Pulmonary Endarterectomy Surgery Research Articles

The impact of right ventricular energy failure on the results of pulmonary endarterectomy and balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension

Objective: We aimed to investigate the effect of right ventricular energy failure (RVEF) on hemodynamic and clinical outcomes in patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) surgery or balloon pulmonary angioplasty (BPA). Patients and Methods: A total of 100 CTEPH patients planned for PEA or BPA were included in the study. Based on the presence of RVEF during diagnosis, patients divided into two groups. Hemodynamic data from right heart catheterization (RHC) were compared before and after procedures in 3-6 months follow up period. Results: Patients with RVEF revealed a decrease in mean pulmonary artery pressure (mPAP) from 54.67±12.27 mmHg to 36.12±11.76 mmHg (p:<0.001), mean right atrial pressure (mRAP) from 13.40±4.08 mmHg to 9.76±4.56 mmHg (p:0.003), and pulmonary vascular resistance (PVR) from 11.36±5.15 Wood Units (WU) to 5.46±3.30 WU (p <0.001). In the non-RVEF group, mPAP decreased from 38.82±12.61 mmHg to 30.81±10.57 mmHg (p:<0.001), mRAP from 7.09±3.02mmHg to 7.15±3.07mmHg (p: 0.917), and PVR from 6.33±3.65 WU to 4.09±2.31 WU (p:<0.001). Conclusion:The presence of RVEF at the time of diagnosis in CTEPH patients does not have a negative impact on early perioperative and 3-month postoperative outcomes following PEA or BPA. This high-risk patient group demonstrated significant hemodynamic and clinical benefits from both PEA and BPA.

Spontaneous retroperitoneal haemorrhage after pulmonary endarterectomy surgery

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.

Risk factor and correlation between postoperative serum myoglobin and acute kidney injury after pulmonary endarterectomy.

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. 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. 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. 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.

Hyperlactatemia in patients undergoing pulmonary endarterectomy with deep hypothermic circulatory arrest: Risk factors and its effects on the outcome.

To determine the risk factors of hyperlactatemia in pulmonary endarterectomy (PEA) surgery and assess whether elevated blood lactate levels are associated with adverse outcomes. In this retrospective observational study, a total of 111 consecutive patients who underwent PEA for chronic thromboembolic pulmonary hypertension at the XXX Hospital between December 2016 and January 2022 were included. We retrospectively evaluated arterial blood samples analyzed intraoperatively. The pre- and intraoperative risk factors for hyperlactatemia and the postoperative outcomes were recorded. Lactate levels gradually increased during surgery. The optimal cut-off lactate level for major postoperative complications, calculated using receiver operating characteristic analysis, was 7.0mmol/L. Deep hypothermic circulatory arrest (DHCA) duration, nadir hematocrit, and preoperative pulmonary vascular resistance were risk factors for lactate levels >7mmol/L. Moreover, the intraoperative peak lactate level during PEA under DHCA was found to be a statistically significant predictor of major complications being associated with longer mechanical ventilation time (r = 0.294; p = .003) and intensive care unit length of stay (r = 0.327; p = .001). Deep hypothermic circulatory arrest duration, nadir hematocrit, and preoperative pulmonary vascular resistance were associated with hyperlactatemia. Increased lactate levels were independent predictors of longer mechanical ventilation time, intensive care unit length of stay, and major complications.

A Health State Utility Study to Elicit Societal Values Associated with Pulmonary Hypertension.

Pulmonary hypertension (PH) is a rare, severe, and progressive pulmonary vascular disease, which includes five subgroups with similar presentation. Symptoms include dyspnea, and fatigue, and can significantly impact one's health-related quality of life (HRQL). Although treatments are mainly medical, PH group 4, chronic thromboembolic pulmonary hypertension (CTEPH), can be managed with procedures, ie pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA). Currently, drugs in Europe are only approved for pulmonary arterial hypertension (PAH), and CTEPH therefore the aim of this study was to elicit novel societal health state utilities in the UK for PAH and CTEPH based on disease severity, functional class (FC), clinical events, and treatment procedures specifically for CTEPH. Six health states were defined: World Health Organization (WHO)-FC II, WHO-FC III, and WHO-FC IV [defined by the New York Heart Association (NYHA)]; PH-related hospitalization; and "BPA procedure and recovery" and "PEA surgery and recovery". Health states were based on a targeted literature review and two rounds of interviews with clinical experts (N = 4) and patients (N = 6). Draft health states were validated in cognitive debriefing interviews with clinical experts (N = 3). Health states were valued by the UK general public (n = 200), using a visual analogue scale (VAS) and time trade-off (TTO) assessment with the lead time method. The mean TTO values/utilities were 0.81 (FC II), 0.80 (BPA), 0.78 (PEA), 0.59 (FC III), 0.28 (FC IV), and 0.25 (PH-related hospitalization). Each progression in FC was associated with worse TTO scores. This study reports societal utility values for PAH and CTEPH in the UK. It provides first utility estimates for states such as BPA procedure and recovery, PEA surgery and recovery and PH-related hospitalization for this population. The results show important distinctions between FC, treatment procedures, and hospitalization, and the significant burden of disease on HRQL.

Balloon pulmonary angioplasty outcomes in patients previously treated by pulmonary endarterectomy surgery are inferior to those of inoperable patients

AbstractPulmonary endarterectomy (PEA) may not achieve full clearance of vascular obstructions in patients with more distal chronic thromboembolic pulmonary hypertension (CTEPH). Balloon pulmonary angioplasty (BPA) may be indicated to treat these residual vascular lesions. We compared whether patients post‐PEA (PP) treated by BPA derived similar benefit to those who had inoperable CTEPH (IC), and assessed predictors of BPA response after surgery. We treated 109 patients with BPA—89 with IC and 20 PP. Serial right heart catheterization performed at baseline (immediately before BPA) and 3 months after completing BPA, compared pulmonary vascular resistance (PVR), mean pulmonary artery pressure (mPAP) as well as change in WHO functional class and 6‐minute walk distance. We also assessed the impact of total thrombus tail length (TTTL) from photographed PEA surgical specimens and PP computed tomography pulmonary angiography (CTPA)‐quantified residual disease burden on BPA response. PP and IC groups did not differ significantly in terms of demographics, baseline hemodynamics or procedural characteristics. However, IC derived greater hemodynamic benefit from BPA: ΔPVR (−27.9 ± 20.2% vs. −13.9 ± 23.9%, p &lt; 0.05) and ΔmPAP (−17.1 ± 14.4% vs. −8.5 ± 18.0%, p &lt; 0.05). There was a negative correlation between pre‐BPA PVR and TTTL (r = −0.47, p &lt; 0.05) which persisted post‐BPA. PVR, mPAP, WHO FC and 6MWD were not improved significantly post‐BPA in PP patients. BPA response was not related to TTTL terciles or CTPA‐quantified residual disease burden. Patients PP experienced inferior response to BPA, despite similar baseline and procedural characteristics to IC. BPA does not abolish the relationship between TTTL and postsurgical PVR in PP patients, suggesting that BPA is less effective in treating residual PH after surgery in an experienced surgical center.

Progress in the treatment of acute pulmonary embolism and chronic thrombo-embolic pulmonary hypertension/disease.

The combination of an initial clinical approach aimed at evaluating the early risk of mortality with subsequent diagnostic and therapeutic approaches articulated on the overall patient's profile is recommended in acute pulmonary embolism (PE). The presence of pulmonary hypertension associated with the persistence of chronic vascular obstructions in the pulmonary arteries after one or more acute thrombo-embolic events identifies a condition defined as chronic thrombo-embolic pulmonary hypertension (CTEPH). The evolution of technology and knowledge in the field of imaging has allowed us to qualify the computed tomography angiography of the pulmonary arteries as the gold standard for the diagnostic confirmation of both acute PE and CTEPH. In both these conditions, the first therapeutic step is the immediate initiation of anticoagulant therapy. In acute high-risk PE, in addition to anticoagulant therapy, thrombolytic therapy is recommended; in the event of contraindications to thrombolysis, surgical embolectomy or percutaneous catheter-directed treatment represents viable treatment options. In CTEPH, the combination of data collected from cardiac catheterization, computed tomography angiography, and conventional angiography of pulmonary arteries allows a team of experts to identify candidates for pulmonary endarterectomy surgery. Inoperable patients should be considered for percutaneous balloon angioplasty of the pulmonary arteries which can improve patients' symptoms, quality of life, and prognosis.

Distribution of thrombus predicts severe reperfusion pulmonary edema after pulmonary endarterectomy

ObjectivesPatients underwent pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to investigate the effect of thrombus distribution on the occurrence of severe reperfusion pulmonary edema (RPE) and identify specific parameters for predicting severe RPE. MethodsPatients with CTEPH who underwent PEA surgery were retrospectively analyzed. The thrombus in pulmonary arteries were evaluated through computed tomography pulmonary angiography. Based on presence of prolonged artificial ventilation, extracorporeal membrane oxygenation required, or perioperative death due to RPE, the patients were divided into the severe RPE and without severe RPE groups. Main resultsAmong the 77 patients (29 women), 16 (20.8%) patients developed severe RPE. The right major pulmonary artery (RPA) (0.64[0.58, 0.73] vs 0.58[0.49, 0.64]; p = 0.008) and pulmonary artery trunk (PAT) thrombus ratios (0.48[0.44, 0.61] vs 0.42[0.39, 0.50]; p = 0.009) (the PAT ratio is expressed as the sum of the right middle lobe clot burden and right lower lobe clot burden divided by the total clot burden multiplied by 100) of the severe RPE group was significantly higher than that of the without severe RPE group. Receiver operator characteristics curve identified a PAT ratio of 43.4% as the threshold with areas under the curve = 0.71(95%CI 0.582; 0.841) for the development of severe RPE (sensitivity 0.875, specificity 0.541). The logistic regression analysis demonstrated that age, period from symptom onset to PEA, NT-pro BNP, preoperative mPAP, preoperative PVR, RPA ratio, and PAT ratio were associated with the development of severe RPE. Multivariable logistic regression analysis revealed PAT ratio (odds ratio = 10.2; 95% confidence interval 1.87, 55.53, P = 0.007) and period from symptom onset to PEA (OR = 1.01; 95% CI = 1.00–1.02, P = 0.015) as independent risk factors for the development of severe RPE. ConclusionsThe thrombus distribution could be a key factor in the severity of RPE. PAT ratio and medical history could predict the development of severe RPE.

Chronic thromboembolic pulmonary hypertension resulting in decompensated right heart failure

Chronic thromboembolic pulmonary hypertension is a notoriously underdiagnosed cause of severe pulmonary hypertension. It is a form of precapillary pulmonary hypertension (PH) that results from intraluminal thrombus organization and fibrous formation which ultimately results in the complete obliteration of pulmonary arteries, resulting in increased pulmonary vascular resistance which leads to the development of pulmonary hypertension and as a result right heart failure. The mechanism involves the narrowing of the pulmonary artery which increases blood pressure within the lungs and impairs blood flow which increases the workload of the right side of the heart ultimately causing right heart failure. Pulmonary hypertension can also cause arrhythmias, blood clots, and bleeding in the lungs. Even though CTEPH is a deadly condition, among all forms of pulmonary hypertension, CTEPH is the only curable form. Echocardiography is the initial assessment tool for suspected PH. A right heart catheterization may be performed to confirm the presence of pulmonary hypertension. Confirmation of CTEPH requires a V/Q scan. Although ventilation/perfusion scintigraphy has a major role in the evaluation of patients with suspected CTEPH, nowadays CTA chest is being used widely as it produces much better-quality images compared to V/Q scan. Without treatment, the prognosis is very poor. Out of three treatment modalities such as; pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA), and medical therapy, surgery is the gold standard. The physician must be familiar with the disease entity, early diagnosis, and appropriate treatment to improve survival. Here we present a literature review on this topic.

The prognostic value of vasoresponse to nitric oxide in patients with chronic thromboembolic pulmonary hypertension

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by pulmonary artery obstructions due to organized chronic thrombotic material in major pulmonary arteries. In addition, about half of the patients suffer from a small vessel pulmonary arteriopathy that is a strong predictor of outcomes. Currently available treatment of CTEPH includes interventional strategies such as pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA), and non-interventional strategies with PH-specific medications. A simple way of assessing small vessel disease is the degree of vasodilation (“vasoresponse”) in response to inhaled nitric oxide (iNO). In idiopathic pulmonary arterial hypertension vasoresponse serves as the best marker for good prognosis and treatment selection. In CTEPH, the prognostic value of vasoresponse remains unclear. Purpose We investigated the prognostic value of three definitions of vasoresponse to nitric oxide in patients with CTEPH. Methods We studied 325 CTEPH patients who underwent baseline diagnostic right heart catheterization (RHC) with 40ppm iNO testing at a general hospital (AKH) between 1995 and 2019. Cox regression models, adjusting for covariates including age, sex, comorbidities, and markers for disease severity at baseline, such as proBNP, GFR, NYHA functional class, were used to determine the risk of death or lung transplantation with respect to vasoresponse. We analysed three currently used definitions of vasoresponse to nitric oxide – the classical definition (CD) as a 10mmHg reduction in mPAP to a level below 40mmHg; an absolute definition (AD) as a 10mmHg reduction in mPAP regardless of resulting mPAP; and the percent definition (PD) as a 10% reduction in mPAP regardless of resulting mPAP. Results Patients had a median age of 62 (interquartile range [IQR]: 50, 71) at time of baseline right heart catheterization and 50% were female. During a median observation time of 5 years (IQR: 2.2, 9.0), the combined endpoint of death or lung transplantation occurred in 88 cases (27%). In the cox regression model PD vasoresponders, showed improved survival when undergoing PEA (p=0.0019). In PD vasoresponsive patients who were not given PEA surgery (n=66), PH medication therapy was associated with improved survival (p=0.0053), whereas BPA had no association with survival (p=0.58). In PD non-vasoresponsive patients who were not given PEA surgery (n=107) BPA improved survival (p&amp;lt;0.0001), whereas PH medication therapy did not improve survival (p=0.08). Conclusion The PD vasoresponse to iNO carries valuable prognostic information about freedom from death or lung transplantation in patients with CTEPH. In patients who are not eligible for PEA, PD vasoresponse can improve optimal therapy selection. Funding Acknowledgement Type of funding sources: None.

Five year risk assessment and treatment patterns in patients with chronic thromboembolic pulmonary hypertension.

Repeated risk assessments and treatment patterns over long time are sparsely studied in chronic thromboembolic pulmonary hypertension (CTEPH); thus, we aimed to investigate changes in risk status and treatment patterns in incident patients with CTEPH over a 5year period. Descriptive and explorative study including 311 patients diagnosed with CTEPH 2008-2019 from the Swedish pulmonary hypertension registry, stratified by pulmonary endarterectomy surgery (PEA). Risk and PH-specific treatment were assessed in surgically treated (PEA) and medically treated (non-PEA) patients at diagnosis and up to 5years follow-up. Data are presented as median (Q1-Q3), count or per cent. Prior to surgery, 63% in the PEA-group [n=98, age 64 (51-71) years, 37% female] used PH-specific treatment and 20, 69, and 10% were assessed as low, intermediate or high risk, respectively. After 1year post-surgery, 34% had no PH-specific treatment or follow-up visit registered despite being alive at 5years. Of patients with a 5year visit (n=23), 46% were at low and 54% at intermediate risk, while 91% used PH-specific treatment. In the non-PEA group [n=213, age 72 (65-77) years, 56% female], 28% were assessed as low, 61% as intermediate and 11% as high risk. All patients at high risk versus 50% at low risk used PH-specific treatment. The 1year mortality was 6%, while the risk was unchanged in 57% of the patients; 14% improved from intermediate to low risk, and 1% from high to low risk. At 5years, 27% had a registered visit and 28% had died. Of patients with a 5year visit (n=58), 38% were at low, 59% at intermediate and 1% at high risk, and 86% used PH-specific treatment. Risk status assessed pre-surgery did not foresee long-term post-PEA risk and pre-surgery PH-specific treatment did not foresee long-term post-PEA treatment. Medically treated CTEPH patients tend to remain at the same risk over time, suggesting a need for improved treatment strategies in this group.

Chronic thromboembolic pulmonary hypertension: diagnosis, operability assessment and patient selection for pulmonary endarterectomy

Healthcare providers outside pulmonary hypertension (PH) centers having misinformation or insufficient education, and a general lack of treatment awareness contribute to a massive underdiagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), diagnostic delay and refusal of surgery by patients. Together with the subjective operability assessment, this leads to too few patients undergoing pulmonary endarterectomy (PEA); even though this surgery results in improved survival and exercise capacity. Acute pulmonary embolism (PE) survivors should undergo a CTEPH screening strategy. Patients screened positive and those with CTEPH symptoms (with or without history of PE), should undergo transthoracic echocardiography (TTE) to determine the probability of PH. High PH probability patients should undergo a ventilation/perfusion (V/Q) scan. A negative scan rules out CTEPH. Patients with a positive V/Q scan, but also patients with findings suggestive for CTEPH on computed tomography pulmonary angiography (CTPA) to diagnose acute PE, should be referred to a CTEPH center. Further diagnostic work-up currently consists of catheter based pulmonary angiography, CTPA and right heart catheterization. However, new imaging technologies might replace them in the near future, with one single imaging tool to screen, diagnose and assess operability as the ultimate goal. Operability assessment should be performed by a multidisciplinary CTEPH team. PEA surgery should be organized in a single center per country or for each forty to fifty million inhabitants in order to offer the highest level of expertise. Informing patients about PEA should preferably be done by the treating surgeon. Based on the estimated incidence of CTEPH and with a better education of patients and healthcare providers, despite the advent of new interventional and medical therapies for CTEPH, the number of PEA surgeries performed should still have the potential to grow significantly.

Pulmonary thromboendarterectomy in chronic thromboembolic pulmonary hypertension: the Spanish experience.

Chronic thromboembolic pulmonary hypertension (CTEPH) can be cured by pulmonary endarterectomy (PEA). It is considered the best and only curable treatment option for patients with accessible lesions evaluated as optimal candidates. We describe the experience of the two reference centers in Spain, in order to reinforce the need for referring CTEPH patients to a specialized center to be assessed by a Multidisciplinary Expert Team. We included a population of 338 patients who met the definition for CTEPH and underwent PEA between January 2007 and December 2019. The surgery was indicated in almost 60% of patients assessed. Demographic, anthropometric, hemodynamic and echocardiographic features are listed for PEA patients. Immediate and one-year postoperative outcomes as well as overall mortality were analyzed. Mean age was 53.5±15.0 years, 53.8% were men; a total of 68.5% were in WHO functional class III-IV; and most of them were in a preoperative hemodynamic condition: mean pulmonary arterial pressure (mPAP) was 46.5±13.1 mmHg and mean pulmonary vascular resistance (PVR) was 764.5±392.8 dyn·s·cm-5. PEA surgery was performed with cardiopulmonary bypass (CBP) and circulatory arrest, with very few complications [including neurological, postoperative reperfusion edema, extracorporeal membrane oxygenation (ECMO) implant and cardiac failure] and optimal postoperative results, where exercise capacity increased and mPAP and PVR values decreased significantly. Presence of persistent pulmonary hypertension (PH) at the six-month right heart catheterization was evaluated. A 3.3% perioperative mortality was achieved. Overall, one-, three- and five-year survival rates were analyzed by Kaplan-Meier's method (94.8%, 93.3% and 90.5% respectively), as well as for residual PH patients. Mortality risk factors were assessed. Outstanding PEA results were seen in the immediate, one-year and long-term outcomes. The incidence of complications, including in-hospital mortality and long-term mortality were also below European rates.

Chronic Thromboembolic Pulmonary Hypertension: An Update.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease observed in a small proportion of patients after acute pulmonary embolism (PE). CTEPH has a high morbidity and mortality rate, related to the PH severity, and a poor prognosis, which mirrors the right ventricular dysfunction involvement. Pulmonary endarterectomy (PEA) reduces pulmonary vascular resistance, making it the treatment of choice and should be offered to operable CTEPH patients, as significant symptomatic and prognostic improvement has been observed. Moreover, these patients may also benefit from the advances made in surgical techniques and pulmonary hypertension-specific medication. However, not all patients are eligible for PEA surgery, as some have either distal pulmonary vascular obstruction and/or significant comorbidities. Therefore, surgical candidates should be carefully selected by an interprofessional team in expert centers. This review aims at making an overview of the risk factors and latest developments in diagnostic tools and treatment options for CTEPH.

Chronic thromboembolic pulmonary hypertension: a review of risk factors, management and current challenges

ABSTRACT Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) is an under-diagnosed complication of acute pulmonary embolism (PE), whereby there is incomplete resolution of pulmonary emboli, which then become organized into obstructive fibrotic material with an associated small vessel vasculopathy. CTEPH is diagnosed by abnormal lung perfusion despite 3-months of oral anticoagulation with persistent pulmonary artery thromboembolic/ fibrotic material confirmed on cross sectional imaging and pulmonary hypertension on right heart catheterization. Without treatment the prognosis is bleak, but there are now three treatment modalities that are effective in treating patients with CTEPH: pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA) and medical therapy. Areas Covered We provide a comprehensive review of the literature with particular emphasis on the epidemiology, pathophysiology, diagnosis, and the established and emerging treatments of CTEPH. Expert opinion It is important to recognize that CTEPH is complication of an acute PE as there are effective and sometimes curative treatments available. In European and North American practice, PEA surgery remains the cornerstone of therapy in patients with CTEPH and accessible disease without significant comorbidity. For those patients with a disease burden that is inaccessible or fails to justify the risk of major surgery, initiating medical therapy and considering BPA is appropriate. Those with persistent pulmonary hypertension after PEA should also receive medical therapy and be considered for BPA or re-do endarterectomy. The available therapies are not necessarily mutually exclusive, and the treatment decision is often subjective and individualized, based on the local available expertise. Further research is needed to understand the optimal treatment strategy for patients and in particular to identify ‘responders’ and assess the synergies between these three treatment strategies.

Epidemiology of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic.

This study investigated the epidemiology and survival outcomes of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic, wherein pulmonary endarterectomy (PEA) surgery was the only targeted treatment option until 2015. This study included all consecutive adults newly diagnosed with CTEPH in the Czech Republic between 2003 and 2016. Incidence/prevalence rates were calculated using general population data extracted from the Institute of Health Information and Statistics of the Czech Republic. Kaplan–Meier estimates of survival from diagnosis until 2018 were calculated. Of a total of 453 patients observed, 236 (52.1%) underwent PEA (median time from diagnosis to PEA: 2.9 months) and 71 (34.1%) had residual pulmonary hypertension (PH) post‐PEA. CTEPH incidence rate (95% confidence interval [CI]) between 2006 and 2016 was 4.47 (4.05; 4.91) patients per million (ppm) per year, and the prevalence (95% CI) was 37.43 (33.46; 41.73) ppm in 2016. The rate of CTEPH‐related hospitalizations (95% CI) per 100 person‐years was 24.4 (22.1; 26.9) for operated patients and 34.2 (30.9; 37.7) for not‐operated patients. Median overall survival (95% CI) for all patients from CTEPH diagnosis was 11.2 (9.4; not reached) years. Five‐year survival probability (95% CI) was 95.3% (89.9; 97.9) for operated patients without residual PH, 86.3% (75.3; 92.7) for operated patients with residual PH and 61.2% (54.0; 67.6) for not‐operated patients. This study reported epidemiological estimates of CTEPH in the Czech Republic consistent with estimates from other national systematic registries; and indicates an unmet medical need in not‐operated patients and operated patients with residual PH.

EXERCISE HEMODYNAMICS TO REVEAL LATENT PULMONARY VASCULAR ABNORMALITIES AMONG CHRONIC THROMBOEMBOLIC DISEASE PATIENTS

BACKGROUND Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by persistent and organized pulmonary thrombi and pulmonary hypertension (PH). Right heart catheterization (RHC) is the gold standard for diagnosis of PH, defined by a resting mean pulmonary artery pressure (mPAP) > 20 mmHg. Chronic organized thrombi, in the absence of PH at rest is termed chronic thromboembolic disease (CTED). Pulmonary endarterectomy (PEA) is indicated for CTEPH, however PEA is not routinely endorsed for CTED. Exercise is increasingly employed as a provocative maneuver during RHC to elicit abnormalities of pulmonary vascular and cardiac physiology in patients without PH at rest who are at risk of pulmonary vascular disease. Exercise RHC may expose abnormalities of pulsatile or resistive pulmonary vascular afterload in CTED patients and may potentially identify a subset of CTED patients who benefit from PEA surgery. We describe the proportion of CTED patients with latent pulmonary vascular hemodynamic abnormalities and their hemodynamic characteristics. METHODS AND RESULTS Consecutive patients with a resting mPAP ≤ 20 mmHg and CTED who completed an exercise hemodynamic study between November 2016 and September 2020 were included. We defined exercise abnormalities of pulmonary resistive load as mPAP/CO > 3 WU and exercise abnormalities of pulsatile load as the ratio of pulmonary pulse pressure to pulmonary artery wedge pressure (PP/PAWP) > 2.5. Thirty-seven CTED patients (32% female, 55±15 years) were analyzed. Resting hemodynamics for the entire cohort and exercise hemodynamics for patients with and without latent pulmonary vascular abnormalities are shown (Table 1). Twenty-two patients (59%) demonstrated latent pulmonary vascular abnormalities; 12 had an elevated pulsatile load (PP/PAWP 5.3±4.5), 5 had an elevated resistive load (mPAP/CO 3.7±0.7 WU) and 5 had both (mPAP/CO 3.7±0.5 WU, PP/PAWP 7.3±3.6). Patients with latent pulmonary vascular abnormalities exercised to lower work-rates (P=0.03) and had lower exercise PAWP (P=0.02). They were also characterized by higher pulmonary vascular resistance (P < 0.001) and impaired pulmonary artery compliance (P < 0.001) compared to patients without latent pulmonary vascular abnormalities. CONCLUSION Patients with CTED and resting mPAP ≤ 20mmHg, exercise demonstrated latent abnormal pulmonary vascular responses in over 50% of patients. These patients predominantly demonstrated abnormalities of pulsatile afterload relative to resistive afterload. Further study is required to understand if these patients benefit from PEA surgery. Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by persistent and organized pulmonary thrombi and pulmonary hypertension (PH). Right heart catheterization (RHC) is the gold standard for diagnosis of PH, defined by a resting mean pulmonary artery pressure (mPAP) > 20 mmHg. Chronic organized thrombi, in the absence of PH at rest is termed chronic thromboembolic disease (CTED). Pulmonary endarterectomy (PEA) is indicated for CTEPH, however PEA is not routinely endorsed for CTED. Exercise is increasingly employed as a provocative maneuver during RHC to elicit abnormalities of pulmonary vascular and cardiac physiology in patients without PH at rest who are at risk of pulmonary vascular disease. Exercise RHC may expose abnormalities of pulsatile or resistive pulmonary vascular afterload in CTED patients and may potentially identify a subset of CTED patients who benefit from PEA surgery. We describe the proportion of CTED patients with latent pulmonary vascular hemodynamic abnormalities and their hemodynamic characteristics. Consecutive patients with a resting mPAP ≤ 20 mmHg and CTED who completed an exercise hemodynamic study between November 2016 and September 2020 were included. We defined exercise abnormalities of pulmonary resistive load as mPAP/CO > 3 WU and exercise abnormalities of pulsatile load as the ratio of pulmonary pulse pressure to pulmonary artery wedge pressure (PP/PAWP) > 2.5. Thirty-seven CTED patients (32% female, 55±15 years) were analyzed. Resting hemodynamics for the entire cohort and exercise hemodynamics for patients with and without latent pulmonary vascular abnormalities are shown (Table 1). Twenty-two patients (59%) demonstrated latent pulmonary vascular abnormalities; 12 had an elevated pulsatile load (PP/PAWP 5.3±4.5), 5 had an elevated resistive load (mPAP/CO 3.7±0.7 WU) and 5 had both (mPAP/CO 3.7±0.5 WU, PP/PAWP 7.3±3.6). Patients with latent pulmonary vascular abnormalities exercised to lower work-rates (P=0.03) and had lower exercise PAWP (P=0.02). They were also characterized by higher pulmonary vascular resistance (P < 0.001) and impaired pulmonary artery compliance (P < 0.001) compared to patients without latent pulmonary vascular abnormalities. Patients with CTED and resting mPAP ≤ 20mmHg, exercise demonstrated latent abnormal pulmonary vascular responses in over 50% of patients. These patients predominantly demonstrated abnormalities of pulsatile afterload relative to resistive afterload. Further study is required to understand if these patients benefit from PEA surgery.

Chronic Thromboembolic Pulmonary Hypertension Medical Management.

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.

Risk Factors for Prolonged Mechanical Ventilation After Pulmonary Endarterectomy: 7 Years' Experience From an Experienced Hospital in China.

Background: Prolonged mechanical ventilation (PMV) is common after cardiothoracic surgery, whereas the mechanical ventilation strategy after pulmonary endarterectomy (PEA) has not yet been reported. We aim to identify the incidence and risk factors for PMV and the relationship between PMV and short-term outcomes.Methods: We studied a retrospective cohort of 171 who undergoing PEA surgery from 2014 to 2020. Cox regression with restricted cubic splines was performed to identify the cutoff value for PMV. The Least absolute shrinkage and selection operator regression and logistic regressions were applied to identify risk factors for PMV. The impacts of PMV on the short-term outcomes were evaluated.Results: PMV was defined as the duration of mechanical ventilation exceeding 48 h. Independent risk factors for PMV included female sex (OR 2.911; 95% CI 1.303–6.501; P = 0.009), prolonged deep hypothermic circulatory arrest (DHCA) time (OR 1.027; 95% CI 1.002–1.053; P = 0.036), increased postoperative blood product use (OR 3.542; 95% CI 1.203–10.423; P = 0.022), elevated postoperative total bilirubin levels (OR 1.021; 95% CI 1.007–1.034; P = 0.002), increased preoperative pulmonary artery pressure (PAP) (OR 1.031; 95% CI 1.014–1.048; P < 0.001) and elongated postoperative right ventricular anteroposterior dimension (RVAD) (OR 1.119; 95% CI 1.026–1.221; P = 0.011). Patients with PMV had longer intensive care unit stays, higher incidences of postoperative complications, and higher in-hospital medical expenses.Conclusions: Female sex, prolonged DHCA time, increased postoperative blood product use, elevated postoperative total bilirubin levels, increased preoperative PAP, and elongated postoperative RVAD were independent risk factors for PMV. Identification of risk factors associated with PMV in patients undergoing PEA may facilitate timely diagnosis and re-intervention for some of these modifiable factors to decrease ventilation time and improve patient outcomes.

The Choice of Priming Solution for Cardiopulmonary Bypass during Pulmonary Endarterectomy for CTEPH Patients Has Important Impact

<h3>Purpose</h3> Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). In recent decades, refined surgical PEA technique using cardiopulmonary bypass (CPB) has achieved significant reduction in mortality. Hemodilution generated by crystalloid priming solution in heart surgery causes a number of disadvantages, and no standard for PEA is described. We aim to assess the outcome of CTEPH patients after protocol change for CPB with adding 5% albumin to priming solution during PEA. <h3>Methods</h3> Since changing the CPB protocol in 2018, 28 CTEPH patients were retrospectively compared to a historical control by 1:1 propensity score matching gender, age, PVR (pulmonary vascular resistance) before PEA surgery. The resulting two cohort groups were analysed for intraoperative fluid balance, vasoactive inotropic score (VIS), operative time and their influence on outcome. Regression models of hospitalization, ICU (intensive care unit) and intubation time considered the total blood volume balance as mediator of the effect of the new protocol. <h3>Results</h3> Lower CPB-balance (p<0.001), VIS score (p=0.04), shorter operative time (p=0.03) and hospitalization (p=0.008) were observed in the cohort operated with the new protocol. Postoperative 30 and 90 day mortality and morbidity were similar between groups. Shorter operative time (mean change (mc) 1.4, 95% CI [0.8. 2.0], p< 0.0001) and new protocol (mc -3.68, 95% CI [-5.22, -2.14], p< 0.0001) are independently associated with lower CPB balance, resulting in a beneficial effect on VIS score, ICU, intubation and hospitalization time (mean ratio (mr) 1.12, 1.11, 1.14, 1.05, p<0.0001, p=0.0011, 0.0013, 0.008, respectively). The protocol change shows positive trend with significant impact on hospitalization time (p=0.02). <h3>Conclusion</h3> Priming protocol for CPB with addition of 5% albumin had a beneficial effect on the CPB balance and improved postoperative outcome after PEA for CTEPH patients.