P5022Dual-energy CT was effective to evaluate of microvasculopathy in chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
H Onishi ◽  
Y Taniguchi ◽  
Y Matsuoka ◽  
K Yanaka ◽  
Y Izawa ◽  
...  

Abstract Background The existence of microvasculopathy in patients with chronic thromboembolic pulmonary hypertension (CTEPH) had been suggested. However, the impact of microvasculopathy for pathophysiology had been unknown. Recently dual-energy CT (DECT) can produce a sensitive iodine distribution map as blood perfusion in lung fields to quantify lung perfusion, also can suggest the existence of microvasculopathy according to poor subpleural perfusion which was published previously. Methods We retrospectively reviewed poor subpleural perfusion (defined as subpleural spaces either not or minimally perfused in all segments) and hemodynamics of 83 treatment-naïve CTEPH patients who underwent DECT from February 2014 to Jan 2019. Patients were divided according to poor subpleural perfusion: a microvasculopathy group (n=44) or a non-microvasculopathy group (n=39). We assessed cardiopulmonary exercise test, right heart catheterization and DECT parameters as quantitative evaluation of pulmonary blood volume (PBV). PBV was calculated as the average of entire lung iodine density. Results PBV value in non-microvasculopathy group showed significant inverse correlation with pulmonary vascular resistance (PVR) (y = 14236 x-1.028 r=−0.530, p<0.01). PBV, SvO2, and %DLCO/VA were significantly lower (22.0 vs. 26.4, p<0.01, 61.3 vs. 66.0, p<0.01, and 59.2 vs 75.9 p<0.01), and systolic pulmonary arterial pressure, PVR, VE/VCO2 slope, BNP were higher (69.3 vs 60.6 p=0.04, 834 vs 586 p<0.01, 45.5 vs. 37.8, p=0.02, and 440 vs 122 p=0.04) in microvasculopathy group, while the other parameters were similar between the two groups. Multivariate analysis revealed that %DLCO/VA was the only predictor of microvasculopathy (OR,0.895 [95% CI, 0.835 - 0.960]; P<0.01). Conclusion Pulmonary blood flow of patients in non-micorvasculopathy group showed inverse correlation with PVR. DECT was effective to assess the microvasculopathy in CTEPH. In our experience, less than 60% of non-operable CTEPH patients have microvasculopathy.

2020 ◽  
pp. 204589402098316
Author(s):  
Hiroyuki Onishi ◽  
Yu Taniguchi ◽  
Yoichiro Matsuoka ◽  
Ken-ichi Yanaka ◽  
Yu Izawa ◽  
...  

Introduction The existence of microvasculopathy in patients with chronic thromboembolic pulmonary hypertension (CTEPH) has been suggested. Recently dual-energy computed tomography (DE-CT) has been used to produce a sensitive iodine distribution map in lung fields to indicate microvasculopathy according to poor subpleural perfusion (PSP). Our aim was to evaluate the impact of microvasculopathy on pathophysiology in CTEPH. Methods According to the extent of PSP, 93 interventional treatment-naïve patients were divided into poorly perfused (n=49) or normally perfused group (n=44). We assessed cardiopulmonary exercise test, right heart catheterization, and DE-CT parameters for quantitative evaluation of lung perfusion of blood volume (lung PBV) score. Results Lung PBV score in normally perfused group was significantly inversely correlated with pulmonary vascular resistance (PVR) (PVR= 6816.1×lung PBV score -0.793, R² = 0.225, p<0.01), but lung PBV score in poorly perfused group was not. Poorly perfused group had higher PVR (879±409 dynes-sec/cm5 vs. 574±279 dynes-sec/cm5, p<0.01) and lower lung PBV score (22.1±5.4 vs. 26.4±6.6, p<0.01) and %DLCO/VA (59.9±15.4% vs. 78.8±14.2%, p<0.01). Conclusions PBV score in the normally perfused group showed an inverse correlation with PVR; however, that in poorly perfused group did not. Microvasculopathy might contribute to severe hemodynamics, apart from pulmonary vascular obstruction. In our experience, more than half of treatment-naïve CTEPH patients have microvasculopathy.


2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Nobutaka Ikeda ◽  
Masaru Hatano ◽  
Takeshi Nagamatsu ◽  
Masato Nakamura

AbstractBackgroundTo date, the management of pregnancy in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and the associated risk of maternal mortality have not been established. Although balloon pulmonary angioplasty (BPA) in patients with CTEPH is still an emerging procedure, this approach represents a promising alternative to pulmonary endarterectomy (PEA), especially in patients with inoperable CTEPH.Case summaryWe present a case of a 34-year-old woman with CTEPH who desired to have a child. Right heart catheterization showed a mean pulmonary artery pressure of 54 mmHg. Since the lesions were observed in the distal part of subsegmental pulmonary arteries, there was no indication for PEA. After improvement in her haemodynamic status by BPA, she became pregnant. At 40 weeks of gestation, a normal baby was delivered vaginally. Both mother and baby have made satisfactory progress.DiscussionIn cases in which the haemodynamic status is improved by effective BPA, pregnancy and childbirth may be possible, even in patients with CTEPH.


2020 ◽  
Vol 10 (3) ◽  
pp. 204589402091788
Author(s):  
Christoph B. Wiedenroth, MD ◽  
Andreas J. Rieth, MD ◽  
Steffen Kriechbaum, MD ◽  
H.-Ardeschir Ghofrani, MD ◽  
Andreas Breithecker, MD ◽  
...  

Background * These authors contributed equally as last authors. Balloon pulmonary angioplasty is an evolving, interventional treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary hypertension at rest as well as exercise capacity is considered to be relevant outcome parameters. The aim of the present study was to determine whether measurement of pulmonary hemodynamics during exercise before and six months after balloon pulmonary angioplasty have an added value. Methods From March 2014 to July 2018, 172 consecutive patients underwent balloon pulmonary angioplasty. Of these, 64 consecutive patients with inoperable CTEPH underwent a comprehensive diagnostic workup that included right heart catheterization at rest and during exercise before balloon pulmonary angioplasty treatments and six months after the last intervention. Results Improvements in pulmonary hemodynamics at rest and during exercise, in quality of life, and in exercise capacity were observed six months after balloon pulmonary angioplasty: WHO functional class improved in 78% of patients. The mean pulmonary arterial pressure (mPAP) at rest was reduced from 41 ± 9 to 31 ± 9 mmHg (p < 0.0001). The mPAP/cardiac output slope decreased after balloon pulmonary angioplasty (11.2 ± 25.6 WU to 7.7 ± 4.1 WU; p < 0.0001), and correlated with N-terminal fragment of pro-brain natriuretic peptide (p = 0.035) and 6-minute walking distance (p = 0.01). Conclusions Exercise right heart catheterization provides valuable information on the changes of pulmonary hemodynamics after balloon pulmonary angioplasty in inoperable CTEPH patients that are not obtainable by measuring resting hemodynamics.


2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Juanni Gong ◽  
Yuanhua Yang ◽  
Jianfeng Wang ◽  
Yidan Li ◽  
Xiaojuan Guo ◽  
...  

Background. MicroRNAs (miRNAs) play an important role in the pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH). However, the potential correlation between miRNA expression and the severity of CTEPH remains unclear. Our previous study indicated that miRNAs hsa-let-7b-3p, hsa-miR-17-5p, hsa-miR-106b-5p, hsa-miR-3202, hsa-miR-665, and hsa-miR-93-5p are closely involved in CTEPH. This study assessed the associations between the expression levels of these miRNAs and clinical parameters in CTEPH patients. Methods. A total of eight CTEPH patients and eight healthy adults as a reference group were included, and clinical data including total protein (TP), albumin (Alb), lactate dehydrogenase (LDH), hydroxybutyrate dehydrogenase (HBDH), uric acid (UA), and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels were collected. Right heart catheterization was conducted to obtain hemodynamic data including cardiac index (CI). The expression levels of let-7b-3p, miR-17-5p, miR-106b-5p, miR-3202, miR-665, and miR-93-5p were measured by quantitative real-time PCR (qPCR). Correlation analysis was applied to estimate the associations between miRNA expression levels and clinical parameters in CTEPH patients. Results. Serum TP and Alb levels were decreased, while LDH, HBDH, and UA levels were increased in CTEPH patients compared with the reference group ( P < 0.05 ). miR-3202 and miR-665 were upregulated, whereas let-7b-3p, miR-17-5p, miR-106b-5p, and miR-93-5p were downregulated in CTEPH patients relative to the reference group ( P < 0.05 ). miR-93-5p expression was positively correlated with NT-proBNP level and negatively correlated with CI ( P < 0.05 ). Moreover, let-7b-3p tended to be positively correlated with mean pulmonary arterial pressure. Conclusions. miR-93-5p expression was associated with the severity of CTEPH and could act as a potential predictor of high-risk CTEPH.


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.


2014 ◽  
Vol 12 (4) ◽  
pp. 179-185
Author(s):  
Coen van Kan ◽  
Paul Bresser

Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as precapillary pulmonary hypertension (PH) as assessed by right heart catheterization, and results from incomplete resolution of the vascular obstruction associated with acute pulmonary embolism (PE). Pulmonary thromboendarterectomy (PTE) is the therapy of choice for CTEPH patients with surgically accessible thrombi. Although associated with potential risks, PTE has been found to improve, and in many cases normalize pulmonary hemodynamics, functional status, and long-term survival. It is critical to undergo careful diagnosis and preoperative selection of patients who will most likely benefit from surgery. We have used published literature along with our personal experiences to review diagnosis of CTEPH and evaluation in advance of the PTE procedure. In patients with PH or suspected PH, a complete diagnostic workup should be performed to identify the underlying etiology of the disease. Pulmonary angiography and right heart catheterization are the preferred assessment tools to diagnose CTEPH. PTE remains the treatment of choice, and for further evaluation of operability and preoperative risk patients should be referred to a CTEPH expert center.


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.


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