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BACKGROUND: Previous studies have demonstrated the safety of pulmonary endarterectomy (PEA) in all body mass index (BMI) strata. However, long-term survival and patient-reported quality of life outcome measures by BMI strata remain unknown. We sought to examine the impact of preoperative BMI on long-term survival, quality of life, and functional outcomes for patients undergoing PEA for chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: Retrospective review of 2004 patients from the UK National Cohort between 2007 and 2021 undergoing PEA for CTEPH (mPAP>20 mmHg and PVR>160 dynes). Patients were stratified into BMI<20, 20-29, 30-39, 40-49, and 50+. All-cause mortality was the primary outcome measure. Secondary outcome measures were 3-6 month postoperative hemodynamics, 6-minute walk distance (6MWD), New York Heart Association (NYHA) class, and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) scores. Kruskal-Wallis, ANOVA, and Kaplan Meier Estimate were used for comparisons. RESULTS: Postoperative hemodynamics and 6MWD at 3-6 months were similar across BMI strata. However, patients with BMI 50+ reported the highest incidence of post-operative NYHA III/IV limitation (53.3%, p<0.001) and the highest residual symptom burden by CAMPHOR (p<0.001). Overall 5-year survival was lowest in patients with BMI 50+ (70.2%) and BMI<20 (73.4%), whilst highest in BMI 30-39 (88.2%, p=0.008). 10-year Kaplan-Meier Estimates predicted lowest survival in BMI 50+ and BMI<20. CONCLUSION: PEA remains safe and effective for all patients regardless of BMI. Despite similar hemodynamic outcomes, patients with BMI 50+ are at greatest risk of long-term all-cause mortality and patients with BMI 50+ experience residual symptomatic limitation.

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a consequence of unresolved organized thromboembolic obstruction of the pulmonary arteries, which can cause pulmonary hypertension (PH) and right heart failure (RVF). Due to its subtle signs, it is challenging to determine its exact incidence and prevalence. Furthermore, CTEPH may also present without any prior venous thromboembolic (VTE) history, contributing to underdiagnosis and undertreatment. Diagnosis requires a high degree of suspicion and is ruled out by a normal V/Q scintigraphy. Additional imaging by CT and/or conventional angiography, as well as right heart catheterization are required to confirm CTEPH and formulate treatment plans. Pulmonary thromboendarterectomy (PTE) is the treatment of choice for eligible patients and can be potentially curative. PTE has a low mortality rate of 1-2% at expert centers and offers excellent long-term survival. Furthermore, recent advances in the techniques allow distal endarterectomy with comparable outcomes. There are alternative treatment options available for those who may not be operable or have prohibitive risks, providing some benefit. However, CTEPH is a progressive disease with low long-term survival rates if left untreated. Given excellent short and long-term outcomes of surgery, as well as the benefits seen with other treatment modalities in non-candidate patients, it is crucial that precapillary PH and CTEPH are ruled out in any patient with dyspnea of unexplained etiology. These patients should be referred to expert centers where accurate operability assessment, and appropriate treatment strategies can be offered by a multidisciplinary team.

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Background and objectives: Endothelin-1 (ET-1) and transforming growth factor-ß (TGF-ß) play a pivotal role in the pathophysiology and vascular remodeling of chronic thromboembolic pulmonary hypertension (CTEPH) which is an under-diagnosed complication of acute pulmonary embolism (PE). Currently, pulmonary endarterectomy (PEA) is still the treatment of choice for selected patients suffering from CTEPH. The aim of this study was to evaluate the preoperative and postoperative circulating levels of ET-1 and TGF-ß in subjects affected by CTEPH undergoing successful surgical treatment by PEA. Methods: The data from patients diagnosed with CTEPH who underwent PEA at the Foundation IRCCS Policlinico San Matteo Hospital (Pavia, Italy) were prospectively recorded in the Institutional database. Circulating ET-1 and TGF-ß levels were assessed by an ELISA commercial kit before PEA, at 3 months and 1 year after PEA. The demographic data, preoperatory mean pulmonary arterial pressure (mPAP), cardiac output (CO), and pulmonary vascular resistance (PVR) were also recorded. Univariate and multivariate analyses were performed. Results: The analysis included 340 patients with complete ET-1 measurements and 206 patients with complete TGF-ß measurements. ET-1 significantly decreased both at 3 months (p < 0.001) and at 1 year (p = 0.009) after PEA. On the other hand, preoperatory TGF-ß levels did not significantly change after PEA. Furthermore, ET-1, but not TGF-ß, was a good predictor for increased mPAP in multivariate analyses (p < 0.05). Conclusions: ET-1 but not TGF ß was significantly modulated by PEA in subjects affected by CTEPH up to 1 year after surgery. The mechanisms leading to prolonged elevated circulating TGF-ß levels and their clinical significance have to be further elucidated.

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There has been a rapid expansion in centers performing balloon pulmonary angioplasty (BPA) and pulmonary thromboendarterectomy (PTE) for chronic thromboembolic pulmonary hypertension (CTEPH). The purpose of this scoping review was to identify cardiothoracic imaging predictors of outcomes and to identify gaps to address in future work. A scoping review was conducted using the framework outlined by Arksey and O'Malley and Levac et al. in MEDLINE and EMBASE. The study protocol was preregistered in OSF Registries and performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines. There were 1117 identified studies, including 48 involving pulmonary thromboendarterectomy (n = 25) and balloon pulmonary angioplasty (n = 23). CT was the most common preoperative imaging modality used (n = 21) and CT level of disease was the most reported imaging predictor of outcomes for pulmonary thromboendarterectomy. Although must studies evaluated hemodynamic improvements, imaging was of additional use in predicting clinically significant procedural complications after balloon pulmonary angioplasty, as well as mortality and long-term outcome after pulmonary endarterectomy. Predictors reported in MRI and digital subtraction angiography were less commonly reported and warrant multicenter validation. Cardiothoracic imaging may predict clinically significant outcomes after balloon pulmonary angioplasty and pulmonary thromboendarterectomy. Radiologists involved in the assessment of CTEPH patients should be aware of key predictors and future investigations could focus on multicenter validation and new technologies.

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Balloon pulmonary angioplasty (BPA) to treat chronic thromboembolic pulmonary hypertension (CTEPH) is generally reserved for distal obstruction precluding pulmonary endarterectomy (PEA) but can be used in patients with proximal disease who are at high surgical risk or refuse surgery. This single-center retrospective study compared BPA efficacy in patients with proximal versus distal CTEPH. Of the 478 patients, 36 had proximal disease, follow-up was 11.6 months and mean number of BPA 6. After BPA, PVR, and mean pulmonary artery pressure decreased significantly in the proximal and distal groups (from 6.5 to 4.0 WU and 39 to 31 mmHg and from 7.6 to 3.8 WU and 44 to 31 mmHg, respectively, p < 0.001 for all comparisons). NYHA class also improved significantly in both groups, from 3 to 2, whereas the 6-min walk distance, cardiac output, and serum NT pro-BNP showed significant improvements only in the distal group. Thus, when PEA for CTEPH is technically feasible but not performed due to severe comorbidities or patient refusal, BPA can produce significant hemodynamic improvements, albeit less marked than in patients with distal disease. Better patient selection to BPA might improve outcomes in patients with proximal disease who are ineligible for PEA.

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Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed sequela of acute pulmonary embolism with varied clinical presentation causing significant morbidity among the affected population. There exist important differences in the occurrence, clinical features and diagnosis of CTEPH between men and women, with women carrying a greater predisposition for the disease. Ongoing studies have also pointed out variations among men and women, in the treatment offered and long-term outcomes including mortality. This focused review article highlights important sex-associated differences in multiple aspects of CTEPH including its epidemiology, clinical features, diagnosis, treatment and outcomes as reported in current literature and highlights the need for future research to facilitate a clearer understanding of these differences.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease where blood clots remain stuck in the lungs after a previous clotting episode which leads to high blood pressure in the lung arteries. One interesting aspect is that CTEPH affects men and women differently. Women show different symptoms and may have better survival rates than men, especially if they receive surgery to remove the clots. The reasons for these differences are not fully understood. Diagnosing CTEPH is challenging because its symptoms are similar to other heart and lung conditions, which can cause treatment delays. It is important to consider referring patients with possible CTEPH to specialists early for accurate diagnosis and treatment. Developing new treatments and collecting data will help improve care for these patients.

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BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension with a low incidence. Despite pulmonary endarterectomy(PEA) being the preferred treatment for CTEPH, for patients who failed medical therapy and who are not suitable candidates for PEA, lung transplantation (LT) is still the only effective treatment for end-stage CTEPH; however, there are currently very few reports on the efficacy of LT for CTEPH. METHODS: We retrospectively analyzed the clinical data of seven patients diagnosed with CTEPH between July 2019 and July 2021. The follow-up deadline was March, 2022. RESULTS: The mean age at admission was 54 ± 12 years. The average value of mean pulmonary artery pressure (mPAP) was 40 ± 5 mmHg. The mean preoperative oxygenation index(PaO2/FiO2) was 203 ± 56 mm Hg. After evaluation, one patient underwent left LT and the rest underwent bilateral LT. Three patients received intraoperative veno-venous extracorporeal membrane oxygenation (ECMO) support, and four patients received intraoperative veno-arterial ECMO support. The average postoperative mPAP was 19 ± 4 mmHg. The mean postoperative oxygenation index(PaO2/FiO2) was 388 ± 83 mmHg. There was a significant difference between the preoperative and postoperative mPAP and oxygenation index(PaO2/FiO2). All patients recovered well and were discharged 37 ± 19 days postoperatively. The mean follow-up duration was 19 ± 8 months. There was no recurrence of CTEPH. CONCLUSIONS: LT is an effective treatment for end-stage CTEPH, which can improve cardiopulmonary function and quality of life and prolong survival. Patients who are unable to tolerate PEA should be considered for LT as early as possible when internal medicine failed.

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INTRODUCTION: Balloon pulmonary angioplasty (BPA) is an effective intervention for patients with chronic thromboembolic pulmonary disease (CTEPD). We aimed to identify the patient group with a low success rate or high complication rate of BPA, which is still unclear. METHODS: Both CTEPD patients with or without pulmonary hypertension (CTEPH and NoPH-CTEPD) were included. CTEPH patients were divided into groups with or without pulmonary endarterectomy (PEA-CTEPH and NoPEA-CTEPH). The efficacy and safety of BPA were compared among the groups. RESULTS: There were 450, 66, and 41 sessions in the NoPEA-CTEPH, PEA-CTEPH, and NoPH-CTEPD groups, respectively. The success rate (≥1 degree improvement in flow grade) in the PEA-CTEPH group was 94.5%, significantly lower than that in the NoPEA-CTEPH (97.1%) and NoPH-CTEPD (98.4%) groups (p = 0.014). The percentage of complete flow recovery in treated vessels was also lower in PEA-CTEPH group. BPA-related complication rate in NoPEA-CTEPH, PEA-CTEPH, and NoPH-CTEPD patients was 6.1%, 6.0%, and 0.0%, respectively (p = 0.309). One BPA-related death occurred (solely in NoPEA-CTEPH). Mean pulmonary artery pressure ≥41.5 mm Hg was a predictor of BPA-related complications. NoPEA-CTEPH patients had more improvement in 6-min walk distance (6MWD, 87 ± 93 m NoPEA-CTEPH vs. 40 ± 43 m PEA-CTEPH vs. 18 ± 20 m NoPH-CTEPD, p = 0.012). CONCLUSIONS: BPA was safe and effective for all CTEPD groups with less improvement for the PEA-CTEPH and NoPH-CTEPD groups. The success rate of BPA was lower in the PEA-CTEPH group and the complication rate was lower in the NoPH-CTEPD group. Pre-BPA treatment to lower pulmonary artery pressure should not be overlooked in CTEPD patients.

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Objective: Pulmonary artery sarcoma (PAS) is an exceedingly rare and insufficiently investigated disease, leading to uncertain in its optimal management. This study aims to present our institutional experience and the outcomes of pulmonary endarterectomy for PAS. Methods: We gathered clinical characteristics, intraoperative data, postoperative outcomes, and prognosis information from PAS patients who underwent surgical treatment at our institution between December 2016 and September 2023. Results: A total of 20 patients with PAS underwent pulmonary endarterectomy. The median age of the patients was 52 (IQR 45, 57) years, with 12 patients (60%) being female. Intimal sarcoma was confirmed in 19 patients, while the remaining one was diagnosed with large cell neuroendocrine carcinoma. The perioperative mortality rate was three cases (15%). Follow-up was conducted for a median duration of 14 months (range: 1-61). During the follow-up period, 11 patients experienced recurrence or metastasis, and 5 patients succumbed to the disease. The estimated cumulative survival rates at 1 and 2 years for all 20 patients were 66.4% and 55.3%, respectively. Conclusion: Pulmonary endarterectomy emerges as a palliative but effective approach for managing PAS, particularly when complemented with postoperative therapies such as chemotherapy and targeted therapy, which collectively contribute to achieving favorable long-term survival outcomes.

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Background: In operable chronic thromboembolic pulmonary hypertension (CTEPH) patients, the utilization of bridging therapy with targeted medications prior to pulmonary endarterectomy (PEA) remains a topic of controversy, despite being common in cases of severe hemodynamic impairment. This study aims to assess the impact of riociguat as a bridging therapy on postoperative hemodynamics and outcomes. Methods: We conducted a retrospective study involving patients undergoing PEA from December 2016 to November 2023. Patients were categorized into two groups based on the use of riociguat before PEA. Pulmonary vascular resistance (PVR) following riociguat administration was assessed pre-PEA. Postoperative outcomes, including mortality, complications, and hemodynamics, were compared, employing propensity score matching analysis. Results: Among the patients, 41.8% (n=56) received riociguat as bridging therapy. In patients with PVR ≥800 dynes·sec·cm-5, riociguat resulted in a reduction in PVR {1,207 [974-1,698] vs. 1,125 [928-1,486] dynes·sec·cm-5, P<0.01}, while no significant difference was observed in patients with PVR <800 dynes·sec·cm-5 {641 [474-740] vs. 600 [480-768] dynes·sec·cm-5, P=0.46}. After propensity score matching, each group included 26 patients. The overall perioperative mortality rate was 2.6%. Postoperative PVR {326 [254-398] vs. 361 [290-445] dynes·sec·cm-5, P=0.35} was similar in the riociguat group compared to the control group. The incidence of residual pulmonary hypertension (PH) and other postoperative outcomes were also comparable. Conclusions: The use of riociguat as bridging therapy demonstrated hemodynamic improvement before PEA in patients with high preoperative PVR. However, no additional benefits in postoperative mortality or hemodynamics were observed.

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Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical practices remains limited. The CTEPH global cross-sectional scientific survey (CLARITY) aimed to gather information on the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the treatment and management of CTEPH patients. The survey was circulated to hospital-based medical specialists through Scientific Societies and other medical organizations from September 2021 to May 2022. The majority of the 212 respondents involved in the treatment of CTEPH were from centers performing up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures per year. Variation was observed in the reported proportion of patients deemed eligible for PEA/BPA, as well as those that underwent the procedures, including multimodal treatment and subsequent follow-up practices. Prescription of pulmonary arterial hypertension-specific therapy was reported for a variable proportion of patients in the preoperative setting and in most nonoperable patients. Reported use of vitamin K antagonists and direct oral anticoagulants was similar (86% vs. 82%) but driven by different factors. This study presents heterogeneity in treatment approaches for CTEPH, which may be attributed to center-specific experience and region-specific barriers to care, highlighting the need for new clinical and cohort studies, comprehensive clinical guidelines, and continued education.

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Chronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of patients referred to PH centers. It is a three-compartment disease involving proximal (lobar-to-segmental) and distal (subsegmental) pulmonary arteries that are obstructed by persistent fibrothrombotic material, and precapillary pulmonary arteries that can be affected as in pulmonary arterial hypertension. It is a rare complication of pulmonary embolism (PE), with an incidence of around 3% in PE survivors. The observed incidence of CTEPH in the general population is around six cases per million but could be three times higher than this, as estimated from PE incidence. However, a previous venous thromboembolic episode is not always documented. With advances in multimodality imaging and therapeutic management, survival for CTEPH has improved for both operable and inoperable patients. Advanced imaging with pulmonary angiography helps distinguish proximal from distal obstructive disease. However, right heart catheterization is of utmost importance to establish the diagnosis and hemodynamic severity of PH. The therapeutic strategy relies on a stepwise approach, starting with an operability assessment. Pulmonary endarterectomy (PEA), also known as pulmonary thromboendarterectomy, is the first-line treatment for operable patients. Growing experience and advances in surgical technique have enabled expansion of the distal limits of PEA and significant improvements in perioperative and mid- to long-term mortality. In patients who are inoperable or who have persistent/recurrent PH after PEA, medical therapy and/or balloon pulmonary angioplasty (BPA) are effective treatment options with favorable outcomes that are increasingly used. All treatment decisions should be made with a multidisciplinary team that includes a PEA surgeon, a BPA expert, and a chest radiologist.

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BACKGROUND: Assessment of the pattern of the RV outflow tract Doppler provides insights into the hemodynamics of chronic thromboembolic pulmonary hypertension (CTEPH). We studied whether pre-operative assessment of timing of the pulmonary flow systolic notch by Doppler echocardiography is associated with long-term survival after pulmonary endarterectomy (PEA) for CTEPH. METHODS: Fifty-nine out of 61 consecutive CETPH patients (mean age 53 ± 14 years, 34% male) whom underwent PEA between June 2002 and June 2005 were studied. Clinical, echocardiographic and hemodynamic variables were assessed pre-operatively and repeat echocardiography was performed 3 months after PEA. Notch ratio (NR) was assessed with pulsed Doppler and calculated as the time from onset of pulmonary flow until notch divided by the time from notch until end of pulmonary flow. Long-term follow-up was obtained between May 2021 and February 2022. RESULTS: Pre-operative mean pulmonary artery pressure (mPAP) was 45 ± 15 mmHg and pulmonary vascular resistance (PVR) was 646 ± 454 dynes.s.cm-5. Echocardiography before PEA showed that 7 patients had no notch, 33 had a NR < 1.0 and 19 had a NR > 1.0. Three months after PEA, echocardiography revealed a significant decrease in sPAP in long-term survivors with a NR < 1.0 and a NR > 1.0, while a significant increase in TAPSE/sPAP was only observed in the NR < 1.0 group. Mean long-term clinical follow-up was 14 ± 6 years. NR was significantly different between survivors and non-survivors (0.73 ± 0.25 vs. 1.1 ± 0.44, p < 0.001) but no significant differences were observed in mPAP or PVR. Long-term survival at 14 years was significantly better in patients with a NR < 1.0 compared to patients with a NR > 1.0 (83% vs. 37%, p = < 0.001). CONCLUSION: Pre-operative assessment of NR is a predictor of long-term survival in CTEPH patients undergoing PEA, with low mortality risk in patients with NR < 1.0. Long-term survivors with a NR < 1.0 and NR > 1.0 had a significant decrease in sPAP after PEA. However, the TAPSE/sPAP only significantly increased in the NR < 1.0 group. In the NR < 1.0 group, the 6-min walk test increased significantly between pre-operative and at 1-year post-operative follow-up. NR is a simple echocardiographic parameter that can be used in clinical decision-making for PEA.

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Spontaneous retroperitoneal hematoma (SRH) is a rare complication of anticoagulation therapy. Presentation may vary from limb paresis to hypovolemic shock due to blood loss. The optimal treatment is controversial. It can be managed conservatively or surgically. We report a case of a 73-year-old man presenting with progressively worsening abdominal pain and severe pain radiating to his left lower limb twenty-five days after his pulmonary endarterectomy (PEA) surgery. He was on anticoagulation per our institutional protocol for PEA patients. Investigations revealed a large, spontaneously occurring iliopsoas hematoma. Our patient was treated conservatively, and the SRH stabilised.

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Pulmonary endarterectomy (PEA) is the standard treatment for chronic thromboembolic pulmonary hypertension. However, it poses risks of perioperative vascular complications, which can lead to serious clinical outcomes. This study introduces a novel noninvasive and radiation-free clinical imaging tool, electrical impedance tomography (EIT), for real-time bedside assessment of lung perfusion after PEA. It identifies ventilation-perfusion mismatches arising from postoperative complications, particularly valuable for patients with hemodynamic instability, thus eliminating risks tied to CT room transfers. The article reports a case where EIT was used to identify an in-situ thrombosis post-PEA, marking the first such application. The emphasis is on early detection using EIT, which offers a promising approach for therapeutic interventions and improved postoperative evaluations.

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Background: Acute kidney injury (AKI) is a common and life-threatening complication following pulmonary endarterectomy (PEA). Our study aimed to investigate the risk factors associated with AKI and evaluate the correlation between serum myoglobin (sMb) levels and postoperative AKI. Methods: We conducted a retrospective study involving 134 patients who underwent PEA at China-Japan Friendship Hospital. AKI was defined and staged according to the Kidney Disease Improving Global Outcomes (KDIGO) criteria. Results: During the study period, the incidence of postoperative AKI was 57.5%, and the associated mortality rate was 6.0%. Severe AKI was found to be significantly associated with worse short-term outcomes (P<0.05). Logarithmically transformed postoperative day (POD) 0 sMb levels were significantly associated with AKI [odds ratio (OR) =5.174; 95% confidence interval (CI), 2.307-11.603; P<0.001] and severe AKI (OR =4.605; 95% CI, 1.510-14.048; P=0.007), also had independent predictive value [area under the curve (AUC) =0.776 in AKI and AUC =0.737 in severe AKI]. The optimal cut-off values were 370.544 ng/mL for AKI and 419.473 ng/mL for severe AKI. Furthermore, albumin concentration was found to play a protective role in the development of severe AKI (OR =0.838; 95% CI, 0.716-0.980; P=0.027) when higher than 40.350 g/L. Conclusions: Our findings suggest that a high concentration of POD0 sMb may increase the risk of developing AKI following PEA surgery. Increasing albumin concentration could serve as an effective preventive measure against AKI.

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La hipertensión pulmonar tromboembólica crónica (HPTEC) es una forma potencialmente curable de hipertensión pulmonar (HP) que aparece hasta en 3% de los pacientes tras una embolia pulmonar (EP). En estos pacientes, la EP no se resuelve, dando paso a coágulos fibróticos organizados, con el desarrollo de HP precapilar debido a la obstrucción proximal de las arterias pulmonares. También puede desarrollarse una microvasculopatía distal que contribuye al aumento de la resistencia vascular pulmonar (RVP). La ecocardiografía transtorácica (ETT) es la exploración que permite establecer la sospecha de HP. La gammagrafía pulmonar de ventilación-perfusión (V/Q) es la herramienta fundamental en el estudio de los pacientes con sospecha de HPTEC; si es normal, prácticamente la descarta. El cateterismo cardiaco derecho es obligatorio para el diagnóstico. La HPTEC se define como la existencia de síntomas, defectos de perfusión residuales e HP precapilar tras un periodo mínimo de tres meses de anticoagulación. La angiografía pulmonar ayuda a determinar la extensión y la accesibilidad quirúrgica de las lesiones tromboembólicas. Las personas con HPTEC son candidatas a anticoagulación indefinida. La endarterectomía pulmonar es el tratamiento de elección, resultando en una mejoría clínica y hemodinámica significativa. Aproximadamente un 25% de los pacientes presentan HP residual postendarterectomía. La angioplastia pulmonar con balón (APB) es una técnica endovascular dirigida a lesiones más distales, de utilidad para sujetos con HPTEC inoperable o HP persistente/recidivante postendarterectomía. Ambos tipos de pacientes también se pueden beneficiar de tratamiento farmacológico para la HP. Las tres terapias constituyen los pilares de la terapia, que ha evolucionado hacia un enfoque multimodal.(AU)

Chronic thrombo-embolic pulmonary hypertension (CTEPH) is a potentially curable form of pulmonary hypertension (PH) that develops in up to 3% of patients after pulmonary embolism (PE). In these patients, PE does not resolve, leading to organized fibrotic clots, with the development of precapillary PH as a result of the proximal obstruction of the pulmonary arteries. In addition, a distal microvasculopathy may also develop, contributing to the increase of pulmonary vascular resistance. Transthoracic echocardiography is the diagnostic tool that allows to establish the suspicion of PH. Ventilation-perfusion lung scintigraphy is the fundamental tool in the study of patients with suspected CTEPH; if it is normal, virtually rules out the diagnosis. Right heart catheterization is mandatory for the diagnosis of these patients. CTEPH is defined as the existence of symptoms, residual perfusion defects and precapillary PH after a minimum period of three months of anticoagulation. Pulmonary angiography helps determine the extent and surgical accessibility of thromboembolic lesions. CTEPH patients are candidates for long-term anticoagulation. Pulmonary endarterectomy is the treatment of choice, resulting in significant clinical and hemodynamic improvement. About 25% of patients have residual PH post-endarterectomy. Balloon pulmonary angioplasty is an endovascular technique that targets more distal lesions, being potentially useful for patients with inoperable CTEPH or persistent/recurrent PH post-endarterectomy. Both types of patients may also benefit from pharmacological treatment for PH. These three therapies are the cornerstone of CTEPH treatment, which has evolved towards a multimodal approach.(AU)

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Pulmonary endarterectomy (PEA) is the first-line treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, some patients with CTEPH are considered inoperable, and in the last decade, balloon pulmonary angioplasty (BPA) has emerged as a viable therapeutic option for these patients with prohibitive surgical risk or recurrent pulmonary hypertension following PEA. Numerous international centers have increased their procedural volume of BPA and have reported improvements in pulmonary hemodynamics, patient functional class and right ventricular function. Randomized controlled trials have also demonstrated similar findings. Recent refinements in procedural technique, increased operator experience and advancements in procedural technology have facilitated marked reduction in the risk of complications following BPA. Current guidelines recommend BPA for patients with inoperable CTEPH and persistent pulmonary hypertension following PEA. The pulmonary arterial endothelium plays a vital role in the pathophysiologic development and progression of CTEPH.

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To verify whether the new hemodynamic definition of pulmonary hypertension (PH) has any implication in treatment of Chronic Thrombo-Embolic Pulmonary Disease (CTEPD) patients without PH, we retrospectively analysed the clinical and functional changes determined by pulmonary endarterectomy (PEA) in 63 CTEPD patients without PH who underwent surgery at our center, comparing those in whom the hemodynamic diagnosis of PH met recent guideline recommendations versus those in whom the diagnosis only met previous hemodynamic thresholds. The results show that the vast majority of CTEPD patients without PH operated at our center would now be defined as chronic thromboembolic pulmonary hypertension (CTEPH) patients. PEA did not result in any improvement in exercise capacity nor in right ventricular function or lung function test in patients with mean pulmonary artery pressure (mPAP) ≤ 20 mm Hg and pulmonary vascular resistance (PVR) ≤ 2 WU; on the contrary, hemodynamic parameters, exercise capacity, right ventricular function and lung function significantly improved in patients with mPAP between 21 and 24 mm Hg.

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