scholarly journals Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401983221
Author(s):  
Vasile Foris ◽  
Luka Brcic ◽  
Philipp Douschan ◽  
Gabor Kovacs ◽  
Elvira Stacher-Priehse ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Lung Fibrosis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Lung Function Test ◽  
Right Heart Catheterization ◽  
Smoking History ◽  
Heart Catheterization ◽  
Lung Pathology ◽  
Function Test

The diagnosis of idiopathic pulmonary arterial hypertension (iPAH) is complex and, besides invasive hemodynamic evaluation, includes several diagnostic steps to exclude any underlying diseases. The role of a decreased diffusion capacity of the lung for carbon monoxide (DLCO) is a matter of discussion. Here, we present a 76-year-old man with a smoking history of 30 pack-years who was diagnosed with iPAH after chronic thromboembolic pulmonary hypertension was excluded based on a negative perfusion scan, an underlying heart disease was excluded based on echocardiography and right heart catheterization, and a significant lung disease was excluded based on lung function test (FVC = 101% predicted, FEV1 = 104% predicted, FEV1/FVC = 77, TLC = 97% predicted) and thin-slice computed tomography (CT) scan. Just DLCO was reduced to 40% predicted, suggesting a possible structural lung disease. Postmortem examination demonstrated severe interstitial lung fibrosis combined with microscopic emphysema. This indicates that both CT imaging and pulmonary function test may be insensitive to a diffuse peripheral combined pattern of fibrosis and emphysema and that DLCO may be the only sensitive marker of this significant lung pathology.

scholarly journals Successful right heart remodelling and subsequent pregnancy in a patient with chronic thromboembolic pulmonary hypertension undergoing balloon pulmonary angioplasty: a case report

2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Nobutaka Ikeda ◽  
Masaru Hatano ◽  
Takeshi Nagamatsu ◽  
Masato Nakamura
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Subsequent Pregnancy ◽  
Heart Catheterization ◽  
Right Heart ◽  
Promising Alternative ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

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.

scholarly journals Exercise right heart catheterization before and after balloon pulmonary angioplasty in inoperable patients with chronic thromboembolic pulmonary hypertension

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 ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Exercise Capacity ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Walking Distance ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

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.

scholarly journals Expression of miR-93-5p as a Potential Predictor of the Severity of Chronic Thromboembolic Pulmonary Hypertension

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Juanni Gong ◽  
Yuanhua Yang ◽  
Jianfeng Wang ◽  
Yidan Li ◽  
Xiaojuan Guo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Mirna Expression ◽  
Reference Group ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Clinical Parameters ◽  
Potential Predictor ◽  
Expression Levels ◽  
Thromboembolic Pulmonary Hypertension

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.

scholarly journals Pulmonary hypertension in patients with interstitial pneumonia with autoimmune features

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402094411
Author(s):  
Bashar N. Alzghoul ◽  
Robert Hamburger ◽  
Thomas Lewandowski ◽  
Brandon Janssen ◽  
Daniel Grey ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Regression Analysis ◽  
Interstitial Lung Disease ◽  
Right Ventricular ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Linear Regression Analysis ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart

Pulmonary hypertension in interstitial lung diseases is associated with increased mortality and hospitalizations and reduced exercise capacity. Interstitial pneumonia with autoimmune features (IPAF) is a recently described interstitial lung disease. The characteristics of pulmonary hypertension in IPAF patients are unknown. We sought to characterize patients with IPAF based on their echocardiographic probability of pulmonary hypertension and compare patients with and without pulmonary hypertension identified by right heart catheterization. We conducted a retrospective study of patients seen in the interstitial lung disease clinic from 2015 to 2018. Forty-seven patients with IPAF were identified. Patients were classified into low, intermediate and high echocardiographic pulmonary hypertension probabilities. A sub-group analysis of patients with pulmonary hypertension and without pulmonary hypertension (IPAF-PH vs. IPAF-no PH) identified by right heart catheterization was also performed. Linear regression analysis was performed to study the association between 6-min-walk-distance (6MWD) and pulmonary vascular resistance (PVR) while adjusting for age and body mass index. Right ventricular hypertrophy (>5 mm), right ventricular enlargement (>41 mm) and right ventricular systolic dysfunction defined as fractional area change% ≤35 was present in 76%, 24%, and 39% of patients, respectively. Pulmonary hypertension was identified in 12.7% of patients. IPAF-PH patients had higher mean pulmonary artery pressure and lower cardiac output compared to the IPAF-no PH group (34 mmHg vs. 19 mmHg, p = 0.002 and 4.0 vs. 5.7 L/min, p = 0.023, respectively). Lower 6MWD was associated with higher PVR on regression analysis ( p = 0.002). Pulmonologists should be aware that a significant number of IPAF patients may develop pulmonary hypertension. Reduced 6MWD may suggest the presence of pulmonary hypertension in IPAF patients.

Prevalence of Coronary Artery to Pulmonary Artery Collaterals in Patients with Chronic Thromboembolic Pulmonary Hypertension: Retrospective Analysis from a Single Center

2016 ◽  
Vol 66 (02) ◽  
pp. 180-186 ◽  
Author(s):  
Bulent Mutlu ◽  
Ashok Paudel ◽  
Cigdem Ileri ◽  
Halil Atas ◽  
Bedrettin Yildizeli ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Coronary Artery ◽  
Pulmonary Artery ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Thromboembolic Pulmonary Hypertension ◽  
Study Population ◽  
Coronary Angiograms

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.

scholarly journals Predictors of Mortality in Patients with Interstitial Lung Disease-Associated Pulmonary Hypertension

2020 ◽  
Vol 9 (12) ◽  
pp. 3828
Author(s):  
Esam H. Alhamad ◽  
Joseph G. Cal ◽  
Nuha N. Alrajhi ◽  
Waleed M. Alharbi
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Cox Regression ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Systolic Pulmonary Artery Pressure ◽  
Hemodynamic Parameters ◽  
Rank Analysis ◽  
Minute Walk

Background: Pulmonary hypertension (PH) is a well-established complication in interstitial lung disease (ILD) patients. The aim of this study is to investigate the physiological and hemodynamic parameters that predict mortality in patients with ILD-PH. Methods: Consecutive ILD patients who underwent right heart catheterization (n = 340) were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival. Results: In total, 96 patients had PH and an additional 56 patients had severe PH. The overall survival of idiopathic pulmonary fibrosis (IPF) patients with PH was significantly worse than the survival of patients with other types of ILD with PH (p < 0.0001 by log-rank analysis). Patients with a reduced diffusing capacity of the lung for carbon monoxide (DLco) (<35% predicted), six-minute walk test final oxygen saturation by pulse oximetry (SpO2) < 88% and pulmonary vascular resistance ≥4.5 Wood units in the ILD-PH cohort had significantly worse survival. IPF diagnosis, forced vital capacity, DLco, systolic pulmonary artery pressure and cardiac index were identified as independent predictors of survival among the ILD-PH cohort. Conclusions: Patients with ILD-PH have poor prognosis. Physiological and hemodynamic parameters were important factors independently associated with outcome.

scholarly journals Diagnosis and Preoperative Evaluation of Chronic Thromboembolic Pulmonary Hypertension

2014 ◽  
Vol 12 (4) ◽  
pp. 179-185
Author(s):  
Coen van Kan ◽  
Paul Bresser
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Assessment Tools ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Term Survival ◽  
Preoperative Selection ◽  
Thromboembolic Pulmonary Hypertension

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.

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