scholarly journals Persistent exercise intolerance after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 55 (6) ◽  
pp. 2000109
Author(s):  
Dieuwertje Ruigrok ◽  
Lilian J. Meijboom ◽  
Esther J. Nossent ◽  
Anco Boonstra ◽  
Natalia J. Braams ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Exercise Capacity ◽  
Cardiopulmonary Exercise Testing ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Exercise Intolerance ◽  
Pulmonary Endarterectomy ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension ◽  
Preoperative Predictors ◽  
Residual Pulmonary Hypertension

AimHaemodynamic normalisation is the ultimate goal of pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH). However, whether normalisation of haemodynamics translates into normalisation of exercise capacity is unknown. The incidence, determinants and clinical implications of exercise intolerance after PEA are unknown. We performed a prospective analysis to determine the incidence of exercise intolerance after PEA, assess the relationship between exercise capacity and (resting) haemodynamics and search for preoperative predictors of exercise intolerance after PEA.MethodsAccording to clinical protocol all patients underwent cardiopulmonary exercise testing (CPET), right heart catheterisation and cardiac magnetic resonance (CMR) imaging before and 6 months after PEA. Exercise intolerance was defined as a peak oxygen consumption (V′O2) <80% predicted. CPET parameters were judged to determine the cause of exercise limitation. Relationships were analysed between exercise intolerance and resting haemodynamics and CMR-derived right ventricular function. Potential preoperative predictors of exercise intolerance were analysed using logistic regression analysis.Results68 patients were included in the final analysis. 45 (66%) patients had exercise intolerance 6 months after PEA; in 20 patients this was primarily caused by a cardiovascular limitation. The incidence of residual pulmonary hypertension was significantly higher in patients with persistent exercise intolerance (p=0.001). However, 27 out of 45 patients with persistent exercise intolerance had no residual pulmonary hypertension. In the multivariate analysis, preoperative transfer factor of the lung for carbon monoxide (TLCO) was the only predictor of exercise intolerance after PEA.ConclusionsThe majority of CTEPH patients have exercise intolerance after PEA, often despite normalisation of resting haemodynamics. Not all exercise intolerance after PEA is explained by the presence of residual pulmonary hypertension, and lower preoperative TLCO was a strong predictor of exercise intolerance 6 months after PEA.

scholarly journals Exercise intolerance in chronic thromboembolic pulmonary hypertension after pulmonary angioplasty

2020 ◽  
Vol 56 (1) ◽  
pp. 1901982
Author(s):  
Hanako Kikuchi ◽  
Ayumi Goda ◽  
Kaori Takeuchi ◽  
Takumi Inami ◽  
Takashi Kohno ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Oxygen Consumption ◽  
Exercise Capacity ◽  
Cardiopulmonary Exercise Testing ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Exercise Intolerance ◽  
Peak Oxygen Consumption ◽  
Ventilatory Efficiency ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

IntroductionExercise pulmonary hypertension is common in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who experience shortness of breath during exercise and reduced exercise capacity despite normalised pulmonary arterial pressure (PAP) at rest; however, the relationship between exercise pulmonary hypertension and exercise capacity remains unclear. Here we aimed to determine whether exercise pulmonary hypertension is related to exercise capacity and ventilatory efficiency in CTEPH patients with normalised resting haemodynamics after pulmonary balloon angioplasty (BPA).Patients and methodsIn total, 249 patients with CTEPH treated with BPA (mean±sd age 63±14 years; male:female 62:187) with normal mean PAP (mPAP) (<25 mmHg) and pulmonary arterial wedge pressure (≤15 mmHg) at rest underwent cardiopulmonary exercise testing with right heart catheterisation. mPAP–cardiac output (CO) during exercise was plotted using multipoint plots. Exercise pulmonary hypertension was defined by a mPAP–CO slope >3.0.ResultsAt rest, pulmonary vascular resistance was significantly higher in the exercise pulmonary hypertension group (n=116) than in the non-exercise pulmonary hypertension group (n=133). Lower peak oxygen consumption (13.5±3.8 versus 16.6±4.7 mL·min−1·kg−1; p<0.001) was observed in the former group. The mPAP–CO slope was negatively correlated with peak oxygen consumption (r= −0.45, p<0.001) and positively correlated with the minute ventilation versus carbon dioxide output slope (r=0.39, p<0.001).ConclusionsImpaired exercise capacity and ventilatory efficiency were observed in patients with CTEPH who had normalised PAP at rest but exercise pulmonary hypertension.

scholarly journals The extent of enlarged bronchial arteries is not correlated with the development of reperfusion pulmonary edema after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096867
Author(s):  
Yumiko Ikubo ◽  
Takayuki J. Sanada ◽  
Nobuhiro Tanabe ◽  
Akira Naito ◽  
Hiroki Shoji ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Edema ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
Bronchial Arteries ◽  
Thromboembolic Pulmonary Hypertension ◽  
Residual Pulmonary Hypertension

This study investigated whether dilated bronchial arteries are associated with reperfusion pulmonary edema in patients with chronic thromboembolic pulmonary hypertension. Results showed that the extent of enlarged bronchial arteries was not associated with the development of reperfusion pulmonary edema, whereas the residual pulmonary hypertension had a significant association.

scholarly journals CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 56 (4) ◽  
pp. 1902096
Author(s):  
Michael Newnham ◽  
Katherine Bunclark ◽  
Nisha Abraham ◽  
Samantha Ali ◽  
Liliana Amaral-Almeida ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Patient Reported Outcome ◽  
Pulmonary Endarterectomy ◽  
Minimally Clinically Important Difference ◽  
Recommended Treatment ◽  
Patient Reported ◽  
Thromboembolic Pulmonary Hypertension ◽  
Clinically Significant ◽  
Residual Pulmonary Hypertension

BackgroundPulmonary endarterectomy (PEA) is the recommended treatment for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) score is an internationally validated patient-reported outcome (PRO) measure for CTEPH. It assesses three domains: activity, quality of life (QoL) and symptoms. We assessed PROs in patients with CTEPH undergoing PEA.MethodsThis retrospective observational study of consecutive CTEPH patients undergoing PEA at the UK national PEA centre between 2006 and 2017 assessed change in CAMPHOR score from baseline (pre-PEA) until up to 5 years post-PEA. CAMPHOR scores were compared between 1) those with and without clinically significant residual pulmonary hypertension and 2) those undergoing PEA and propensity-matched CTEPH patients who were not operated on. The minimally clinically important difference (MCID) was calculated using an anchor-based method.ResultsOut of 1324 CTEPH patients who underwent PEA, 1053 (80%) had a CAMPHOR score recorded pre-PEA, 934 (71%) had a score recorded within a year of PEA and 784 (60%) had both. There were significant improvements between pre- and post-PEA in all three CAMPHOR domains (median±interquartile range activity −5±7, QoL −4±8, symptoms −7±8; all p<0.0001). Improvements in CAMPHOR score were greater and more sustained in those without clinically significant residual pulmonary hypertension. CTEPH patients undergoing PEA had better CAMPHOR scores than those not operated on. The MCID in CAMPHOR score was −3±5 for activity, −4±7 for QoL and −6±7 for symptoms.ConclusionsPROs are markedly improved by PEA in patients with CTEPH, more so in those without clinically significant residual pulmonary hypertension.

scholarly journals Oxygen Pathway Limitations in Patients with Chronic Thromboembolic Pulmonary Hypertension

Circulation ◽  
2021 ◽  
Author(s):  
Erin J. Howden ◽  
Sergio Ruiz-Carmona ◽  
Mathias Claeys ◽  
Ruben de Bosscher ◽  
Rik Willems ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Pulmonary Hypertension ◽  
Cardiac Output ◽  
Exercise Capacity ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
Pulmonary Hemodynamics ◽  
Vascular Intervention ◽  
Diffusion Capacity ◽  
Thromboembolic Pulmonary Hypertension

Background: Exertional intolerance is a limiting and often crippling symptom in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Traditionally the etiology has been attributed to central factors, including ventilation-perfusion mismatch, increased pulmonary vascular resistance and right heart dysfunction and uncoupling. Pulmonary endarterectomy and, balloon pulmonary angioplasty provide substantial improvement of functional status and hemodynamics. However, despite normalization of pulmonary hemodynamics, exercise capacity often does not return to age-predicted. By systematically evaluating the oxygen (O 2 ) pathway we aimed to elucidate the cause/s of functional limitations in CTEPH patients before and after pulmonary vascular intervention. Methods: Using exercise cardiac magnetic resonance (CMR) imaging with simultaneous invasive hemodynamic monitoring, we sought to quantify the steps of the O2 transport cascade from the mouth to the mitochondria in patients with CTEPH (n=20) as compared to healthy subjects (n=10). Furthermore we evaluated the effect of pulmonary vascular intervention (pulmonary endarterectomy or balloon angioplasty) on the individual components of the cascade (n=10). Results: Peak VO2 was significantly reduced in CTEPH patients relative to controls (56±17 vs 112±20% of predicted, p<0.0001). The difference was due to impairments in multiple steps of the O 2 cascade, including O 2 delivery (product of cardiac output and arterial O 2 content), skeletal muscle diffusion capacity, and pulmonary diffusion. The total O 2 extracted in the periphery, i.e. ΔAVO 2 , was not different. Following pulmonary vascular intervention, peak VO 2 increased significantly (12.5±4.0 to 17.8±7.5 ml/kg/min, p=0.036) but remained below age-predicted (70±11%). The O 2 delivery was improved due to an increase in peak cardiac output and lung diffusion capacity. However, peak exercise ΔAVO2 was unchanged, as was skeletal muscle diffusion capacity. Conclusions: We demonstrated that CTEPH patients have significant impairment of all steps in the O 2 utilisation cascade resulting in markedly impaired exercise capacity. Pulmonary vascular intervention increased peak VO 2 , by partly correcting O 2 delivery but having no impact on abnormalities in peripheral O 2 extraction. This suggests that current interventions only partially address patients' limitations and that additional therapies may improve functional capacity.

scholarly journals Dynamic vascular changes in chronic thromboembolic pulmonary hypertension after pulmonary endarterectomy

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402090788 ◽  
Author(s):  
Dieuwertje Ruigrok ◽  
Natalia J. Braams ◽  
Esther J. Nossent ◽  
Peter I. Bonta ◽  
Anco Boonstra ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Vascular Lesions ◽  
Heart Catheterization ◽  
Pulmonary Endarterectomy ◽  
Ct Pulmonary Angiography ◽  
Thromboembolic Pulmonary Hypertension ◽  
Residual Pulmonary Hypertension

Residual pulmonary hypertension is an important sequela after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. Recurrent thrombosis or embolism could be a contributor to this residual pulmonary hypertension but the potential extent of its role is unknown in part because data on incidence are lacking. We aimed to analyze the incidence of new intravascular abnormalities after pulmonary endarterectomy and determine hemodynamic and functional implications. A total of 33 chronic thromboembolic pulmonary hypertension patients underwent routine CT pulmonary angiography before and six months after pulmonary endarterectomy, together with right heart catheterization and exercise testing. New vascular lesions were defined as (1) a normal pulmonary artery before pulmonary endarterectomy and containing a thrombus, web, or early tapering six months after pulmonary endarterectomy or (2) a pulmonary artery already containing thrombus, web, or early tapering at baseline, but increasing six months after pulmonary endarterectomy. Nine of 33 (27%) chronic thromboembolic pulmonary hypertension patients showed new vascular lesions on CT pulmonary angiography six months after pulmonary endarterectomy. In a subgroup of patients undergoing CT pulmonary angiography 18 months after pulmonary endarterectomy, no further changes in lesions were noted. Hemodynamic and functional outcomes were not different between patients with and without new vascular lesions. New vascular lesions are common after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension; currently their origin, dynamics, and long-term consequences remain unknown.

scholarly journals Secondary left heart failure occurred during VA-ECMO assistance for severe residual pulmonary hypertension after pulmonary endarterectomy: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Feng Long ◽  
Ming Luo ◽  
Zhen Qin ◽  
Bo Wang ◽  
Ronghua Zhou
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Dysfunction ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Left Ventricular ◽  
Left Heart ◽  
Pulmonary Endarterectomy ◽  
Ventricular Afterload ◽  
Thromboembolic Pulmonary Hypertension ◽  
Va Ecmo ◽  
Residual Pulmonary Hypertension

Abstract Background In patients of chronic thromboembolic pulmonary hypertension undergoing pulmonary endarterectomy, veno-arterial extracorporeal membrane oxygenation (VA-ECMO) provides full haemodynamic support. However, during a rescue treatment of VA-ECMO for patients with difficulty weaning from cardiopulmonary bypass, a significantly increase left ventricular afterload through retrograde infusion of arterialized blood into the descending aorta may occur. Case presentation We report a 70-year-old man who suffered severe residual pulmonary hypertension following pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. Preoperative echocardiogram showed a dilated and poorly functioning right ventricle, as well as a small left heart with normal function (TAPES9.6 mm, LVEF64%, average E/E′11.94, lateral E′12.1 cm/s, tricuspid regurgitation velocity 2.5 m/s), while postoperative echocardiography revealed a significant decrease of whole ventricular function on postoperative day 1(TAPES4mm, LVEF28%, average E/E′15, lateral E′6.7 cm/s, tricuspid regurgitation velocity 4.1 m/s), indicating the patient developed severe secondary left ventricular dysfunction on the basis of right ventricular dysfunction, during VA-ECMO support. Then comprehensive measures were adopted, such as down-regulating VA-ECMO flow rate, adjusting respiratory parameters, using vasoactive drugs, as well as prostacyclin. Eventually, the pulmonary hypertension decreased to moderate degree, and the heart function improved gradually. Conclusions In the face of severe residual pulmonary hypertension and sencondary left ventricular dysfunction associated with VA-ECMO, comprehensive measures described above may facilitate recovery. ECMO flow titration to maintain relatively low flow rate is very important to not only maintain systemic perfusion, but also reduce left ventricular afterload and ensure pulsatile perfusion of pulmonary artery.

scholarly journals Comparative Effectiveness of Exercise Training for Patients With Chronic Thromboembolic Pulmonary Hypertension After Pulmonary Endarterectomy: A Systematic Review and Meta-Analysis

2021 ◽  
Vol 8 ◽  
Author(s):  
Ya-Lin Zhao ◽  
Ping Yuan ◽  
Qin-Hua Zhao ◽  
Su-Gang Gong ◽  
Rui Zhang ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systematic Review ◽  
Pulmonary Hypertension ◽  
Exercise Training ◽  
Exercise Capacity ◽  
Meta Analysis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
Thromboembolic Pulmonary Hypertension

Background: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) still experience reduced exercise capacity despite pulmonary endarterectomy (PEA). Exercise training improves the exercise capacity and quality of life (QoL) in patients with PH, but data on the effects of exercise training on these patients are scarce. The aim of this meta-analysis and systematic review was to evaluate the effectiveness and safety of exercise training in CTEPH after PEA.Methods: We searched the relevant literature published before January 2020 for the systematic review and meta-analysis using the PubMed, EMBASE, and Cochrane Library databases. The primary outcome was a change in the 6-min walking distance (6 MWD). We also assessed the effect of exercise on the peak oxygen uptake (VO2) or peak VO2/kg, oxygen uptake anaerobic threshold, workload, oxygen pulse, hemodynamics, arterial blood gases, oxygen saturation, N-terminal pro-brain-type natriuretic peptide (NT-proBNP), quality of life (QoL) and pulmonary function tests.Results: We included 4 studies with 208 exercise-training participants. In the pooled analysis, short-term exercise training can improve the 6 MWD of 58.89 m (95% CI: 46.26–71.52 m, P &lt; 0.0001). There was a significant increase in the peak VO2/kg or peak VO2 after exercise training (3.15 ml/min/kg, 95% CI: 0.82–5.48, P = 0.008; 292.69 ml/min, 95% CI: 24.62–560.75, P = 0.032, respectively). After exercise training, the maximal workload and O2 pulse significantly improved. Three months of exercise training increased the right ventricular ejection fraction by 3.53% (95% CI: 6.31–11.94, P &lt; 0.00001, I2 = 0) independently of PEA surgery. In addition, NT-proBNP plasma levels significantly improved with exercise training after PEA [weighted mean difference (WMD): −524.79 ng/L, 95% CI: 705.16 to −344.42, P &lt; 0.0001, I2 = 0]. The partial pressure of oxygen and pH improved progressively over 12 weeks of exercise training (WMD: 4 mmHg, 95% CI: 1.01–8.33, P = 0.01; WMD: 0.03, 95% CI: 0.02–0.04, P &lt; 0.0001, respectively). Subscales of the QoL measured by the SF-36 questionnaire had also improved. In addition, exercise training was well-tolerated with a low dropout rate, and no major adverse events occurred during exercise training.Conclusion: Exercise training may be associated with a significant improvement in the exercise capacity and QoL among CTEPH patients after PEA and was proven to be safe. However, more large-scale multicentre studies are needed to confirm the effectiveness and safety of exercise training in CTEPH patients after PEA.PROSPERO registration number:CRD42021235275.

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