P4677Balloon pulmonary angioplasty improves pulmonary arterial compliance in patients with inoperable chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Umemoto ◽  
K Abe ◽  
K Horimoto ◽  
K Hosokawa ◽  
H Tsutsui

Abstract Background Right ventricular (RV) pressure overload is directly related to the increase in mortality in pulmonary hypertension. Pulmonary arterial compliance (CPA; stroke volume/pulmonary pulse pressure) was reported to be an independent determinant of RV systolic afterload in patients with pulmonary arterial hypertension (PAH). Recently, balloon pulmonary angioplasty (BPA) has been reported to reduce mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (RPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, the effects of BPA on CPA remain unclear. Purpose The aim of this study was to investigate the impact of BPA on CPA in patients with inoperable CTEPH. Methods We retrospectively analyzed 78 patients (388 BPA sessions) with inoperable CTEPH who underwent BPA in our hospital from September 2012 to June 2018. Total number of BPA sessions was 5.0±1.8 (range 1–10). The pressure values were obtained from right heart catheterization at baseline (n=78), just after the final BPA (n=78) and follow-up (n=19) periods. The intervals from baseline to the final BPA and the final BPA to follow-up were 593±498 days and 397±276 days, respectively. Results Mean age was 60.5±12.6 years old, and 64 (82%) were female. All patients were symptomatic (WHO functional class II/III/IV 16/55/7). Patients who had pulmonary vasodilators decreased from 70 (90%) at baseline to 23 (28%) at the final BPA and 2 (15%) at follow-up. BPA reduced mPAP and RPA significantly from baseline to the final BPA and follow-up periods. BPA also improved CPA with significant reduction of pulse pressure despite no significant changes in stroke volume between baseline and follow-up (Table). CPA between the final BPA and follow-up was equivalent (p=0.95). Conclusions BPA improved CPA just after the final BPA in inoperable CTEPH patients. In addition, CPA was preserved during the follow-up after the final BPA sessions. These data suggest that BPA consistently unloads RV systolic afterload in those patients.

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096383
Author(s):  
Thenappan Thenappan ◽  
Nadine Al-Naamani ◽  
Stefano Ghio ◽  
Hossein-Ardeschir Ghofrani ◽  
Paul M. Hassoun ◽  
...  

Pulmonary arterial compliance is a measure of the pulsatile afterload of the right ventricle. Lower pulmonary arterial compliance is associated with reduced right ventricular function and worse prognosis in pulmonary hypertension. The effect of pulmonary vasodilators on pulmonary arterial compliance has not been evaluated in detail in pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. In this post hoc analysis of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension in the PATENT and CHEST studies, we evaluated the change in pulmonary arterial compliance with riociguat versus placebo. Association of pulmonary arterial compliance with clinical outcomes was assessed using Kaplan–Meier and Cox proportional hazards analyses. Compared with placebo, riociguat significantly improved pulmonary arterial compliance in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Pulmonary arterial compliance at baseline was associated with survival and clinical worsening-free survival in pulmonary arterial hypertension but only with clinical worsening-free survival in chronic thromboembolic pulmonary hypertension. In patients with pulmonary arterial hypertension, pulmonary arterial compliance at follow-up ≥1.6 mL/mmHg was associated with better outcomes than pulmonary arterial compliance <1.6 mL/mmHg. In patients with chronic thromboembolic pulmonary hypertension, pulmonary arterial compliance at follow-up did not predict outcomes. Cox proportional hazards analyses showed no association between change in pulmonary arterial compliance and outcomes in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. In conclusion, riociguat improved pulmonary arterial compliance in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Pulmonary arterial compliance at baseline or follow-up, rather than change in pulmonary arterial compliance, is of prognostic importance for outcomes.


Respiration ◽  
2021 ◽  
pp. 1-10
Author(s):  
Christian Nagel ◽  
Nicola Benjamin ◽  
Benjamin Egenlauf ◽  
Christina A. Eichstaedt ◽  
Christine Fischer ◽  
...  

<b><i>Background:</i></b> Pulmonary arterial compliance (PAC) is a prognostic parameter in pulmonary arterial hypertension (PAH) reflecting the elasticity of the pulmonary vessels. <b><i>Objectives:</i></b> The objective of this post hoc analysis of a prospective randomized controlled trial (RCT) was to assess the effect of exercise training on PAC and stroke volume (SV) in patients with PAH and persistent/inoperable chronic thromboembolic pulmonary hypertension (CTEPH). <b><i>Method:</i></b> From the previous RCT, 43 out of 87 patients with severe PAH (<i>n</i> = 29) and CTEPH (<i>n</i> = 14) had complete haemodynamic examinations at baseline and after 15 weeks by right heart catheterization and were analysed (53% female, 79% World Health Organization functional class III/IV, 58% combination therapy, 42% on supplemental oxygen therapy, training group <i>n</i> = 24, and control group <i>n</i> = 19). Medication remained unchanged for all patients. <b><i>Results:</i></b> Low-dose exercise training at 4–7 days/week significantly improved PAC (training group 0.33 ± 0.65 mL/mm Hg vs. control group −0.06 ± 1.10 mL/mm Hg; mean difference 0.39 mL/mm Hg, 95% confidence interval [CI] 0.15–0.94 mL/mm Hg; <i>p</i> = 0.004) and SV (training group 9.9 ± 13.4 mL/min vs. control group −4.2 ± 11.0 mL/min; mean difference 14.2 mL, 95% CI 6.5–21.8 mL; <i>p</i> &#x3c; 0.001) in the training versus control group. Furthermore, exercise training significantly improved cardiac output and pulmonary vascular resistance at rest, peak oxygen consumption, and oxygen pulse. <b><i>Conclusions:</i></b> Our findings suggest that supervised exercise training may improve right ventricular function and PAC at the same time. Further prospective studies are needed to evaluate these findings.


2013 ◽  
Vol 305 (2) ◽  
pp. H259-H264 ◽  
Author(s):  
Robert V. MacKenzie Ross ◽  
Mark R. Toshner ◽  
Elaine Soon ◽  
Robert Naeije ◽  
Joanna Pepke-Zaba

This study analyzed the relationship between pulmonary vascular resistance (PVR) and pulmonary arterial compliance ( Ca) in patients with idiopathic pulmonary arterial hypertension (IPAH) and proximal chronic thromboembolic pulmonary hypertension (CTEPH). It has recently been shown that the time constant of the pulmonary circulation (RC time constant), or PVR × Ca, remains unaltered in various forms and severities of pulmonary hypertension, with the exception of left heart failure. We reasoned that increased wave reflection in proximal CTEPH would be another cause of the decreased RC time constant. We conducted a retrospective analysis of invasive pulmonary hemodynamic measurements in IPAH ( n = 78), proximal CTEPH ( n = 91) before (pre) and after (post) pulmonary endarterectomy (PEA), and distal CTEPH ( n = 53). Proximal CTEPH was defined by a postoperative mean pulmonary artery pressure (PAP) of ≤25 mmHg. Outcome measures were the RC time constant, PVR, Ca, and relationship between systolic and mean PAPs. The RC time constant for pre-PEA CTEPH was 0.49 ± 0.11 s compared with post-PEA-CTEPH (0.37 ± 0.11 s, P < 0.0001), IPAH (0.63 ± 0.14 s, P < 0.001), and distal CTEPH (0.55 ± 0.12 s, P < 0.05). A shorter RC time constant was associated with a disproportionate decrease in systolic PAP with respect to mean PAP. We concluded that the pulmonary RC time constant is decreased in proximal CTEPH compared with IPAH, pre- and post-PEA, which may be explained by increased wave reflection but also, importantly, by persistent structural changes after the removal of proximal obstructions. A reduced RC time constant in CTEPH is in accord with a wider pulse pressure and hence greater right ventricular work for a given mean PAP.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Brooke Currie ◽  
Evan Davies ◽  
Amélie Beaudet ◽  
Larissa Stassek ◽  
Leah Kleinman

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots and scar tissue in the blood vessels of the lungs. Health-related quality of life is often significantly impaired in patients with CTEPH. However, a better understanding of how CTEPH symptoms affect patients’ lives is needed to optimally assess the impact of the disease and treatment. Objectives This qualitative study aimed to better understand the symptoms of CTEPH and how they affect patients’ lives, as well as to determine the appropriateness of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire for use in this patient population. Methods Adults diagnosed with CTEPH, recruited from two clinical sites in the US, participated in one-to-one qualitative telephone interviews. They described their experience of CTEPH symptoms and the impact these symptoms have on their lives. They also provided feedback on the comprehensibility and relevance of the PAH-SYMPACT™‘s instructions, items, and response options. Results Participants (N = 12) had a mean age of 62.5 years. Two thirds were female and most (83%) had undergone pulmonary endarterectomy and/or balloon pulmonary angioplasty. The most frequently endorsed symptoms were shortness of breath (endorsed by all 12 participants), fatigue (11 participants), and lightheadedness (10 participants). All participants identified shortness of breath as an “extremely important” symptom, and seven participants rated fatigue as “extremely important.” The most frequent impacts of CTEPH were on ability to walk quickly (endorsed by all 12 participants), ability to walk up inclines or stairs (11 participants), and ability to carry things (11 participants). The PAH-SYMPACT™ items were relevant to most participants and reflected their experience of CTEPH. All participants indicated that no important CTEPH symptoms were missing from the PAH-SYMPACT™. Overall, the instructions, items, and response options of the PAH-SYMPACT™ were clear and easy to understand. Conclusions The symptoms and impacts experienced by patients with CTEPH align with items included in the PAH-SYMPACT™. The PAH-SYMPACT™ appears to be fit for purpose for assessing disease status in patients with CTEPH.


2019 ◽  
Vol 55 (2) ◽  
pp. 1802108 ◽  
Author(s):  
Athénaïs Boucly ◽  
Capucine Morélot-Panzini ◽  
Gilles Garcia ◽  
Jason Weatherald ◽  
Xavier Jaïs ◽  
...  

Dynamic hyperinflation is observed during exercise in 60% of patients with clinically stable pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), intensifying exertional dyspnoea. The impact of dynamic changes in respiratory mechanics during exercise on qualitative dimensions of dyspnoea in these patients has not been evaluated.26 patients (PAH n=17; CTEPH n=9) performed an incremental symptom-limited cycle exercise test. Minute ventilation (V′E), breathing pattern, operating lung volumes and dyspnoea intensity were assessed throughout exercise. Dyspnoea quality was serially assessed during exercise using a three-item questionnaire (dyspnoea descriptors). The inflection point of tidal volume (VT) relative to V′E was determined for each incremental test. Changes in inspiratory capacity during exercise defined two groups of patients: hyperinflators (65%) and non-hyperinflators (35%). Multidimensional characterisation of dyspnoea was performed after exercise using the Multidimensional Dyspnea Profile.In hyperinflators, inspiratory capacity decreased progressively throughout exercise by 0.36 L, while remaining stable in non-hyperinflators. The “work/effort” descriptor was most frequently selected throughout exercise in both types of patients (65% of all responses). At the VT/V′E inflection, work/effort plateaued while “unsatisfied inspiration” descriptors became selected predominantly only in hyperinflators (77% of all responses). In the affective domain, the emotion most frequently associated with dyspnoea was anxiety.In pulmonary hypertension patients who develop hyperinflation during exercise, dyspnoea descriptors referring to unsatisfied inspiration become predominant following the VT/V′E inflection. As these descriptors are generally associated with more negative emotional experiences, delaying or preventing the VT/V′E inflection may have important implications for symptom management in patients with pulmonary hypertension.


2016 ◽  
Vol 66 (02) ◽  
pp. 180-186 ◽  
Author(s):  
Bulent Mutlu ◽  
Ashok Paudel ◽  
Cigdem Ileri ◽  
Halil Atas ◽  
Bedrettin Yildizeli ◽  
...  

Background Our aim was to determine the prevalence of coronary artery − pulmonary artery collaterals in patients with chronic thromboembolic pulmonary hypertension (CTEPH) by retrospectively evaluating coronary angiograms of eligible consecutive patients who had undergone pulmonary endarterectomy (PEA). We also aimed to evaluate predictors and potential clinical associates of these collaterals. Methods Coronary angiograms of 83 consecutive CTEPH patients who had undergone coronary angiography before PEA operation between January 1, 2012 and June 1, 2015 were retrospectively evaluated for presence of coronary artery − pulmonary artery collaterals. Medical records of all patients were also retrospectively reviewed for demographic information, cardiovascular risk factors, preoperative right heart catheterization reports, operation reports, and follow-up data. Data of CTEPH patients with coronary artery − pulmonary artery collaterals were compared with data of CTEPH patients without such collaterals. Results There were 15 patients (18.1%) with definite and 4 patients (4.8%) with probable coronary artery − pulmonary artery collaterals among the study population. CTEPH patients with collaterals had higher preoperative pulmonary artery pressures, higher pulmonary vascular resistance (PVR) and lower cardiac index values compared with CTEPH patients without collaterals. However, CTEPH patients with collaterals displayed higher amount of reduction in PVR after PEA compared with patients without collaterals. There were no significant differences between groups regarding incidence of reperfusion injury or mortality. Conclusion Prevalence of coronary artery − pulmonary artery collaterals seems to be increased in our CTEPH patients compared with the general population. The presence of coronary artery − pulmonary artery collaterals is often combined with proximal disease with the possibility of increased reduction of PVR after PEA operation.


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