Early return of reflected waves increases right ventricular wall stress in chronic thromboembolic pulmonary hypertension

In chronic thromboembolic pulmonary hypertension (CTEPH), proximal localization of vessel obstructions is associated with poor right ventricular (RV) function compared with distal localization, though pulmonary vascular resistance, vascular compliance, characteristic impedance, and the magnitude of wave reflection are similar. In proximal CTEPH, the RV is exposed to an earlier return of the reflected wave. Early wave reflection may increase RV wall stress and compromise RV function.

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Regional Right Ventricular Abnormalities Implicate Distinct Pathophysiological Conditions in Patients With Chronic Thromboembolic Pulmonary Hypertension

Journal of the American Heart Association ◽

10.1161/jaha.120.018096 ◽

2020 ◽

Vol 9 (21) ◽

Author(s):

Hidenori Moriyama ◽

Takashi Kawakami ◽

Masaharu Kataoka ◽

Takahiro Hiraide ◽

Mai Kimura ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Right Ventricular ◽

Pulmonary Vascular Resistance ◽

Pulmonary Artery Pressure ◽

Vascular Resistance ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Area Change ◽

Thromboembolic Pulmonary Hypertension ◽

Rv Function

Background Right ventricular (RV) dysfunction is a prognostic factor for cardiovascular disease. However, its mechanism and pathophysiology remain unknown. We investigated RV function using RV‐specific 3‐dimensional (3D)‐speckle‐tracking echocardiography (STE) in patients with chronic thromboembolic pulmonary hypertension. We also assessed regional wall motion abnormalities in the RV and chronological changes during balloon pulmonary angioplasty (BPA). Methods and Results Twenty‐nine patients with chronic thromboembolic pulmonary hypertension who underwent BPA were enrolled and underwent right heart catheterization and echocardiography before, immediately after, and 6 months after BPA. Echocardiographic assessment of RV function included both 2‐dimensional‐STE and RV‐specific 3D‐STE. Before BPA, global area change ratio measured by 3D‐STE was significantly associated with invasively measured mean pulmonary artery pressure and pulmonary vascular resistance ( r =0.671 and r =0.700, respectively). Dividing the RV into the inlet, apex, and outlet, inlet area change ratio showed strong correlation with mean pulmonary artery pressure and pulmonary vascular resistance before BPA ( r =0.573 and r =0.666, respectively). Only outlet area change ratio was significantly correlated with troponin T values at 6 months after BPA ( r =0.470), and its improvement after BPA was delayed compared with the inlet and apex regions. Patients with poor outlet area change ratio were associated with a delay in RV reverse remodeling after treatment. Conclusions RV‐specific 3D‐STE analysis revealed that 3D RV parameters were novel useful indicators for assessing RV function and hemodynamics in pulmonary hypertension and that each regional RV portion presents a unique response to hemodynamic changes during treatment, implicating that evaluation of RV regional functions might lead to a new guide for treatment strategies.

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Abstract 15125: Percutaneous Transluminal Pulmonary Angioplasty Improves Hemodynamics and Right Ventricular Function in Patients with Chronic Thromboembolic Pulmonary Hypertension -One Year Follow-up Study

Circulation ◽

10.1161/circ.130.suppl_2.15125 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Masanobu Miura ◽

Koichiro Sugimura ◽

Kotaro Nochioka ◽

Tatsuo Aoki ◽

Shunsuke Tatebe ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Right Ventricular Function ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Pulmonary Hemodynamics ◽

Before And After ◽

Thromboembolic Pulmonary Hypertension ◽

Percutaneous Transluminal ◽

Rv Function

Objectives: Right ventricular function (RV) is an important prognostic indicator of pulmonary hypertension. Recent studies have demonstrated that percutaneous transluminal pulmonary angioplasty (PTPA) improves pulmonary hemodynamics in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). In this study, we examined whether PTPA also improves right ventricular dysfunction in those patients. Methods: We performed a total of 252 PTPA procedures (median 4 procedures per patient) for 56 consecutive patients with inoperable CTEPH, after stabilizing their condition using conventional pulmonary vasodilators. Among them, we enrolled 33 patients who had finished 1-year follow-up after final PTPA in the present study (female 81.8%, median age 60 yrs.). RV function was evaluated by tricuspid annular plane systolic excursion (TAPSE) on echocardiogram (42.4%, n=14) and RV ejection fraction (RVEF) on cardiac magnetic resonance imaging (39.4%, n=13) before and after PTPA. Results: No patient died during the PTPA procedure or during the 1-year follow-up period. Comparisons before and after PTPA showed marked improvement of WHO functional class III/IV (75.7 to 0%, P<0.001), 6-min walking distance (316 to 480 m, P<0.001), and brain natriuretic peptide level (93 to 23 pg/ml, P<0.001) and significant hemodynamic improvements for mean pulmonary artery pressure (42.3±10.7 to 24.4±5.7mmHg, P<0.001), cardiac index (2.2±0.6 to 2.7±0.6 L/min•m2, P<0.001) and pulmonary vascular resistance (786±384 to 265±93 dyn•sec•cm5, P<0.001). Furthermore, RV function was also significantly improved for both TAPSE (18.0±4.0 to 23.4±4.3mm, P<0.001) and RVEF (37.9±11.0 to 52.4±7.1%, P<0.001) (Figure). Conclusions: PTPA improves not only pulmonary hemodynamics but also RV function in patients with inoperable CTEPH.

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Right Ventricular Function Predicts Adverse Clinical Outcomes in Patients With Chronic Thromboembolic Pulmonary Hypertension: A Three-Dimensional Echocardiographic Study

Frontiers in Medicine ◽

10.3389/fmed.2021.697396 ◽

2021 ◽

Vol 8 ◽

Author(s):

Yidan Li ◽

Lirong Liang ◽

Dichen Guo ◽

Yuanhua Yang ◽

Juanni Gong ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Clinical Outcomes ◽

Heart Function ◽

Three Dimensional ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Heart Catheterization ◽

Right Heart ◽

Thromboembolic Pulmonary Hypertension ◽

Rv Function

Background: Right ventricular (RV) function plays a vital role in the prognosis of patients with chronic thromboembolic pulmonary hypertension (CTEPH). We used new machine learning (ML)-based fully automated software to quantify RV function using three-dimensional echocardiography (3DE) to predict adverse clinical outcomes in CTEPH patients.Methods: A total of 151 consecutive CTEPH patients were registered in this prospective study between April 2015 and July 2019. New ML-based methods were used for data management, and quantitative analysis of RV volume and ejection fraction (RVEF) was performed offline. RV structural and functional parameters were recorded using 3DE. CTEPH was diagnosed using right heart catheterization, and 62 patients underwent cardiac magnetic resonance to assess right heart function. Adverse clinical outcomes were defined as PH-related hospitalization with hemoptysis or increased RV failure, including conditions requiring balloon pulmonary angioplasty or pulmonary endarterectomy, as well as death.Results: The median follow-up time was 19.7 months (interquartile range, 0.5–54 months). Among the 151 CTEPH patients, 72 experienced adverse clinical outcomes. Multivariate Cox proportional-hazard analysis showed that ML-based 3DE analysis of RVEF was a predictor of adverse clinical outcomes (hazard ratio, 1.576; 95% confidence interval (CI), 1.046~2.372; P = 0.030).Conclusions: The new ML-based 3DE algorithm is a promising technique for rapid 3D quantification of RV function in CTEPH patients.

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Abstract 14298: Right Ventricular Strain Before and After Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension

Circulation ◽

10.1161/circ.142.suppl_3.14298 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Darrin Wong ◽

Averie Tigges ◽

Lawrence Ang ◽

MITUL PATEL ◽

Hyong Kim ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Pearson Correlation ◽

Global Longitudinal Strain ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Right Ventricular Strain ◽

Balloon Pulmonary Angioplasty ◽

Before And After ◽

Thromboembolic Pulmonary Hypertension ◽

Rv Function

Background: Right ventricular (RV) function is impaired in chronic thromboembolic pulmonary hypertension (CTEPH). Balloon pulmonary angioplasty (BPA) may be an alternative treatment for patients (pts) who are not candidates for pulmonary thromboendarterectomy (PTE). We assessed the RV global and segmental strain patterns in pts who underwent BPA. Methods: Between 12/2016 and 5/2019, 22 pts had completed BPA treatment and had transthoracic echocardiograms (echos) before and after treatment. Of those 22 pts, 14 had echos with adequate imaging of the RV. Epsilon EchoInsight® was used to measure global and segmental RV strain. Tricuspid annular plane excursion (TAPSE) was also measured. Paired t-tests were used to determine mean differences for pre & post BPA, and Pearson correlation coefficient was used to determine association with hemodynamics. Results: RV global longitudinal strain (GLS) significantly improved after BPA (-11.1±3.4 to -16.2±2.9 % p<0.05). The apical and mid segmental strain also significantly improved after BPA (-5.0±9.1 to -12.5±7.5%, p=0.03; -13.3±6.2 to -17.5±5.4%, p<0.01 respectively). Basal strain did not significantly change (-15.2±6.3 to -18.5±9.2%, p=0.22). Furthermore, TAPSE did not change significantly (1.87±0.5 to 1.95±0.5 cm, p=0.55). Hemodynamically, mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) significantly decreased (40.3±10.5 to 34.0±10.3 mmHg, p=0.001 and 5.9±3.1 to 3.9±1.8 WU, p=0.01) while cardiac output remained unchanged (5.6±2.0 to 5.7±1.1 L/min, p=0.86). There was no correlation between improvement in RV GLS and decrease in mPAP and PVR. Conclusion: RV function as measured by RV GLS significantly improved after BPA, though only the mid and apical segments improved. TAPSE and RV basal strain did not change after BPA. Previous studies have shown that GLS did not improve significantly after PTE. Furthermore, TAPSE significantly decreases after PTE. The reasons for the difference in RV function after PTE vs BPA are not entirely clear, but may be due to operability and location of disease, as well as immediate post-operative RV stunning after PTE.

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Abstract 14221: Right Ventricular Adaptation to Pressure Overload: Differences Between Chronic Thromboembolic Pulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension

Circulation ◽

10.1161/circ.142.suppl_3.14221 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Natalia J Braams ◽

Joost W van Leeuwen ◽

anton vonk noordegraaf ◽

Harm Jan Bogaard ◽

Lilian J Meijboom ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Right Ventricular ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Idiopathic Pulmonary Arterial Hypertension ◽

Diastolic Stiffness ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Arterial ◽

Rv Function

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (iPAH)) are both associated with right ventricular (RV) failure and death. Although both conditions develop in the pre-capillary pulmonary vasculature, patient characteristics are different. CTEPH patients are older, predominantly male and more often have a history of venous thromboembolism. Therefore, the RV might be affected differently in CTEPH compared to iPAH. We aimed to compare RV adaptation in CTEPH and iPAH. Methods: Between 2000 and 2019 all treatment naive iPAH and CTEPH patients diagnosed in the Amsterdam UMC were included if a right heart catheterization and cardiac magnetic resonance imaging (CMR) were performed at the time of diagnosis. RV volumes, mass and function were assessed with CMR. RV contractility, afterload, RV-pulmonary artery (RV-PA) coupling and diastolic stiffness (Eed) were obtained using single beat pressure-volume loop analysis. Differences in RV phenotypes between iPAH and CTEPH were analyzed using multiple linear regression with interaction testing after correcting for confounders. Results: A total of 235 patients were included, 116 with CTEPH and 119 with iPAH. CTEPH patients were older, predominantly male, had a higher systemic blood pressure and a lower pulmonary vascular resistance at the time of diagnosis. After correcting for these confounders, RV function and RV-PA coupling were similar in both groups. However, CTEPH patients had a higher RV end-diastolic volume index (87±27 ml/m2 vs. 82±25 ml/m2), and a lower RV wall thickness (0,6±0,1 g/ml vs. 0,7±0,2 g/ml; figure 1A). The increase in afterload in CTEPH was associated with a disproportionally larger increase in diastolic stiffness compared to iPAH, independent of RV wall thickness (figure 1B). Conclusions: Despite a similar RV function, the RV in CTEPH is more dilated and stiffer than the RV in iPAH, independent of age, sex and afterload.

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EXPRESS: Chronic thromboembolic pulmonary hypertension following pulmonary embolism with a right ventricular thrombus: A report of two cases

Pulmonary Circulation ◽

10.1177/20458940211013668 ◽

2021 ◽

pp. 204589402110136

Author(s):

Tailong Zhang ◽

Weitao Liang ◽

Longrong Bian ◽

Zhong Wu

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Imaging Features ◽

Pulmonary Endarterectomy ◽

Right Heart ◽

Thromboembolic Pulmonary Hypertension ◽

The Right ◽

Cardiac Masses

Right heart thrombus (RHT) accompanied by chronic thromboembolic pulmonary hypertension (CTEPH) is a rare entity. RHT may develop in the peripheral veins or in situ within the right heart chambers. The diagnosis of RHT is challenging, since its symptoms are typically non-specific and its imaging features resemble those of cardiac masses. Here, we report two cases of RHT with CTEPH that presented as right ventricular masses initially. Both patients underwent simultaneous pulmonary endarterectomy (PEA) and resection of the ventricular thrombi. Thus, when mass-like features are confirmed by imaging, RHT should be suspected in patients with CTEPH, and simultaneous RHT resection is required along with PEA.

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Decreased time constant of the pulmonary circulation in chronic thromboembolic pulmonary hypertension

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.00128.2013 ◽

2013 ◽

Vol 305 (2) ◽

pp. H259-H264 ◽

Cited By ~ 45

Author(s):

Robert V. MacKenzie Ross ◽

Mark R. Toshner ◽

Elaine Soon ◽

Robert Naeije ◽

Joanna Pepke-Zaba

Keyword(s):

Pulmonary Hypertension ◽

Time Constant ◽

Pulmonary Circulation ◽

Wave Reflection ◽

Arterial Compliance ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Left Heart Failure ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Arterial ◽

Rc Time Constant

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.

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