kg/min) in identifying severe CTEPH was found to be moderate with the area under ROC curve (AUC) of 0.769 and 0.740, respectively. Furthermore, the combination of VE @ AT (L/min) and VO2 @ peak (mL/kg/min) had a moderate utility value in identifying severe CTEPH with the AUC of 0.843.Conclusion: Our research suggests that CPET and PFT can noninvasively and effectively evaluate, monitor and predict the severity of CTEPH. [ABSTRACT FROM AUTHOR]

In the Phase III PATENT-1 (NCT00810693) and CHEST-1 (NCT00855465) studies, riociguat demonstrated efficacy vs placebo in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Clinical effects were maintained at 2 years in the long-term extension studies PATENT-2 (NCT00863681) and CHEST-2 (NCT00910429). This post hoc analysis of hemodynamic data from PATENT-1 and CHEST-1 assessed whether riociguat improved right ventricular (RV) function parameters including stroke volume index (SVI), stroke volume, RV work index, and cardiac efficiency. REVEAL Risk Score (RRS) was calculated for patients stratified by SVI and right atrial pressure (RAP) at baseline and follow-up. The association between RV function parameters and SVI and RAP stratification with long-term outcomes was assessed. In PATENT-1 (n = 341) and CHEST-1 (n = 238), riociguat improved RV function parameters vs placebo (p < 0.05). At follow-up, there were significant differences in RRS between patients with favorable and unfavorable SVI and RAP, irrespective of treatment arm (p < 0.0001). Multiple RV function parameters at baseline and follow-up were associated with survival and clinical worsening-free survival (CWFS) in PATENT-2 (n = 396; p < 0.05) and CHEST-2 (n = 237). In PATENT-2, favorable SVI and RAP at follow-up only was associated with survival and CWFS (p < 0.05), while in CHEST-2, favorable SVI and RAP at baseline and follow-up were associated with survival and CWFS (p < 0.05). This post hoc analysis of PATENT and CHEST suggests that riociguat improves RV function in patients with PAH and CTEPH. [ABSTRACT FROM AUTHOR]

Abstract Background In chronic thromboembolic pulmonary hypertension (CTEPH) impaired pulmonary hemodynamics lead to right heart failure. Natriuretic peptides reflect hemodynamic disease severity. Pregnancy-associated plasma protein-A (PAPP-A) might address another aspect of CTEPH - chronic tissue injury and inflammation. This study assessed dynamics of PAPP-A in CTEPH patients who undergo therapy with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA). Methods The study included a total of 125 CTEPH patients scheduled for treatment (55 PEA/ 70 BPA) and a control group of 58 patients with pulmonary hypertension other than CTEPH. Biomarker measurement was performed at baseline and follow-up in the CTEPH cohort, prior to each BPA in the BPA cohort and once in the control group. Results The median PAPP-A level was slightly higher (p = 0.05) in CTEPH patients [13.8 (11.0–18.6) mU/L], than in the control group [12.6 (8.6–16.5) mU/L], without a difference between the BPA and PEA group (p = 0.437) and without a correlation to mean pulmonary artery pressure (p = 0.188), pulmonary vascular resistance (p = 0.893), cardiac index (p = 0.821) and right atrial pressure (p = 0.596). PEA and BPA therapy decreased the mean pulmonary artery pressure (p

Objectives: Electrocardiogram (ECG) and measurement of plasma brain natriuretic peptides (BNP) are established markers of right ventricular dysfunction (RVD) in the setting of acute pulmonary embolism (PE) but their value at long-term follow-up is largely unknown. The purpose of this prospective study was to determine the prevalence of ECG abnormalities, describe levels of N-terminal proBNP (NT-proBNP), and establish their association with dyspnea at long-term follow-up after PE., Design: All Swedish patients diagnosed with acute PE in 2005 ( n  = 5793) were identified through the Swedish National Patient Registry. Surviving patients in 2007 ( n  = 3510) were invited to participate. Of these, 2105 subjects responded to a questionnaire about dyspnea and comorbidities. Subjects with dyspnea or risk factors for development of chronic thromboembolic pulmonary hypertension were included in the study in a secondary step, which involved collection of blood samples and ECG registration., Results: Altogether 49.3% had a completely normal ECG. The remaining participants had a variety of abnormalities, 7.2% had atrial fibrillation/flutter (AF). ECG with any sign of RVD was found in 7.2% of subjects. Right bundle branch block was the most common RVD sign with a prevalence of 6.4%. An abnormal ECG was associated with dyspnea. AF was associated with dyspnea, whereas ECG signs of RVD were not. 61.2% of subjects had NT-proBNP levels above clinical cut-off (>125 ng/L). The degree of dyspnea did not associate independently with NT-proBNP levels., Conclusions: We conclude that the value of ECG and NT-proBNP in long term follow-up after PE lies mostly in differential diagnostics.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening pulmonary vascular disease caused by the presence of a prolonged thrombus in the pulmonary artery. CTEPH is a distinct disease entity classified as group 4 pulmonary hypertension according to the World Symposium on Pulmonary Hypertension. It is the only potentially curable cause of pulmonary hypertension. However, timely diagnosis and treatment are often hampered by nonspecific symptoms and signs and a lack of physician awareness regarding the condition. Thus, it is important to be familiar with the clinical features of CTEPH and the associated diagnostic processes. Herein, we cover the diagnostic approach for CTEPH using multimodal imaging tools in a clinical setting.

Massive pulmonary hemorrhage during pulmonary thromboendarterectomy (PTE) can be managed by a conservative approach with mechanical ventilatory support, positive end-expiratory pressure, lung isolation, reversal of heparin, and correct of coagulopathy. We present three challenging cases that developed intrapulmonary hemorrhage during/after PTE and managed successfully. The first patient had bleeding from the bronchial artery and right internal mammary collaterals, which was managed by coil-embolization. The second patient had a breach in the blood airway barrier in the right upper lobar segment of the lung, and the repair was done using a surgical absorbable hemostat. The third patient developed reperfusion injury, he was instituted on veno-venous extracorporeal membranous oxygenation, a week later, the patient recovered completely. An algorithm was adopted and modified to our requirements; all the 3 challenging intrapulmonary hemorrhage cases were successfully managed. This algorithm can be used for satisfactory outcomes in patients who suffer intrapulmonary hemorrhage during PTE. [ABSTRACT FROM AUTHOR]

Introduction The gold standard curative treatment for chronic pulmonary thromboembolic disease and pulmonary hypertension (CTEPH) is pulmonary thromboendarterectomy (PTE). Balloon pulmonary angioplasty (BPA) is emerging for distal CTEPH patients, where lesion is inaccessible for surgery. This is the first study conducted in Indian patients for evaluating the impact of BPA in patients with distal CTEPH. Aims and Objectives To evaluate the effect of BPA in patients with distal CTEPH with the help of New York Heart Association (NYHA) functional class and 6-minute walk distance (6MWD). To study decrease in pulmonary artery pressure and improvement in right atrial and right ventricular function with the help of echocardiography. Material and Methods Study population—CTEPH patients presenting to the hospital during the study interval of 16 months from January 2017 to April 2018. A. Method: This was an observational retrospective and prospective follow-up study. All distal CTEPH patients after meeting inclusion and exclusion criteria were taken for study. B. Detailed case records containing information on demographics, clinical features and necessary blood and imaging investigations, ventilation/perfusion (V/Q) scan, computed tomography pulmonary angiography (CTPA), right heart catheterization (RHC) study, pulmonary angiogram, and BPA procedure details were obtained for all participants. C. After 8 weeks of last BPA session, patients were assessed clinically and then 6-minute walk test, blood investigations and echocardiography were done. Data Analysis Statistical analysis was performed by using the software SPSS 22.0 version. We used paired t-test to test the significant difference in the mean pre- and post-BPA. p-value < 0.05 will be considered as statistically significant. Results In our study, mean age of presentation was 39.81 ± 12 years. Out of 11 patients, 5 were females and 6 were males. Mean duration of symptoms was 40.5 months. The total number of BPA sessions performed were 30. The minimum number of BPA sessions undergone was 1 and maximum number of BPA sessions undergone by one patient was 5, with 45% of patients undergoing 2 BPA sessions. The total number of segmental arteries dilated was 104. Segmental vessels dilated per each session was 3.46. There was statistically significant improvement in NYHA class and 6MWD after BPA. 6MWD increased from 299 m to 421 m (p-value < 0.001). This improvement in functional capacity is strongly associated with the improvement in right ventricular (RV) function (tricuspid annular plane systolic excursion [TAPSE] from 15.3 mm to 18.9 mm) and with the reduction in pulmonary artery systemic pressure (PASP) (from 92 mm Hg to 60 mm Hg). Conclusion In patients with distal CTEPH who undergo BPA, there was statistically significant improvement in 6MWD. These changes correspond to a treatment-induced reduction in pulmonary artery pressure and lend support to use of BPA in patients with distal CTEPH. ECHO and 6MWD can be used for evaluating BPA efficacy and monitoring disease progression. [ABSTRACT FROM AUTHOR]

Chronic thrombo-embolic pulmonary hypertension (CTEPH) develops in a subset of patients after acute pulmonary embolism. In CTEPH, pulmonary vascular resistance, which is initially elevated due to the obstructions in the larger pulmonary arteries, is further increased by pulmonary microvascular remodeling. The increased afterload of the right ventricle (RV) leads to RV dilation and hypertrophy. This RV remodeling predisposes to arrhythmogenesis and RV failure. Yet, mechanisms involved in pulmonary microvascular remodeling, processes underlying the RV structural and functional adaptability in CTEPH as well as determinants of the susceptibility to arrhythmias such as atrial fibrillation in the context of CTEPH remain incompletely understood. Several large animal models with critical clinical features of human CTEPH and subsequent RV remodeling have relatively recently been developed in swine, sheep, and dogs. In this review we will discuss the current knowledge on the processes underlying development and progression of CTEPH, and on how animal models can help enlarge understanding of these processes.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots and scar tissue in the blood vessels of the lungs. Health-related quality of life is often significantly impaired in patients with CTEPH. However, a better understanding of how CTEPH symptoms affect patients' lives is needed to optimally assess the impact of the disease and treatment. Objectives: This qualitative study aimed to better understand the symptoms of CTEPH and how they affect patients' lives, as well as to determine the appropriateness of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire for use in this patient population. Methods: Adults diagnosed with CTEPH, recruited from two clinical sites in the US, participated in one-to-one qualitative telephone interviews. They described their experience of CTEPH symptoms and the impact these symptoms have on their lives. They also provided feedback on the comprehensibility and relevance of the PAH-SYMPACT™'s instructions, items, and response options. Results: Participants (N = 12) had a mean age of 62.5 years. Two thirds were female and most (83%) had undergone pulmonary endarterectomy and/or balloon pulmonary angioplasty. The most frequently endorsed symptoms were shortness of breath (endorsed by all 12 participants), fatigue (11 participants), and lightheadedness (10 participants). All participants identified shortness of breath as an "extremely important" symptom, and seven participants rated fatigue as "extremely important." The most frequent impacts of CTEPH were on ability to walk quickly (endorsed by all 12 participants), ability to walk up inclines or stairs (11 participants), and ability to carry things (11 participants). The PAH-SYMPACT™ items were relevant to most participants and reflected their experience of CTEPH. All participants indicated that no important CTEPH symptoms were missing from the PAH-SYMPACT™. Overall, the instructions, items, and response options of the PAH-SYMPACT™ were clear and easy to understand. Conclusions: The symptoms and impacts experienced by patients with CTEPH align with items included in the PAH-SYMPACT™. The PAH-SYMPACT™ appears to be fit for purpose for assessing disease status in patients with CTEPH. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (iPAH) are both associated with right ventricular (RV) failure and mortality. However, CTEPH patients are older, more often male and usually have more co-morbidities than iPAH patients, including a history of venous thromboembolism. Therefore, RV adaptation to pressure-overload in CTEPH may be different than in iPAH. We included all treatment-naive CTEPH and iPAH patients diagnosed in the Amsterdam UMC between 2000 and 2019 if cardiac magnetic resonance imaging (CMR) and a right heart catheterization were performed at time of diagnosis. Load-dependent RV volumes and mass were assessed with CMR. Load-independent RV contractility, afterload and diastolic stiffness in relation to afterload were obtained using single beat pressure-volume loop analysis. Differences in RV characteristics between CTEPH and iPAH were analyzed using multiple linear regression with interaction testing after correcting for confounders. We included 235 patients in this study and performed pressure-volume loop analysis in 136 patients. In addition to being older and more often male, CTEPH patients had a lower pulmonary vascular resistance than iPAH patients at the time of diagnosis. After correcting for these confounders, CTEPH patients had a somewhat higher RV end-diastolic volume index (87 ± 27 ml vs 82 ± 25 ml; p <.01), and a lower RV relative wall thickness (0.6 ± 0,1 g/ml vs 0.7 ± 0,2 g/ml; p <.01). The correlation coefficient of RV diastolic stiffness to afterload was higher in CTEPH compared to iPAH (p <.05; independent of age and gender). Despite differences in patient characteristics, disease etiology and physiology, RV functional parameters in CTEPH and iPAH are mostly similar. The right ventricle in CTEPH is marginally more dilated, stiffer and less hypertrophic than in iPAH. [ABSTRACT FROM AUTHOR]

Understanding patient journey and burden of disease in patients with chronic thromboembolic pulmonary hypertension (CTEPH) helps improve diagnostic and treatment processes. This study aimed to explore patient journey from time of disease onset to a definitive diagnosis and disease burden in Japanese patients with CTEPH. A mixed-methods study exploring patient journey and disease burden of 33 Japanese patients with a definitive diagnosis of CTEPH. The patients from 2 university hospitals underwent semistructured interviews. Data were transcribed into verbatim records, and 2 independent researchers conducted thematic analyses. Data concerning patient journey were also analyzed quantitatively with supplementary use of medical records. Median times from initial onset of symptoms to a confirmed diagnosis and first visitation to a medical institution to a definitive diagnosis of CTEPH were 32 and 20 months, respectively. Thematic analyses found that, for patients, reasons for delay in seeking initial consultations included misattribution of symptoms to aging or lack of physical strength. For healthcare providers, reasons for delays in diagnosis included poor recognition of CTEPH and difficulty in recalling the disease as a differential diagnosis. Burdens of CTEPH were caused by physical symptoms, and mental and social issues, including restriction of daily activities owing to oxygen therapy, disappointment with the intractable nature of the disease, poor understanding of the disease by other people, and lack of social networks. This study highlighted physical, mental, and social burdens in patients with CTEPH and possible missed opportunities in making the diagnosis of CTEPH during the patient journey. Increasing disease awareness in healthcare providers and networking among patients may contribute to better patient care. i. Although many patients with chronic thromboembolic pulmonary hypertension (CTEPH) have a chief complaint of exertional breathlessness, early diagnosis is difficult because of the low frequency and lack of general awareness of the disease, as well as the lack of characteristic symptoms and physical findings that differentiate CTEPH from other diseases. ii. The study showed that the time from initial onset of symptoms to diagnosis of CTEPH was long, and in many patients, the disease already was advanced by the time a diagnosis was made. Reasons for such delays included misinterpretation of initial symptoms by patients and difficulty in recalling CTEPH as a differential diagnosis by physicians. Patients with CTEPH experienced physical, mental, and social burdens related to the intractable and rare nature of this disease. iii. These findings suggest that raising awareness of the disease among patients with risk factors for CTEPH, promoting physician-patient cooperation to prevent diagnostic errors, and developing networks among patients with CTEPH could be effective in achieving early diagnosis of the disease and reducing the disease burdens in these patients. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed and undertreated sequelae of acute pulmonary embolism. In this comprehensive review, we provide an introductory overview of CTEPH, highlight recent advances in its diagnostic imaging, and describe the surgical technique for pulmonary thromboendarterectomy (PTE), the only established curative treatment for CTEPH. We also discuss the emerging role of balloon pulmonary angioplasty, both independently and combined with PTE, for patients with inoperable, residual, or refractory pulmonary hypertension post PTE. Finally, we stress the importance of a specialized multidisciplinary team approach to CTEPH patient care and share our approach to optimizing care for these patients. [ABSTRACT FROM AUTHOR]

Purpose: The aim of the study was to investigate the potential of electrocardiogram (ECG)-gated computed tomography pulmonary angiography (CTPA) as a predictor of disease severity in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Method: Forty-five CTEPH patients with a mean age of 63.8 years±12.7 y (±standard deviation) who had undergone ECG-gated CTPA and right heart catheterization (RHC) were included in the study. Right ventricular to left ventricular volume ratio (RVV/LVV), diameter ratio on 4-chamber view (RVD4CH/LVD4CH), pulmonary trunk (PT) diameter, PT to aortic diameter ratio (PT/A), and septal angle were correlated to mean pulmonary artery pressure (mPAP). Moreover, RVV/LVV and RVD4CH/LVD4CH were adjusted to pulmonary diameter index (PADi) and PT/A index. Areas under the curve (AUC) for predicting mPAP above 40 mmHg, 35 mmHg, and 30 mmHg were calculated. Results: RVD4CH/LVD4CH revealed the strongest correlation to mPAP before (r = 0.6507) and after (r = 0.7650; p < 0.0001) PT/A adjustment. The AUCs for predicting pH with mPAP over 40 mmHg and 30 mmHg were 0.9229 and 0.864, respectively. A cutoff value of 1.298 enabled prediction of pH with mPAP over 40 mmHg with a sensitivity, specificity, positive predictive, and negative predictive value of 80.00 %, 95.83 %, 88.46 %, and 94.12 %, respectively. Intra- and interobserver variability were excellent for all parameters. Conclusion: Combining different and easily evaluable ECG-gated CTPA parameters enables excellent prediction of pulmonary hemodynamics in CTEPH patients. Ventricular diameter ratio on 4-chamber view adjusted by the PT/A ratio yielded the best correlation to mPAP.

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Background: In the patients with pulmonary embolism (PE), PE itself can cause haemoptysis and other reasons can also cause haemoptysis. Therefore, the clinical characteristics and the causes of haemoptysis are lacking. Methods: A retrospective analysis was performed that involved screening 583 PE patients and determining that haemoptysis occurred in 141 cases. Of these, eight cases were omitted due to anticoagulation‐related haemoptysis or unavailable data, leaving 133 cases that were enrolled in final analysis (127 acute and 6 chronic case of PE). We classified the acute PE patients who combined with diseases which can cause haemoptysis to non‐simple group (n = 61) and those without these diseases to simple group (n = 66). Results: The incidence of haemoptysis in PE patients was 23.75%. In the simple group, the amount of haemoptysis ≤ 5 mL was 80.30% (53/66) and ≤ 20 mL was 90.91% (60/66). In the non‐simple group who combined with lung cancer, the amount of haemoptysis ≤ 5 mL was 68.4% (26/38) and ≤ 20 mL was 86.8% (33/38). Further analyses revealed that the amount of haemoptysis in the non‐simple group was larger than that in the simple group (median 5 [5‐125] vs. 5 [5‐5], p < 0.001; volume ≥ 100 mL: 29.5% vs. 6.1%, p< 0.001). Among all the PE patients, chronic thromboembolic pulmonary hypertension (CTEPH), PE combined with tuberculosis (TB) and PE combined with bronchiectasis were independent risk factors for the amount of haemoptysis ≥ 100 mL (OR = 15.00, (95% CI: 2.235‐100.652); 12.00, (3.101‐46.437); 60.00, (6.552‐549.441), respectively). Conclusions: The haemoptysis caused by acute PE or PE combined with lung cancer was mild and was characterised by blood in sputum. PE combined with TB, bronchiectasis and CTEPH are associated with moderate to massive haemoptysis, with a greater risk of haemoptysis ≥ 100 mL. [ABSTRACT FROM AUTHOR]

To investigate if the pulmonary arterial hypertension (PAH) risk assessment tool presented in the 2015 ESC/ERS guidelines is valid for patients with chronic thromboembolic pulmonary hypertension (CTEPH) when taking pulmonary endarterectomy (PEA) into account. Design. Incident CTEPH patients registered in the Swedish PAH Registry (SPAHR) between 2008 and 2016 were included. Risk stratification performed at baseline and follow-up classified the patients as low-, intermediate-, or high-risk using the proposed ESC/ERS risk algorithm. Results. There were 250 CTEPH patients with median age (interquartile range) 70 (14) years, and 53% were male. Thirty-two percent underwent PEA within 5 (6) months. In a multivariable model adjusting for age, sex, and pharmacological treatment, patients with intermediate-risk or high-risk profiles at baseline displayed an increased mortality risk (Hazard Ratio [95% confidence interval]: 1.64 [0.69–3.90] and 5.39 [2.13–13.59], respectively) compared to those with a low-risk profile, whereas PEA was associated with better survival (0.38 [0.18–0.82]). Similar impact of risk profile and PEA was seen at follow-up. Conclusion. The ESC/ERS risk assessment tool identifies CTEPH patients with reduced survival. Furthermore, PEA improves survival markedly independently of risk group and age. Take home message: The ESC/ERS risk stratification for PAH predicts survival also in CTEPH patients, even when taking PEA into account. [ABSTRACT FROM AUTHOR]

Systemic Sclerosis (SSc) is a multi-systemic connective tissue disease of unknown etiology. Although many pathological processes play a role in the basis of pulmonary hypertension (PHT) that develops secondary to SSc, vasculopathy has an important place. Chronic thromboembolic hypertension (CTEPH) is in the group 4 PHT class. CTEPH distinguishes it from other causes of PHT by having both surgical and medical treatment options. CTEPH is a pathology that develops chronically and can be overlooked due to its nonspecific symptoms. Early diagnosis and treatment can reduce morbidity and mortality. The physiopathology of vasculopathy secondary to CTEPH and vasculopathy of SSc made us suspect that similar processes operate in both diseases. The processes that cause and follow endothelial damage are similar in both diseases. If this pathophysiological mechanism can be clarified, possible new treatment options can be discovered. We diagnosed CTEPH with the examinations we performed in two of our patients with SSc and interstitial lung disease, both of which developed PHT. We aimed to discuss the immunopathogenesis with two case reports. [ABSTRACT FROM AUTHOR]

Background Hypodense filling defects within the pulmonary veins on computed tomography described as pulmonary vein sign (PVS) have been noted in acute pulmonary embolism and shown to be associated with poor prognosis. We evaluated venous flow abnormalities in chronic thromboembolic pulmonary hypertension (CTEPH) to determine its usefulness in the computed tomography assessment of CTEPH. Methods and Results Blinded retrospective computed tomography analysis of 50 proximal CTEPH cases and 3 control groups—50 acute pulmonary embolism, 50 nonthromboembolic cohort, and 50 pulmonary arterial hypertension. Venous flow reduction was assessed by the following: (1) presence of a filling defect of at least 2 cm in a pulmonary vein draining into the left atrium, and (2) left atrium attenuation (>160 Hounsfield units). PVS was most prevalent in CTEPH. Compared with all controls, sensitivity and specificity of PVS for CTEPH is 78.0% and 85.3% (95% CI, 64.0–88.5 and 78.6–90.6, respectively) versus 34.0% and 70.7% (95% CI, 21.2−48.8 and 62.7–77.8) in acute pulmonary embolism, 8.0% and 62% (95% CI, 2.2–19.2 and 53.7–69.8) in nonthromboembolic and 2.0% and 60% (95% CI, 0.1−10.7 and 51.7−67.9) in pulmonary arterial hypertension. In CTEPH, lobar and segmental arterial occlusive disease was most commonly associated with corresponding absent venous flow. PVS detection was highly reproducible (Kappa=0.96, 95% CI, 0.90–1.01, P

Background: Residual pulmonary vascular occlusion (RPVO) affects one half of patients after a pulmonary embolism (PE). The relationship between the risk factors and therapeutic interventions for the development of RPVO and chronic thromboembolic pulmonary hypertension is unknown., Methods: This retrospective review included PE patients within a 26-month period who had baseline and follow-up imaging studies (ie, computed tomography [CT], ventilation/perfusion scans, transthoracic echocardiography) available. We collected the incidence of RPVO, percentage of pulmonary artery occlusion (%PAO), baseline CT %PAO, most recent CT %PAO, and difference between the baseline and most recent %PAO on CT (Δ%PAO)., Results: A total of 354 patients had imaging reports available; 197 with CT and 315 with transthoracic echocardiography. The median follow-up time was 144 days (interquartile range [IQR], 102-186 days). RPVO was present in 38.9% of the 354 patients. The median Δ%PAO was -10.0% (IQR, -32% to -1.2%). Fewer patients with a provoked PE developed RPVO (P ≤ .01), and the initial troponin level was lower in patients who developed RPVO (P = .03). The initial thrombus was larger in the patients who received advanced intervention vs anticoagulation (baseline CT %PAO: median, 61.2%; [IQR, 27.5%-75.0%] vs median, 12.5% [IQR, 2.5%-40.0%]; P ≤ .0001). Catheter-directed thrombolysis (CDT; median Δ%PAO, -47.5%; IQR, -63.7% to -8.7%) and surgical pulmonary embolectomy (SPE; median Δ%PAO, -42.5; IQR, -68.1% to -18.7%) had the largest thrombus reduction compared with anticoagulation (P = .01). Of the 354 patients, 76 developed pulmonary hypertension; however, only 14 received pulmonary hypertension medications and 12 underwent pulmonary thromboendarterectomy. Cancer (odds ratio [OR], 1.7) and planned prolonged anticoagulation (>1 year; OR, 2.20) increased the risk of RPVO. In contrast, the risk was lower for men (OR, 0.61), patients with recent surgery (OR, 0.33), and patients treated with SPE (OR, 0.42). A larger Δ%PAO was found in men (coefficient, -8.94), patients with a lower body mass index (coefficient, -0.66), patients treated with CDT (coefficient, -18.12), and patients treated with SPE (coefficient, -21.69). A lower Δ%PAO was found in African-American patients (coefficient, 7.31)., Conclusions: The use of CDT and SPE showed long-term benefit in thrombus reduction., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) and venous thromboembolism (VTE) are thought to share many common risk factors. Our study aimed to determine the frequencies of 5 thrombosis-related gene single nucleotide polymorphisms (SNPs) associated with VTE in patients with CTEPH (n 129) compared with a control group of healthy individuals without a history of VTE (n 2637)., Methods: The SNPs of the following genes were investigated: F5 (F V Leiden, rs6025), F2 prothrombin (rs1799963), fibrinogen gamma (FGG, rs2066865), F11 (rs2289252) and ABO (non-O, rs8176719) in both groups., Results: The study found that the rs1799963 variant was more common in patients with chronic thromboembolic pulmonary hypertension (CTEPH) compared to the control group (p < .0001). The GA heterozygous variant showed a significant increase with an odds ratio (OR) of 4.480 (95% CI: 2.344-8.562) or a finding by maximum likelihood analysis (MLA) with p < .0001. Additionally, there was a notable increase in the rs8176719 variant with p < .0001 in CTEPH patients. Both the homozygous G/G variant and the heterozygous -/G variant also showed an increase, with OR of 4.2317 (95% CI: 2.45571-7.2919) and 2.4324 (95% CI: 1.46435-4.0403) respectively, or MLA (p < .0001 and p .0006). The study also revealed a higher prevalence of the heterozygous C/T variant of rs2289252 in CTEPH patients, with an OR of 1.5543 (95% CI: 1.02503-2.3568) or MLA (p .0379)., Conclusion: The study suggests that the observed gene polymorphisms F2 (rs1799963), ABO (rs8176719), and F11 (rs2289252) may play a role as independent heritable risk factors in the development of CTEPH., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

This study examined sST2, GDF-15, and galectin-3 as indicators of disease severity and therapy response in chronic thromboembolic pulmonary hypertension (CTEPH). This study included 57 inoperable CTEPH patients who underwent balloon pulmonary angioplasty and 25 controls without cardiovascular disease. Biomarker levels were examined in relation to advanced hemodynamic impairment [tertile with worst right atrial pressure (RAP) and cardiac index], hemodynamic therapy response [normalized hemodynamics (meanPAP ≤25 mmHg, PVR ≤3 WU and RAP ≤6 mmHg) or a reduction of meanPAP ≥25%; PVR ≥ 35%, RAP ≥25%]. GDF-15 [820 (556–1315) pg/ml vs. 370 (314–516) pg/ml; p < 0.001] and sST2 [53.7 (45.3–74.1) ng/ml vs. 48.7 (35.5–57.0) ng/ml; p = 0.02] were higher in CTEPH patients than in controls. At baseline, a GDF-15 level ≥1443 pg/ml (AUC 0.88; OR 31.4) and a sST2 level ≥65 ng/ml (AUC 0.80; OR 10.9) were associated with advanced hemodynamic impairment. At follow-up GDF-15 ≤ 958 pg/ml (AUC = 0.74, OR 18) identified patients with optimal hemodynamic therapy response and ≤760 pg/ml (AUC = 0.79, OR 14). GDF-15 and sST2 levels are higher in CTEPH and identified patients with advanced hemodynamic impairment. Further, decreased GDF-15 levels at follow-up were associated with hemodynamic therapy response. The diagnostic strength was not superior to NT-proBNP. [ABSTRACT FROM AUTHOR]

The diagnostic strategy for chronic thromboembolic pulmonary hypertension (CTEPH) is composed of two components required for a diagnosis of CTEPH: the presence of chronic pulmonary embolism and an elevated pulmonary artery pressure. The current guidelines require that ventilation–perfusion single-photon emission computed tomography (VQ-SPECT) is used for the first step diagnosis of chronic pulmonary embolism. However, VQ-SPECT exposes patients to ionizing radiation in a radiation sensitive population. The prospective, multicenter, comparative phase III diagnostic trial CTEPH diagnosis Europe - MRI (CHANGE-MRI, ClinicalTrials.gov identifier NCT02791282) aims to demonstrate whether functional lung MRI can serve as an equal rights alternative to VQ-SPECT in a diagnostic strategy for patients with suspected CTEPH. Positive findings are verified with catheter pulmonary angiography or computed tomography pulmonary angiography (gold standard). For comparing the imaging methods, a co-primary endpoint is used. (i) the proportion of patients with positive MRI in the group of patients who have a positive SPECT and gold standard diagnosis for chronic pulmonary embolism and (ii) the proportion of patients with positive MRI in the group of patients with negative SPECT and gold standard. The CHANGE-MRI trial will also investigate the performance of functional lung MRI without i.v. contrast agent as an index test and identify cardiac, hemodynamic, and pulmonary MRI-derived parameters to estimate pulmonary artery pressures and predict 6–12 month survival. Ultimately, this study will provide the necessary evidence for the discussion about changes in the recommendations on the diagnostic approach to CTEPH.

Aim. The aim of current study was to estimate the economic burden of the chronic thromboembolic pulmonary hypertension (CTEPH) in Russia based on patient registry. Materials and methods. Cost of illness study was based on data derived from CTEPH patient registry that was developed at the Division of hypertension of FSBI “National Medical Research Center of Cardiology”. Demographic and clinical patient characteristics were analyzed with descriptive statistic methods. Cost of illness study was performed from the state perspective and with bottom-up approach. Bootstrapping was used for calculation of average costs per patient/year. Within the study direct costs (medical costs: outpatient, inpatient, emergency, PAH-specific therapy, concomitant therapy; non-medical costs: pension due to disability status, payments for patients on sick-leave) and indirect costs (loss in GDP) were estimated. Results. Overall, 113 CTEPH patients (67 women and 46 men) from 33 Russian regions were included, mean age of patients with CTEPH was 54.6±13.95 years. Most of the patients (55%) were in able-bodied age. It was found that about half of patients with diagnosed CTEPH had a disability. Average duration of disease at the time of analysis was 6.88±11.41 years. Period from the first occurrence of symptoms to the confirmation of diagnosis of CTEPH was 2.58±5.21 years on average. More than 70% of patients had III and IV FC (WHO) at the time of diagnosis. Mean number of outpatient visits was 1.97±1.65 per patient/year, and inpatient visits were reported for 59% of patients. About 54% of patients used PAH-specific therapy, moreover 46% patients had interruptions of PAH-specific therapy (58.4±66.3 days). The total costs of CTEPH per patient/year were calculated as 805,901 RUB. The overall burden of CTEPH in Russia for total CTEPH population (470 patients) was 379 million RUB per year. Conclusions. CTEPH is the rare disease that is characterized with later diagnosis due to absence of disease-specific symptoms. Therefore economic burden of the CTEPH is significantly low in comparison to widespread cardiovascular diseases. Development of network of expert PH-centers and increase of the access for PAH-specific therapy will help to increase the quality of health care for patients with CTEPH.

Orphan diseases are life-threatening, chronic, progressive and leading to a reduction in life expectancy or disability conditions. Patients with rare diseases are one of the vulnerable population groups, which is caused by several factors, such as insufficient awareness of practitioners about such diseases, complicated and lengthy diagnosis, and imperfection of legal environment in the area of pharmacological support of orphan diseases, when only 24 orphan diseases (List-24) are determined, which are treated at the expense of the RF subjects.Imperfection of federal orphan diseases legislation is shown on the example of providing patients with PAH-specific therapy: only idiopathic form of pulmonary hypertension is included in the federal List-24, there is no well-established mechanism of pharmacological support for patients with chronic thromboembolic pulmonary hypertension (CTEPH) that is not included in the List-24. The article describes the successful experience in providing patients with orphan diseases, regardless of the inclusion of nosology in List-24 in Moscow, through the creation and implementation of the transparent medical and pharmacological support regulation for patients with orthopaedic diseases, which settled the timely process of provision of patients with the necessary therapy. As a result, patients with CTEPH also have access to existing pathogenetic treatment that can prolong life and improve the quality of life of such patients.

Background: In chronic thromboembolic pulmonary hypertension (CTEPH) impaired pulmonary hemodynamics lead to right heart failure. Natriuretic peptides reflect hemodynamic disease severity. Pregnancy-associated plasma protein-A (PAPP-A) might address another aspect of CTEPH - chronic tissue injury and inflammation. This study assessed dynamics of PAPP-A in CTEPH patients who undergo therapy with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA).Methods: The study included a total of 125 CTEPH patients scheduled for treatment (55 PEA/ 70 BPA) and a control group of 58 patients with pulmonary hypertension other than CTEPH. Biomarker measurement was performed at baseline and follow-up in the CTEPH cohort, prior to each BPA in the BPA cohort and once in the control group.Results: The median PAPP-A level was slightly higher (p = 0.05) in CTEPH patients [13.8 (11.0-18.6) mU/L], than in the control group [12.6 (8.6-16.5) mU/L], without a difference between the BPA and PEA group (p = 0.437) and without a correlation to mean pulmonary artery pressure (p = 0.188), pulmonary vascular resistance (p = 0.893), cardiac index (p = 0.821) and right atrial pressure (p = 0.596). PEA and BPA therapy decreased the mean pulmonary artery pressure (p < 0.001) and pulmonary vascular resistance (p < 0.001) and improved the WHO-functional-class (baseline: I:0/II:25/III:80/IV:20 vs. follow-up: I:55/II:58/III:10/IV:2). PAPP-A levels decreased after PEA [13.5 (9.5-17.5) vs. 11.3 (9.8-13.6) mU/L; p = 0.003) and BPA treatment [14.3 (11.2-18.9) vs. 11.1 (9.7-13.3) mU/L; p < 0.001). The decrease of PAPP-A levels is delayed in comparison to N-terminal pro-B-type natriuretic peptide.Conclusion: PAPP-A is overexpressed in CTEPH and decrease significantly after surgical or interventional therapy, however without association to hemodynamics. Further investigation is needed to define the underlying mechanism of PAPP-A expression and changes after therapy in CTEPH. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group-4 pulmonary hypertension caused by organized thrombi in pulmonary arteries and vasculopathy in nonoccluded areas leading to right heart failure and death. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy (PEA), which is the guideline recommended treatment. There is increasing experience with balloon pulmonary angioplasty (BPA) for inoperable patients; this option, like PEA, is reserved for specialized centers with expertise in this treatment method. Inoperable patients are candidates for targeted drug therapy. Riociguat remains the only approved medical therapy for CTEPH patients deemed inoperable or with persistent pulmonary hypertension after PEA. The role of riociguat therapy preoperatively or in tandem with BPA is currently under investigation. The purpose of this review is to evaluate the safety and efficacy of riociguat in the treatment of CTEPH. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) is confirmed by visual analysis of single-photon emission computer tomography (SPECT) ventilation and perfusion (V/Q) images. Defects in the perfusion image discordant with the ventilation image indicate obstructed segments and the positive diagnosis of CTEPH. A quantitative metric and classification algorithm are proposed based on volumetric data from SPECT V/Q images. The difference in ventilation and perfusion volumes (VV-P) is defined as a quantitative metric to identify discordant defects in the SPECT images. The algorithm was validated with 22 patients grouped according to their diagnosis: (1) CTEPH and (2) respiratory pathology. Volumetric data from SPECT perfusion images was also compared before and after treatment for CTEPH. CTEPH was detected with a sensitivity of 0.67 and specificity of 0.80. The performance of volumetric data from SPECT perfusion images for the evaluation of treatment response was studied for two cases and improvement of pulmonary perfusion was observed in one case. This study uses volumetric data from SPECT V/Q images for the diagnosis of CTEPH and its differentiation from respiratory pathologies. The results indicate that the defined metric is a viable option for a quantitative analysis of SPECT V/Q images. [ABSTRACT FROM AUTHOR]

Abstract Background Although atrial function has prognostic significance in many cardiovascular conditions, changes during exercise have not previously been assessed. The aim of this study was to evaluate left atrial (LA) and right atrial (RA) volume and function during incremental exercise, both in normal individuals, healthy athletes, and in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods Fifteen healthy non-athletes, 15 athletes and 15 CTEPH patients underwent multi-slice real-time cardiovascular magnetic resonance imaging at rest and during supine bicycle exercise with simultaneous invasive hemodynamic measurements. Results At rest, athletes had larger indexed maximal RA and LA volumes (iRAVmax, iLAVmax) than CTEPH patients and non-athletes, the latter two groups having similar values. CTEPH patients had lower RA and LA emptying functions (EmF) at rest. During exercise, RA volumes (maximum and minimum) increased in CTEPH patients, whilst decreasing in athletes and non-athletes (P

• Complete endarterectomy must be bilateral requiring careful dissection of the entire pulmonary tree. • Perfect visualization during endarterectomy is essential and can be accomplished by using cardiopulmonary bypass and periods of deep hypothermic circulatory arrest. • Identification and development of the correct endarterectomy plane is crucial. Pulmonary thromboendarterectomy (PTE) remains the treatment of choice for patients with operable chronic thromboembolic pulmonary hypertension (CTEPH). It is a technically demanding operation that offers patients with CTEPH improvements in survival and quality of life. In this article, we describe the operative technique of PTE in detail. [ABSTRACT FROM AUTHOR]

Background: Chronic thromboembolic pulmonary hypertension (CTEPH), a complication of acute pulmonary embolism, is a potentially curable form of pulmonary hypertension (PH). CTEPH is under-diagnosed for a number of reasons leading to delayed referral or missed diagnosis; however, the frequency of misdiagnosis of CTEPH findings on computed tomography (CT) is currently unknown. Purpose: To evaluate the extent of misdiagnosis of CTEPH on CT. Material and Methods: We studied the original CT reports from 35 patients with confirmed CTEPH diagnosis referred to a specialist center for pulmonary endarterectomy during 2011–2016. The patients' CT reports were assessed according to a standardized form and compared to a consensus reading by two expert radiologists. Results: The expert reading identified all CTEPH cases. However, in the original reports, the terminology "CTEPH" was only used in two patients. Another seven descriptive reports picked up the combination of PH and vascular signs of CTEPH without making a definitive diagnosis. Taking these nine cases as positive for CTEPH, the overall sensitivity on a diagnostic level was 26%. Pulmonary arterial abnormalities were described in isolation in 63% with no mention of PH or CTEPH. Signs of PH and mosaic attenuation were documented in 53% and 6% of the original reports, respectively, where it could be seen on the CT examination. Conclusion: The study shows that radiologists frequently miss CTEPH findings, leading to a falsely low sensitivity for CT. There was also a notable discrepancy in how the findings were presented in radiology reports. [ABSTRACT FROM AUTHOR]

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Pulmonary hypertension (PH) is a disease characterized by an increase in the pulmonary vascular resistance that typically progresses to right heart failure and death. It is classified into five groups. Management depends on the group classification. Group four PH, chronic thromboembolic pulmonary hypertension (CTEPH) is thought to be a result of acute pulmonary emboli that cause fibrosis and scarring of the pulmonary arteries with consequent obstruction. The diagnosis of CTEPH is made by identifying perfusion abnormalities on ventilation/perfusion (V/Q) scan. Other studies required for the diagnostic evaluation include transthoracic echocardiogram, right heart catheterization, NT pro-B-type natriuretic peptide and thrombophilia evaluation. Several other tests needed to exclude other causes of pulmonary hypertension include high-resolution computed tomography (HRCT), connective tissue disease evaluation, thyroid function testing, human immunodeficiency virus testing, and liver ultrasonography to exclude portal hypertension. The treatment for CTEPH is surgical pulmonary endarterectomy (PEA). In patients who are not candidates or decline PEA, pulmonary balloon angioplasty may be useful, however, further studies are required. Several pulmonary artery hypertension medications have been studied in the management of inoperable CTEPH or persistent PH following PEA including bosentan (improves hemodynamics but not exercise capacity), macitentan (improves both hemodynamics and clinical parameters), and riociguat (improves both hemodynamics and exercise capacity). However, only riociguat is approved by the Food and Drug Administration for this indication. [ABSTRACT FROM AUTHOR]

Tian-Yu Lian, Xin Jiang, Zhi-Cheng Jing State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China Abstract: Despite advances in treatments and improved survival, patients with pulmonary hypertension still experience poor exercise and functional capacity, which has a significant detrimental impact on their quality of life. The nitric oxide (NO)–soluble guanylate cyclase (sGC)–cyclic guanosine 3',5'-monophosphate (cGMP) pathway has been shown to play an important role in cardiovascular physiology, especially in vasodilation and pulmonary vascular tone. The oral sGC stimulator riociguat has a dual mode of action on the NO–sGC–cGMP pathway: direct stimulation of sGC independent of NO and indirect simulation via sensitization of sGC to endogenous NO. Riociguat is now licensed in >50 countries worldwide, including in Europe, the USA, Canada, and Japan. Approval for the treatment of pulmonary arterial hypertension (PAH) was based on Phase III data from the PATENT studies, in which riociguat significantly improved exercise capacity, pulmonary vascular resistance, a range of secondary end points, and hemodynamic parameters in patients with symptomatic PAH. In the Phase III CHEST studies, riociguat consistently improved exercise capacity in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent/recurrent CTEPH after pulmonary endarterectomy and is now the only drug to be approved for this indication. Riociguat was well tolerated in long-term studies of PAH and CTEPH. This review describes the role of the NO–sGC–cGMP pathway in the pathophysiology of pulmonary hypertension, and reviews the clinical efficacy and safety of riociguat in patients with PAH and inoperable or persistent/recurrent CTEPH. Based on its demonstrated efficacy and established safety profile, riociguat is a promising treatment option for patients with PAH and CTEPH. Keywords: CTEPH, PAH, pulmonary hypertension, riociguat, sGC stimulator

Objective: Chronic thromboembolic pulmonary hypertension is potentially curable via pulmonary thromboendarterectomy. A minority of patients experience recurrence of their symptoms and are eligible for repeat pulmonary thromboendarterectomy. However, little data exist regarding risk factors and outcomes for this patient population., Methods: We performed a retrospective review of the University of California San Diego chronic thromboembolic pulmonary hypertension quality improvement database, including all patients who underwent pulmonary thromboendarterectomy from December 2005 to December 2020. Of the 2019 cases performed during this period, 46 were repeat pulmonary thromboendarterectomy procedures. Demographics, preoperative and postoperative hemodynamics, and surgical complications were compared between the repeat pulmonary thromboendarterectomy group and 1008 first pulmonary thromboendarterectomy group., Results: Repeat pulmonary thromboendarterectomy recipients were more likely to be younger, to have an identified hypercoagulable state, and to have higher preoperative right atrial pressure. Etiologies of recurrent disease include incomplete initial endarterectomy, discontinuation of anticoagulation (noncompliance or for medical reasons), and anticoagulation treatment failure. Patients who received repeat pulmonary thromboendarterectomy had significant hemodynamic improvement, but less pronounced compared with patients who received first pulmonary thromboendarterectomy. Repeat pulmonary thromboendarterectomy was associated with an increased risk of postoperative bleeding, reperfusion lung injury, residual pulmonary hypertension, and increased ventilator, intensive care unit, and hospital days. However, hospital mortality was similar between the groups (2.2% vs 1.9%)., Conclusions: This is the largest reported series of repeat pulmonary thromboendarterectomy surgery. Despite an increase in postoperative complications, this study demonstrates that repeat pulmonary thromboendarterectomy surgery can result in significant hemodynamic improvement with acceptable surgical mortality in an experienced center., (Published by Elsevier Inc.)

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by organized pulmonary thrombi, and pulmonary endarterectomy is the only curative treatment. Since balloon pulmonary angioplasty (BPA) has become an established therapeutic option for inoperable CTEPH, prognosis has improved. Recent reports suggest that arterial stiffness evaluated using the cardio-ankle vascular index (CAVI) may play an important role in the cardio-vascular interaction in CTEPH; however, the details remain unclear. This study aimed to clarify the role of CAVI in CTEPH through hemodynamic changes and ventricular remodeling after BPA., Methods and Results: A total of 23 patients with CTEPH who had undergone BPA were enrolled in this study. The mean pulmonary artery pressure (mPAP) and CAVI significantly decreased after BPA [mPAP, 34 (26-45) mmHg to 20 (19-24) mmHg, p  < 0.0001; CAVI, 9.4 (8.0-10.3) to 8.3 (7.5-9.6), p  = 0.004]. The echocardiographic right ventricle was significantly decreased, and the left ventricular volume was significantly increased after BPA, indicating significant biventricular remodeling after BPA. Changes in CAVI (ΔCAVI) significantly correlated with changes in mPAP ( r  = 0.45, p  = 0.03). Additionally, ΔCAVI was significantly correlated with changes in both right ventricular area and left ventricular volume., Conclusions: Arterial stiffness, evaluated using the CAVI, improved after BPA. Changes in CAVI were significantly correlated with changes in pulmonary arterial pressure and biventricular remodeling. CAVI may play an important role in cardiovascular interactions in patients with CTEPH., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (© 2023 Sato, Ito, Tabata, Ogawa, Saiki and Shimizu.)

Airway management during pulmonary thromboendarterectomy (PTE) can prove challenging, especially in the face of unexpected intraoperative pulmonary hemorrhage. Utilization of proper airway equipment on induction is crucial for the successful management of intraoperative pulmonary hemorrhage. Our case series describes the preoperative risk factors that can lead to intraoperative pulmonary hemorrhage, the preinduction airway equipment considerations for PTE, and the intraoperative management of pulmonary hemorrhage. We summarize the lessons learned at our institution from four cases of post perfusion pulmonary hemorrhage.