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Introduction: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) and decompensated right heart failure (DRHF) have worse outcomes after pulmonary endarterectomy (PEA). We reviewed the role of central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge to recovery after PEA in these patients., Methods: Of 388 consecutive patients undergoing PEA, 40 (10.3%) were admitted with DRHF before PEA. This group was compared to the remaining 348 patients undergoing PEA (elective group). We also compared 2 periods: 2005-2013 (n = 120) and 2014-2019 (n = 268) after which early central VA-ECMO was introduced as a strategy to manage difficulty weaning from cardiopulmonary bypass (CPB)., Results: The proportion of patients with DRHF remained similar between the first and second period (13% vs 9%, p = .2). The number of VA-ECMO bridge to recovery increased from 0.8% in 2005-2013 to 6.3% in 2014-2019 (p = .02). In the second period, 29% of DRHF patients were transitioned intraoperatively from CPB to central VA-ECMO for a median duration of 3 (2-7) days. After the introduction of central VA-ECMO as a bridge to recovery, the hospital mortality in patients with DRHF dropped from 31% in 2005-2013 to 4% in 2014-2019 (p = .03). In the long-term, the functional recovery and survival after discharged from hospital was similar between the DRHF group and the elective group. However, at 5 years, DRHF patients more frequently required PH targeted medical therapy (45% vs 20% in the elective group, p = .002)., Conclusions: Central VA-ECMO as a bridge to recovery is an important treatment strategy that can decrease hospital mortality in patients with DRHF and lead to excellent long-term outcome., (Copyright © 2022 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary endarterectomy (PEA) is a curative procedure for patients with chronic thromboembolic pulmonary hypertension. Body composition and exercise capacity have been associated with adverse outcomes in patients undergoing cardiothoracic operations, but their significance with PEA is unclear. We evaluated the association of body composition and 6-minute walk distance (6MWD) with disease severity, hospital length of stay, discharge disposition, and postoperative functional recovery., Methods: This was a retrospective, single-center cohort study of patients who underwent PEA (January 2014-December 2017). Body composition (skeletal muscle mass and adiposity cross-sectional area) was quantified using thoracic computed tomography with sliceOmatic (TomoVision, Magog, QC, Canada) software. Body mass index was calculated. Association of body composition measures and 6MWD with clinical outcomes was evaluated using multivariable regression models., Results: The study included 127 patients (42% men), aged 58 ± 14 years; body mass index was 31 ± 7 kg/m 2 and 6MWD was 361 ± 165 m). Muscle and 6MWD were associated with disease severity measures. Of those surviving hospitalization (n = 125), a greater 6MWD was associated with a shorter hospital stay (1.9 median days per 100 m; p < .001) and higher likelihood of being discharged directly home from hospital (odds ratio, 2.1 per 100 m; P = .004), independent of age, sex, and body mass index. Those with a lower preoperative 6MWD (per 100 m) had a greater increase in their postoperative 6MWD (52 m; P < .0001), independent of age, sex, and body mass index. Body composition measures were not associated with hospital outcomes or exercise capacity in the first year postoperatively., Conclusions: Exercise capacity was a more prognostic marker of PEA outcomes compared with body composition. Future research is needed to explore pre-PEA rehabilitation strategies., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Pulmonary endarterectomy is the treatment of choice for chronic thromboembolic pulmonary hypertension. This case report outlines the importance of venoarterial extracorporeal membrane oxygenation and plasmapheresis as two important options in the management of heparin-induced thrombocytopenia-positive patients requiring urgent pulmonary endarterectomy., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is still largely underdiagnosed in the general population. Although transthoracic echocardiogram (TTE) is recommended to screen for CTEPH, it may not detect patients with chronic thromboembolic disease (CTED) and mild or exercise-induced pulmonary hypertension (PH) who could also benefit from pulmonary endarterectomy (PEA)., Methods: All patients referred to our CTEPH program with persistent mismatched perfusion defects on ventilation-perfusion (VQ) scan between January 2005 and June 2015 were divided into three groups according to TTE and right heart catheterization (RHC) as follows: (1) typical CTEPH group (PH on RHC and TTE), (2) TTE-negative (neg) CTEPH group (PH on RHC, but not TTE), or (3) CTED group (no PH on RHC and TTE)., Results: Of 225 patients with abnormal VQ scans, 188 (84%) had typical CTEPH, 15 had TTE-neg CTEPH, and 22 had CTED. PEA was performed in 179 patients (80%). Reasons for exclusion in CTEPH patients included primarily distal disease (n = 11) and comorbidities (n = 10). In contrast, the absence of functional limitation was the main cause of exclusion in CTED patients (75% versus 3% in CTEPH patients, p < 0.0001). The 90-day mortality rate after PEA was 4% in the typical CTEPH group and 0% in the TTE-neg CTEPH and CTED groups. Pulmonary arterial pressures and functional class significantly improved after PEA in all three groups., Conclusions: Patients with mild CTEPH can benefit from PEA, but may not be detected by TTE. Symptomatic patients with functional limitation and persistent mismatched perfusion defects on VQ scan should undergo further investigations with pulmonary angiogram and RHC., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Extracorporeal life support (ECLS) for rescue after pulmonary endarterectomy (PEA) has become a viable option. This study aims to present a single-centre experience looking at the indications and outcome of ECLS after PEA., Methods: Retrospective analysis of all patients undergoing PEA from January 2008 to January 2015 in our institution., Results: Among 144 consecutive patients undergoing PEA for chronic thromboembolic pulmonary hypertension, 6 (4%) received ECLS postoperatively for right ventricular (RV) failure (n = 3), severe hypoxaemia (n = 2) and haemorrhagic pulmonary oedema (n = 1). ECLS configuration was central veno-arterial (cVA) in 3 patients, peripheral VA (pVA) in 1 and veno-venous (VV) in 2. One patient with cVA was switched to VV after 5 days. Overall ECLS duration ranged between 3 and 39 (median 5) days. ECLS patients had higher preoperative total pulmonary vascular resistance (TPR) compared with non-ECLS patients (1477 ± 671 vs 954 ± 462 Dynes.s.cm(-5), P = 0.009) and more frequently required hospital admission for RV failure before surgery (50 vs 9%, P = 0.02). The overall in-hospital mortality rate for all patients was 2% (3/144), including one ECLS patient on pVA. The remaining 5 ECLS patients (83%) were discharged from the hospital and are alive after a median follow-up of 11 (range 6-27) months. Two ECLS patients (40%) are on therapy for residual PH compared with 13 (10%) in the non-ECLS patients (P = 0.09)., Conclusions: ECLS is a safe and important rescue option after PEA. The use of ECLS may expand eligibility for PEA by allowing sicker patients to undergo surgery., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: The outcome of patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) after urgent hospitalization for decompensated right heart failure (DRHF) remains unclear., Methods: Among 120 consecutive patients undergoing PEA, 16 (13%) presented with a history of urgent hospitalization for DRHF (severe RHF group). This group was compared with the remaining 104 patients presenting with total peripheral vascular resistance (TPR) < 1,200 dynes · sec · cm(-5) (n = 78, control group) and >1,200 dynes · sec · cm(-5) (n = 26, high TPR group)., Results: DRHF occurred predominantly in patients with TPR > 1,200 dynes · sec · cm(-5) (prevalence of 32% vs 5% in patients with TPR < 1,200 dynes · sec · cm(-5), p < 0.0001). The overall in-hospital mortality after PEA was 4% (n = 5). All deaths occurred in patients with TPR > 1,200 dynes · sec · cm(-5) and DRHF. The proportion of patients with residual PH immediately after surgery was higher in the high TPR group (50%) and severe RHF group (56%) compared with the control group (29%; p = 0.04). In multivariate analysis, risk factors for residual PH after surgery were TPR > 1,200 dynes · sec · cm(-5), Jamieson disease Type III-IV, and female gender. A history of DRHF by itself was not a risk factor for residual PH after PEA. The overall 5-year survival was 87%. After a median follow-up of 20 months, the proportion of patients receiving medical therapy for residual PH was higher in patients with post-operative mean pulmonary artery pressure ≥35 mm Hg (61% vs 9%, p = 0.0007)., Conclusions: DRHF occurs more frequently in patients with TPR > 1,200 dynes · sec · cm(-5), increasing the operative risk in these patients. The outcome of patients with high TPR in the absence of DRHF is excellent. However, patients with residual mean pulmonary artery pressure ≥35 mm Hg frequently receive pulmonary hypertension therapy after PEA., (Copyright © 2015 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (PH). However, this surgery remains performed in few experienced centres only. The goal of the study is to review our overall experience since the implementation of our program in August 2005., Methods: Review all patients referred to our program between August 2005 and July 2011., Results: Among 84 consecutive patients referred to our program, 52 patients underwent elective PEA and 6 emergency PEA. After PEA, 74% patients were extubated within 2 days, 71% were discharged from the intensive care unit within 4 days and 64% were discharged from hospital within 15 days. One patient undergoing elective surgery and 2 patients undergoing emergency surgery died within 30 days of surgery for an operative mortality of 1.9% after elective pulmonary endarterectomy and an overall operative mortality of 5.2%, when the 6 emergency operative cases were included. The total pulmonary vascular resistance decreased from 965±445 to 383±162 dynes per second per cm(-5) and was associated with significant improvement in World Health Organization/New York Heart Association (WHO/NYHA) functional class, 6 minutes walk distance, echocardiographic findings, and brain natriuretic peptide level at 6 months after PEA. After a median follow-up of 23 months (1-65 months), 3 patients had to be started on targeted PH therapy for deterioration of their (WHO/NYHA) functional class., Conclusions: Elective PEA can be performed with limited risk, and results in excellent early and long-term outcome. All patients diagnosed with chronic thromboembolic PH should be referred for consideration of PEA in a specialized centre., (Copyright © 2011 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Despite a major reduction in pulmonary vascular resistance (PVR), patients with chronic thromboembolic pulmonary hypertension (CTEPH) do not always return to functional New York Heart Association (NYHA) class I after pulmonary endarterectomy (PEA). We hypothesized that residual abnormal compliance (Cp) after PEA is associated with incomplete functional recovery despite major improvement in PVR., Methods: The Cp of 34 consecutive patients with CTEPH was assessed before and after PEA. Cp was defined as stroke volume over pulse pressure and was divided into three groups: < 2.0 mL/mm Hg, 2.0 to 3.9 mL/mm Hg, and ≥ 4 mL/mm Hg. To establish predicted Cp after PEA, we collected an age- and gender-matched control group., Results: Before PEA, Cp was < 2.0 mL/mm Hg in 82% (n = 28) of the patients. After PEA, Cp improved to 2.0 to 3.9 mL/mm Hg in 11 patients and to ≥ 4.0 mL/mm Hg in 14 patients. Residual Cp < 2.0 mL/mm Hg was associated with delayed extubation and prolonged hospital stay. At 3 months' follow-up, 13 patients (93%) with postoperative Cp ≥ 4.0 mL/mm Hg returned to NYHA class I, whereas 45% with Cp of 2.0 to 3.9 mL/mm Hg and 25% with Cp < 2.0 mL/mm Hg returned to NYHA class I. In multivariate analysis, postoperative Cp ≥ 4.0 mL/mm Hg was the only predictor of functional recovery to NYHA class I. The group of patients with postoperative Cp ≥ 4.0 mL/mm Hg was also the only group to achieve hemodynamic parameters similar to those of their matched control subjects., Conclusions: Postoperative Cp is an important predictor of recovery after PEA. Residual vascular stiffness after PEA can be associated with persistent functional limitation and lack of complete remodeling of the right ventricle.

Blood transfusion requirements were reviewed for a consecutive series of 25 patients undergoing elective pulmonary endarterectomy (PEA) between August 2005 and March 2009 in our institution. Patients were divided into two groups based on the implementation of a conservative blood transfusion algorithm that combined antifibrinolytic therapy, intraoperative blood sequestration, blood salvage and lack of correction of coagulation parameters in the absence of ongoing bleeding. Despite similar perioperative coagulation profiles in the two groups, the introduction of a conservative blood transfusion algorithm was associated with a significant increase in the number of patients receiving no homologous blood products. Of 16 patients who underwent surgery after the introduction of the algorithm, nine (56%) required no homologous blood products and five (31%) required one or two units of homologous red blood cells only. The international normalized ratio normalized within six to 12 hours after discontinuation of cardiopulmonary bypass without transfusion of fresh frozen plasma or platelets in 13 of the 16 patients. In conclusion, a conservative blood transfusion strategy allows PEA to be safely performed with no or minimal blood product transfusions in a majority of patients despite deep hypothermic circulatory arrest.

Purpose: To perform a systematic review and meta-analysis assessing the safety and efficacy of balloon pulmonary angioplasty (BPA) in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Materials and Methods: Systematic literature searches were performed from inception to June 2022 to identify studies assessing BPA for CTEPH. Outcomes of interest included the following functional and hemodynamic measures: (a) six-minute walk distance (6MWD), (b) New York Heart Association (NYHA) status, (c) World Health Organization (WHO)-Functional Class status, (d) cardiac index (CI), (e) mean pulmonary artery pressure (mPAP), (f) mean right atrial pressure (mRAP), and (g) pulmonary vascular resistance (PVR). Subgroup analysis was also performed for BPA in post-pulmonary endarterectomy (PEA) patients. All reported BPA-related complications were also recorded. Forty unique studies with a total of 1763 patients were identified for meta-analysis. Results: All functional and hemodynamic parameters improved significantly following BPA; 6MWD increased 70 m (95% CI 58–82; P < 0.001), NYHA class improved by − 0.9 classes (95% CI − 1.0 to − 0.8; P < 0.001), WHO-FC class improved by − 1 classes ((95% CI − 1.2 to − 0.9; P < 0.001), CI increased 0.26 L/min/m2 (95% CI 0.17–0.35; P < 0.001), mPAP decreased − 13.2 mmHg (95% CI − 14.7 to − 11.8; P < 0.001), mRAP decreased − 2.2 mmHg (95% CI − 2.8 to − 1.6; P < 0.001), and PVR decreased − 311 dyne/cm/s−5 (95% CI − 350 to − 271; P < 0.001). Meta-analysis of patients who underwent BPA for persistent pulmonary hypertension post-PEA demonstrated significant improvements in 6MWD, WHO-FC, PVR and mPAP. Most common complications included lung injury (8.16%), hemoptysis (7.07%) and vessel injury (5.05%). Conclusion: BPA represents a safe and effective treatment option for select individuals with CTEPH with significant improvements in hemodynamic parameters, improved exercise tolerance and a relatively low risk of major complications. [ABSTRACT FROM AUTHOR]

The article cites a study on the assessment of persistent pulmonary hypertension after acute pulmonary embolism (PE). It found that after the median of 18 weeks of anticoagulation, 17 of 10 patients showed residual pulmonary artery systolic pressure (PAsP) and eight were determined to be suitable candidates for pulmonary endarterectomy (PEA). It implied that the first group should be evaluated despite the absence of PE and the second group are at risk and must be monitored.

The impact of sex on long-term outcomes after pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (PH) remains unclear. We therefore examined the early and long-term outcome after PEA to determine whether sex had an impact on the risk of residual PH and need for targeted PH medical therapy. Retrospective study of 401 consecutive patients undergoing PEA at our institution between August 2005 and March 2020 was performed. Primary outcome was the need for targeted PH medical therapy postoperatively. Secondary outcomes included survival and measures of hemodynamic improvement. Females (N = 203, 51%) were more likely to have preoperative home oxygen therapy (29.6% vs 11.6%, p < 0.01), and to present with segmental and subsegmental disease compared to males (49.2% vs 21.2%, p < 0.01). Despite similar preoperative values, females had higher postoperative pulmonary vascular resistance (final total pulmonary vascular resistance after PEA, 437 Dynes∙s∙cm−5 vs 324 Dynes∙s∙cm−5 in males, p < 0.01). Although survival at 10 years was not significantly different between sexes (73% in females vs 84% in males, p = 0.08), freedom from targeted PH medical therapy was lower in females (72.9% vs 89.9% in males at 5 years, p < 0.001). Female sex remained an independent factor affecting the need for targeted PH medical therapy after PEA in multivariate analysis (HR 2.03, 95%CI 1.03–3.98, p = 0.04). Although outcomes are excellent for both sexes, females had greater need for targeted PH medical therapy in the long-term. Early reassessment and long-term follow-up of these patients are important. Further investigations into possible mechanisms to explain the differences are warranted. [ABSTRACT FROM AUTHOR]