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BACKGROUND The United States Chronic Thromboembolic Pulmonary Hypertension Registry (US-CTEPH-R) was designed to characterize the demographic characteristics, evaluation, clinical course, and outcomes of surgical and nonsurgical therapies for patients with chronic thromboembolic pulmonary hypertension. RESEARCH QUESTION What are the differences in baseline characteristics and 1-year outcomes between operated and nonoperated subjects? STUDY DESIGN AND METHODS This study describes a multicenter, prospective, longitudinal, observational registry of patients newly diagnosed (< 6 months) with CTEPH. Inclusion criteria required a mean pulmonary artery pressure ≥ 25 mm Hg documented by right heart catheterization and radiologic confirmation of CTEPH. Between 2015 and 2018, a total of 750 patients were enrolled and followed up biannually until 2019. RESULTS Most patients with CTEPH (87.9%) reported a history of acute pulmonary embolism. CTEPH diagnosis delays were frequent (median, 10 months), and most patients reported World Health Organization functional class 3 status at enrollment with a median mean pulmonary artery pressure of 44 mm Hg. The registry cohort was subdivided into Operable patients undergoing pulmonary thromboendarterectomy (PTE) surgery (n = 566), Operable patients who did not undergo surgery (n = 88), and those who were Inoperable (n = 96). Inoperable patients were older than Operated patients; less likely to be obese; have a DVT history, non-type O blood group, or thrombophilia; and more likely to have COPD or a history of cancer. PTE resulted in a median pulmonary vascular resistance decline from 6.9 to 2.6 Wood units (P < .001) with a 3.9% in-hospital mortality. At 1-year follow-up, Operated patients were less likely treated with oxygen, diuretics, or pulmonary hypertension-targeted therapy compared with Inoperable patients. A larger percentage of Operated patients were World Health Organization functional class 1 or 2 at 1 year (82.9%) compared with the Inoperable (48.2%) and Operable/No Surgery (56%) groups (P < .001). INTERPRETATION Differences exist in the clinical characteristics between patients who exhibited operable CTEPH and those who were inoperable, with the most favorable 1-year outcomes in those who underwent PTE surgery. CLINICAL TRIAL REGISTRATION ClinicalTrials.gov; No.: NCT02429284; URL: www.clinicaltrials.gov.

Objectives The aim of this study was to investigate the incidence of May-Thurner anatomy (MTA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and identify its predictors. Background MTA is an anatomical variant characterized by compression of left common iliac vein by the overlying right iliac artery. Over time, this leads to venous intimal scarring, blood flow stasis, and the development of deep vein thrombosis (DVT). DVT is a known risk factor for the development of CTEPH. The prevalence of this anatomical variation in patients with CTEPH is unknown. Methods A retrospective chart review was conducted in patients referred to Temple University Hospital’s cardiac catheterization laboratory for the evaluation of CTEPH between January 2016 and June 2020. Among these patients, those who underwent invasive venography were evaluated for the presence of angiographic MTA. Multivariate regression was used to identify factors associated with presence of MTA. Results A total of 193 patients with CTEPH were referred for pulmonary angiography, of whom 148 patients underwent invasive venography. MTA was identified in 44 patients (29.7%). Factors associated with the presence of MTA were lower extremity DVT (odds ratio: 3.5; 95% confidence interval: 1.58-7.8; P = 0.002), and left lower extremity post-thrombotic syndrome (odds ratio: 2.0; 95% confidence interval: 0.98-4.1; P = 0.05). Patients with MTA were more likely to undergo pulmonary thromboendarterectomy than patients without MTA (79.5% vs 58.7%; P = 0.015). Conclusions MTA is very common in patients with CTEPH. History of lower extremity DVT and or left lower extremity post-thrombotic syndrome was associated with the presence of MTA.

The International Consortium for Health Outcomes Measurement assembled an international working group of venous thromboembolism experts and patient representatives to develop a standardised minimum set of outcomes and outcome measurements for integration into clinical practice and potentially research to support clinical decision making and benchmarking of quality of care. 15 core outcomes important to patients and health-care professionals were selected and categorised into four domains: patient-reported outcomes, long term consequences of the disease, disease-specific complications, and treatment-related complications. The outcomes and outcome measures were designed to apply to all patients with venous thromboembolism aged 16 years or older. A measurement tool package was selected for inclusion in the core standard set, with a minimum number of items to be measured at predefined timepoints, which capture all core outcomes. Additional measures can be introduced to the user by a cascade opt-in system that allows for further assessment if required. This set of outcomes and measurement tools will facilitate the implementation of the use of patient-centred outcomes in daily practice.

Background At the University of California, San Diego, routine coronary angiography has generally been performed in men 40 years of age and older and women 45 years of age and older before pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension (CTEPH). The prevalence of significant coronary artery disease (CAD) in this population has not been evaluated, however, and the optimal screening strategy has not been established. This study sought to evaluate whether the current approach may be better optimized on the basis of cardiac risk factors. Methods This study included 462 consecutive patients with CTEPH who were undergoing preoperative coronary angiography for pulmonary thromboendarterectomy. Baseline demographic and medical information was recorded. Major cardiac risk factors included: diabetes, hypertension, hyperlipidemia, body mass index 25 kg/m2 or greater, tobacco use, and family history of CAD. Charts were then reviewed for presence of significant CAD and revascularization. Results Significant CAD was found in 13.4% of patients who underwent routine preoperative coronary angiography; it was present in only 5% of patients younger than 50 years of age, compared with 16% of patients 50 years old and older. No patient younger than 50 years of age without cardiac risk factors was found to have significant CAD. Furthermore, in patients younger than 50 years of age, significant CAD was found only among those with 3 or more major risk factors. Conclusions In patients younger than 50 years of age with CTEPH, the prevalence of significant CAD was low. Omitting preoperative coronary angiography in this subset of patients is reasonable when no coronary risk factors are present. Preoperative coronary angiography is warranted in individuals 50 years of age and older, as well as in those younger than 50 years who have significant risk factors for CAD.

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. (C) 2021 International Society for Heart and Lung Transplantation. All rights reserved.

Inflammatory pseudotumor (IPT), also known as plasma cell granuloma, is a rare lesion of unknown etiology that occurs in many organs, especially in the lung. Here we report five cases of IPT arising in pulmonary artery mimicking chronic thromboembolic disease, not previously documented in the literature. Those cases were identified at our institute among over 2500 pulmonary endarterectomy (PEA) specimens acquired from 2000 to 2017. The cohort included three men and two women with a median age of 41 years (range: 23-54). All patients presented with dyspnea and radiologic findings of pulmonary artery thromboembolism, some concerning for intimal sarcoma. The duration between disease onset and PEA ranged from 6 months to approximately 3 years. Histologically, all cases showed proliferation of spindle cells with marked inflammatory infiltrates composed predominantly of plasma cells, histiocytes, and small lymphocytes. Ancillary studies were performed in each case and ruled out other possibilities, such as sarcoma, lymphoma, plasmacytoma, IgG4-related disease, and infection. IPT arising in pulmonary artery presenting clinically as acute or chronic thromboembolic disease is very unusual, in which clinical data, radiographic findings, and histopathologic features have to be integrated for reaching the proper diagnosis.

Perioperative anticoagulation management for patients with heparin-induced thrombocytopenia requiring cardiopulmonary bypass and deep hypothermic circulatory arrest presents a clinical challenge. Alternative anticoagulants have been used but can cause significant postoperative bleeding. We report the successful use of cangrelor and heparin in a 30-year-old patient with severe heparin-induced thrombocytopenia undergoing urgent pulmonary thromboendarterectomy.

To assess the efficacy of ramelteon in preventing delirium, an acute neuropsychiatric condition associated with increased morbidity and mortality, in the perioperative, ICU setting.Parallel-arm, randomized, double-blinded, placebo-controlled trial.Academic medical center in La Jolla, California.Patients greater than or equal to 18 years undergoing elective pulmonary thromboendarterectomy.Ramelteon 8 mg or matching placebo starting the night prior to surgery and for a maximum of six nights while in the ICU.Incident delirium was measured twice daily using the Confusion Assessment Method-ICU. The safety outcome was coma-free days assessed by the Richmond Agitation-Sedation Scale. One-hundred twenty participants were enrolled and analysis completed in 117. Delirium occurred in 22 of 58 patients allocated to placebo versus 19 of 59 allocated to ramelteon (relative risk, 0.8; 95% CI, 0.5-1.4; p = 0.516). Delirium duration, as assessed by the number of delirium-free days was also similar in both groups (placebo median 2 d [interquartile range, 2-3 d] vs ramelteon 3 d [2-5 d]; p = 0.181). Coma-free days was also similar between groups (placebo median 2 d [interquartile range, 1-3 d] vs ramelteon 3 d [2-4 d]; p = 0.210). We found no difference in ICU length of stay (median 4 d [interquartile range, 3-5 d] vs 4 d [3-6 d]; p = 0.349), or in-hospital mortality (four vs three deaths; relative risk ratio, 0.7; 95% CI, 0.2-3.2; p = 0.717), all placebo versus ramelteon, respectively.Ramelteon 8 mg did not prevent postoperative delirium in patients admitted for elective cardiac surgery.

BACKGROUND AND OBJECTIVES Atrial arrhythmias (AAs) are common after cardiac surgeries including pulmonary thromboendarterectomy (PTE). This study was done to identify patients at highest risk of developing post-PTE AA and their length of stay (LOS). METHODS We reviewed 521 consecutive patients referred to University of California San Diego (UCSD) for PTE and examined their demographics as well as their baseline pulmonary hemodynamics to determine risk factors for AA. RESULTS Overall, 24.2% of patients developed an AA after PTE. Patients who developed AA had a significantly longer Intensive Care Unit (ICU) LOS (median: 5 vs 3 days, P

The aim of this study was to investigate the incidence of May-Thurner anatomy (MTA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and identify its predictors.MTA is an anatomical variant characterized by compression of left common iliac vein by the overlying right iliac artery. Over time, this leads to venous intimal scarring, blood flow stasis, and the development of deep vein thrombosis (DVT). DVT is a known risk factor for the development of CTEPH. The prevalence of this anatomical variation in patients with CTEPH is unknown.A retrospective chart review was conducted in patients referred to Temple University Hospital's cardiac catheterization laboratory for the evaluation of CTEPH between January 2016 and June 2020. Among these patients, those who underwent invasive venography were evaluated for the presence of angiographic MTA. Multivariate regression was used to identify factors associated with presence of MTA.A total of 193 patients with CTEPH were referred for pulmonary angiography, of whom 148 patients underwent invasive venography. MTA was identified in 44 patients (29.7%). Factors associated with the presence of MTA were lower extremity DVT (odds ratio: 3.5; 95% confidence interval: 1.58-7.8; P = 0.002), and left lower extremity post-thrombotic syndrome (odds ratio: 2.0; 95% confidence interval: 0.98-4.1; P = 0.05). Patients with MTA were more likely to undergo pulmonary thromboendarterectomy than patients without MTA (79.5% vs 58.7%; P = 0.015).MTA is very common in patients with CTEPH. History of lower extremity DVT and or left lower extremity post-thrombotic syndrome was associated with the presence of MTA.

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequela of acute pulmonary embolism that is treatable when recognized. Awareness of this disease has increased with recent advancements in therapeutic options, but delays in diagnosis remain common, and diagnostic and treatment guidelines are often not followed. Data gathered from international registries have improved our understanding of CTEPH, but these data may not be applicable to the US population owing to differences in demographics and medical practice patterns. Objective The US CTEPH Registry (US-CTEPH-R) was developed to provide essential information to better understand the demographics, risk factors, evaluation, and treatment of CTEPH in the United States, as well as the short- and long-term outcomes of surgical and nonsurgical therapies in the modern treatment era. Methods Thirty sites throughout the United States enrolled 750 subjects in this prospective, longitudinal, observational registry of patients newly diagnosed with CTEPH. Enrollment criteria included a mean pulmonary artery pressure ≥25 mmHg by right heart catheterization and radiologic confirmation of CTEPH by a multidisciplinary adjudication committee. Following enrollment, subjects were followed biannually until the conclusion of the study. Quality of life surveys were administered at enrollment and biannually, and all other testing was at the discretion of the treating clinician. Details regarding surgical therapy, balloon pulmonary angioplasty, and medical therapy were collected at enrollment and at follow-up, as well as information related to health care utilization and survival. Results Data from this registry will improve understanding of the demographics, risk factors, and treatment patterns of patients with CTEPH, and the longitudinal impact of therapies on quality of life, health care utilization, and survival. Conclusions This manuscript details the methodology and design of the first large, prospective, longitudinal registry of patients with CTEPH in the United States. Trial Registration ClinicalTrials.gov NCT02429284; https://www.clinicaltrials.gov/ct2/show/NCT02429284 International Registered Report Identifier (IRRID) DERR1-10.2196/25397

BACKGROUND Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequela of acute pulmonary embolism that is treatable when recognized. Awareness of this disease has increased with recent advancements in therapeutic options, but delays in diagnosis remain common, and diagnostic and treatment guidelines are often not followed. Data gathered from international registries have improved our understanding of CTEPH, but these data may not be applicable to the US population owing to differences in demographics and medical practice patterns. OBJECTIVE The US CTEPH Registry (US-CTEPH-R) was developed to provide essential information to better understand the demographics, risk factors, evaluation, and treatment of CTEPH in the United States, as well as the short- and long-term outcomes of surgical and nonsurgical therapies in the modern treatment era. METHODS Thirty sites throughout the United States enrolled 750 subjects in this prospective, longitudinal, observational registry of patients newly diagnosed with CTEPH. Enrollment criteria included a mean pulmonary artery pressure ≥25 mmHg by right heart catheterization and radiologic confirmation of CTEPH by a multidisciplinary adjudication committee. Following enrollment, subjects were followed biannually until the conclusion of the study. Quality of life surveys were administered at enrollment and biannually, and all other testing was at the discretion of the treating clinician. Details regarding surgical therapy, balloon pulmonary angioplasty, and medical therapy were collected at enrollment and at follow-up, as well as information related to health care utilization and survival. RESULTS Data from this registry will improve understanding of the demographics, risk factors, and treatment patterns of patients with CTEPH, and the longitudinal impact of therapies on quality of life, health care utilization, and survival. CONCLUSIONS This manuscript details the methodology and design of the first large, prospective, longitudinal registry of patients with CTEPH in the United States. CLINICALTRIAL ClinicalTrials.gov NCT02429284; https://www.clinicaltrials.gov/ct2/show/NCT02429284 INTERNATIONAL REGISTERED REPORT DERR1-10.2196/25397

The United States Chronic Thromboembolic Pulmonary Hypertension Registry (US-CTEPH-R) was designed to characterize the demographic characteristics, evaluation, clinical course, and outcomes of surgical and nonsurgical therapies for patients with chronic thromboembolic pulmonary hypertension.What are the differences in baseline characteristics and 1-year outcomes between operated and nonoperated subjects?This study describes a multicenter, prospective, longitudinal, observational registry of patients newly diagnosed (6 months) with CTEPH. Inclusion criteria required a mean pulmonary artery pressure ≥ 25 mm Hg documented by right heart catheterization and radiologic confirmation of CTEPH. Between 2015 and 2018, a total of 750 patients were enrolled and followed up biannually until 2019.Most patients with CTEPH (87.9%) reported a history of acute pulmonary embolism. CTEPH diagnosis delays were frequent (median, 10 months), and most patients reported World Health Organization functional class 3 status at enrollment with a median mean pulmonary artery pressure of 44 mm Hg. The registry cohort was subdivided into Operable patients undergoing pulmonary thromboendarterectomy (PTE) surgery (n = 566), Operable patients who did not undergo surgery (n = 88), and those who were Inoperable (n = 96). Inoperable patients were older than Operated patients; less likely to be obese; have a DVT history, non-type O blood group, or thrombophilia; and more likely to have COPD or a history of cancer. PTE resulted in a median pulmonary vascular resistance decline from 6.9 to 2.6 Wood units (P.001) with a 3.9% in-hospital mortality. At 1-year follow-up, Operated patients were less likely treated with oxygen, diuretics, or pulmonary hypertension-targeted therapy compared with Inoperable patients. A larger percentage of Operated patients were World Health Organization functional class 1 or 2 at 1 year (82.9%) compared with the Inoperable (48.2%) and Operable/No Surgery (56%) groups (P.001).Differences exist in the clinical characteristics between patients who exhibited operable CTEPH and those who were inoperable, with the most favorable 1-year outcomes in those who underwent PTE surgery.ClinicalTrials.gov; No.: NCT02429284; URL: www.clinicaltrials.gov.

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 Conclusions PVS is easy to detect with higher sensitivity and specificity in CTEPH compared with acute pulmonary embolism and is not a feature of pulmonary arterial hypertension. Asymmetric enhancement of pulmonary veins may serve as an additional parameter in the computed tomography assessment of CTEPH and can be used to differentiate CTEPH from pulmonary arterial hypertension.

The treatment of chronic thromboembolic pulmonary hypertension has expanded considerably. The ability to endarterectomize chronic thromboembolic material, the availability of pulmonary hypertension medical therapy to treat inoperable chronic thromboembolic pulmonary hypertension and/or residual pulmonary hypertension, and the rebirth of pulmonary balloon angioplasty have changed the management landscape. Patient selection requires a multidisciplinary evaluation at an experienced center. What is inoperable chronic thromboembolic pulmonary hypertension to one group may be operable chronic thromboembolic pulmonary hypertension to another. The ultimate challenge then becomes which intervention provides the optimal long-term outcome for any individual patient.

The past four decades have witnessed considerable advancements in our understanding of the pathophysiology, and the diagnosis and treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH). The development of an effective surgical treatment for this disease has been one of the most remarkable achievements to realize a cure for many afflicted with this form of pulmonary hypertension. For those patients with inoperable CTEPH, the past 8 years have been similarly marked by new developments. Studies demonstrating effective pulmonary hypertension-targeted medical therapy and the advancement of balloon pulmonary angioplasty (BPA) now provide patients who are not candidates for surgery beneficial treatment options. However, long-term efficacy of these treatment modalities and the selection of patients appropriate for BPA are topics of ongoing debate and research. This report provides an overview of the current, state-of-the-art therapeutic approach to the CTEPH patient.

Uterine fibroids have been described as an associate to acute venous thromboembolism (VTE), with case reports showing an association between large uterine fibroids, acute deep venous thrombosis (DVT), and acute pulmonary embolism (PE). However, there is little known about the association or causation between uterine fibroids, chronic thromboembolic disease (CTED), and chronic thromboembolic pulmonary hypertension (CTEPH). We report on six women with uterine fibroids and CTEPH, as well as one woman with CTED, all of whom presented with exertional dyspnea, lower extremity swelling, and in the cases of CTEPH, clinical, echocardiographic, and hemodynamic evidence of pulmonary hypertension and right heart failure. Compression of the pelvic veins by fibroids was directly observed with invasive venography or contrast-enhanced computed tomography in five cases. All seven women underwent pulmonary thromboendarterectomy (PTE) followed by marked improvement in functional, clinical, and hemodynamic status.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive pulmonary vascular disease with significant morbidity. It is a result of an alternate natural history in which there is limited resolution of thromboemboli with pulmonary artery obstruction leading to pulmonary hypertension (PH). CTEPH requires a thorough clinical assessment including pulmonary hemodynamics and radiologic evaluation in addition to consultation with an expert center. Surgical intervention remains the optimal management strategy. Select patients may be candidates for catheter-based intervention with balloon pulmonary angioplasty in centers with clinical expertise. Inoperable patients or those with post-intervention PH are treated with pulmonary hypertension-targeted medical therapy.

Background: Left ventricular dyssynchrony has been studied extensively, but this is not the case with right ventricular (RV) dyssynchrony. Our aim was to investigate RV dyssynchrony through 2D speckle strain imaging in patients with chronic thromboembolic pulmonary hypertension (CTEPH) before and after pulmonary thromboendarterectomy (PTE).Methods: We measured 2D peak RV strain (%) of the RV free-wall and time to peak strain (ms) of its three freewall segments (base, mid, and apex) in 127 consecutive CTEPH patients (51 ± 14 years, 59% female) with adequate images pre- and post-PTE. RV strain was calculated using Epsilon Imaging EchoInsight® software. RV dyssynchrony was measured using two methods: the standard deviation of peak systolic strain and the standard deviation of time (ms) to peak strain (RVDT) between the three RV free-wall segments.Results: Mean RV free wall strain did not change significantly after PTE (−11.4 ± 5.7 to −11.2 ± 5.6%, p = 0.67). However, RV dyssynchrony improved dramatic...

There are several mimics of chronic thromboembolic disease and CTEPH. While screening studies such as lung scintigraphy can direct attention to the pulmonary vessels as the source of a patient’s clinical presentation, there are a number of disease states that result in these nonspecific radiographic findings. Historical clues, subtle pulmonary angiographic findings and advanced pulmonary vascular and lung imaging can be critically important in establishing the correct diagnosis, as illustrated in the case presented here.

The diagnosis of pulmonary veno-occlusive disease can be very challenging. Although lung ventilation-perfusion scintigraphy is typically normal in this disease, an abnormal scan can be observed in patients with PVOD. This case presentation illustrates this point, and how the abnormal lung scan and pulmonary angiogram was initially interpreted as CTEPH in this 23 year old male.

That CT angiography demonstrates evidence for chronic thrombus while the perfusion pattern on lung scintigraphy is normal can be readily explained. This case will demonstrate this “discrepancy” and underscores the value of a normal VQ scan in ruling out operable chronic thromboembolic disease.

Treatment options for patients with inoperable chronic thromboembolic pulmonary hypertension have expanded with the availability of balloon pulmonary angioplasty and pulmonary hypertension targeted medical therapy. Often, both options are appropriate for the provision of care for those patients who are not deemed candidates for PTE surgery. As illustrated by this case, the task of selecting patients suitable for balloon pulmonary angioplasty can be a difficult one, typically requiring input from diagnosticians, radiologists, and surgeons experienced in the evaluation and treatment of patients with CTEPH.

While pulmonary hypertension, an abnormal VQ scan and angiographic evidence of thrombus in the pulmonary arteries are necessary to diagnose chronic thromboembolic pulmonary hypertension, an atrial septal defect with Eisenmenger’s syndrome may present with similar findings. Clues to the presence of an ASD include digital clubbing and scintigraphic evidence of right-to-left shunt with uptake of technetium macro aggregated albumin seen in the brain and kidneys. An echocardiogram with bubble study can also demonstrate a right-to-left shunt. Patients with Eisenmenger’s syndrome should have pulmonary hypertension medically managed. There is no benefit to pulmonary thromboendarterectomy for chronic in situ thrombosis frequently seen in these patients.

The importance of establishing the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) centers on the observation that with a thromboendarterectomy, this pulmonary vascular disease is potentially curable. As a result, a careful assessment of patients at-risk for or suspected of having CTEPH is essential, and this case presentation illustrates an appropriate approach for such an evaluation.

Pulmonary thromboendarterectomy remains the best option for patients with chronic thromboembolic pulmonary hypertension as a potential cure for their disease. With advances in this surgical procedure, endarterectomy of the more distal pulmonary vascular bed has become possible. This case provides an example of the clinical benefits that can be achieved with distal vessel thromboendarterectomy.

There are a number of clinical conditions that mimic the radiographic findings in CTEPH, but few are as difficult to distinguish from CTEPH as pulmonary arteritis. However, as the presented case will demonstrate, there are often hints in the evaluation of patients with pulmonary hypertension to suggest large vessel arteritis as the principal diagnosis.

Perfusion defects on standard lung scintigraphy can be due to many causes, though typically from occlusion or partial obstruction of the pulmonary arteries. This case presentation highlights that outflow obstruction due to large pulmonary venous disease may result in perfusion abnormalities, and should be considered in the differential when a clinician is presented with an abnormal lung ventilation-perfusion scan.

The transition from acute to chronic thromboembolic disease can occur to an unpredictable degree in patients who survive an acute pulmonary embolism. In the vast majority of such patients on anti-thrombotic therapy, near complete resolution of the thrombus occurs. In 2–3% of patients, chronic thromboembolic pulmonary hypertension will develop. However, in an unknown percentage of pulmonary embolic survivors, organized thrombotic residua can involve enough of the pulmonary vascular bed to cause cardiopulmonary symptoms in the absence of pulmonary hypertension or right heart dysfunction. This is referred to as chronic thromboembolic disease or CTED, and the case presentation to follow illustrates some of challenges in treatment recommendations for this unique disease.

Establishing the diagnosis and operability of chronic thromboembolic disease in patients with pulmonary hypertension are the critical first steps in determining surgical candidacy for thromboendarterectomy. However, whether or not a CTEPH patient is ultimately a surgical candidate requires a complete overview of comorbidities, a realistic prediction that a PTE will result in a clinical benefit, and patient acceptance. This case presentation explores the decision-making challenges often faced during an evaluation of patients with chronic thromboembolic disease and multiple comorbidities.

The distinction between acute and chronic thromboembolic disease is frequently difficult at the time of initial presentation. However, the importance in making this distinction is underscored by the considerable differences in treatment strategies required for these clinical problems. Not only are there historical clues, but an understanding of the disparate radiographic appearance between acute and chronic thrombi can often prevent an inappropriate intervention. This case presentation highlights these important considerations.

A case of apparent chronic thromboembolic pulmonary hypertension that surprisingly exhibited metastatic squamous cell carcinoma embedded in the organized thrombus endarterectomized from the proximal pulmonary vessels.

Patients with distal CTEPH remain among the most challenging to manage. This case illustrates the importance of screening all pulmonary hypertensive patients for CTEPH, and the value of correctly identifying patients with distal vessel chronic thromboembolic disease where a cure with thromboendarterectomy is feasible.

Lung scintigraphy is an invaluable screening tool in the evaluation of the pulmonary hypertensive patient. The absence of perfusion defects is properly interpreted to exclude occlusive pulmonary vascular disease such as CTEPH. However, unmatched perfusion defects are due to a number of disease states, and as illustrated with this presented case, extensive precapillary, small vessel pulmonary vascular disease needs to be considered among the possibilities.

Large-vessel pulmonary artery sarcomas are rare tumors of the pulmonary vasculature, accounting for only a fraction of primary lung cancers and with a very poor prognosis. They arise proximally from within the pulmonary artery and spread within the arterial lumen, causing obstruction of the vasculature that may result in right ventricular failure. Although they may have a similar presentation to chronic thromboembolic pulmonary disease and CTEPH, there are subtle indicators in history and imaging that can help with their differential diagnosis, with the confirmation usually resulting from tissue biopsy. This case presentation focuses on this CTEPH mimic.

Perfusion defects on ventilation-perfusion scan and eccentric thrombus lining major pulmonary vessels on CT pulmonary angiography in a patient with severe chronic obstructive airways disease and pulmonary hypertension may not represent CTEPH. This case presentation emphasizes the diagnostic treatment challenges in an emphysema patient with CT evidence for chronic thrombus.

Main pulmonary artery occlusion from chronic thromboembolic disease can rarely be the cause for non-perfusion of an entire lung. Careful evaluation of alternative and more common causes for this finding is essential. For those patients with chronic thromboembolic disease, thromboendarterectomy may result in lung reperfusion. However, the achievement of this outcome may be impacted by the extent of collateral systemic blood flow and duration of main pulmonary artery occlusion.

Chronic thromboembolic disease can lead to significant exercise limitations even in the absence of pulmonary hypertension. The decision to proceed with pulmonary thromboendarterectomy is complex, but as in this case, can lead to significant improvements in lung perfusion and exercise tolerance. Though speculative, intervention at this stage of the disease may also prevent the development of significant pulmonary hypertension.