P4671A well-defined subgroup of chronic thromboembolic pulmonary hypertension patients are positive for antiphospholipid antibodies

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Denas ◽  
C Chen ◽  
Y Du ◽  
X Jiang ◽  
Y Zhou ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Risk Factor ◽  
Antiphospholipid Antibodies ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Thromboembolic Pulmonary Hypertension

Abstract Introduction Recurrent thromboembolism is a major risk factor for chronic thromboembolic pulmonary hypertension (CTEPH). Antiphospholipid antibodies (aPL) are a recognized risk factor for recurrent thromboembolism, however, their contribution to the development of CTEPH is currently unknown. Purpose The aim of this study was to evaluate the prevalence of antiphospholipid autoantibodies in a large cohort of consecutive patients with CTEPH. Methods Starting May 2013 to December 2018, 297 CTEPHpatients were consecutively enrolled. Diagnosis was confirmed with standardized right heart catheterization, 2D or/and 3D pulmonary angiography in all patients.All patients were also screened for thrombophilia including lupus anticoagulant IgG/IgM anticardiolipin antibodies and IgG/IgM aβ2GPI antibodies. Clinical and demographic characteristics, laboratory profile and hemodynamic data were compared between patients with aPL positive and aPL negative laboratory profiles. Results Overall, 297 consecutive patients with CTEPH were assessed for aPLs. Of these, 23 patients (7.7%) resulted positive for laboratory tests exploring aPL. Among patients with positive aPL, profile 17 (74%) were positive for all three test exploring aPL (LA+, aCL+, aB2GPI+). When compared to CTEPH patients without aPL test positivity, those positive for aPL were significantly younger (30.0 vs 55.6 years, p<0.0001), had a positive history of pulmonary embolism (95.6% vs 65.7%, p=0.003), deep venous thrombosis (78.3% vs 41.9%, p=0.0008) and were more frequently affected by other autoimmune diseases (43.5% vs 2.9%, p<0.0001). Moreover, positive aPL patients showed better hemodynamics on right heart catheterization, had more proximal lesions, and had more frequently level I and II surgical classification (82.6% vs 40.5%, p=0.0002) on pulmonary angiography. Conclusions These findings suggest that young patients with pulmonary embolism should be assessed for aPLs to guide anticoagulation therapy and prevent recurrences. Additionally, younger age, more proximal lesions and less severe hemodynamic profiles make majority patients with APS good candidates for PEA surgery.

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 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.

scholarly journals 4283Exercise right heart catheterization before and after balloon pulmonary angioplasty in inoperable patients with chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Liebetrau ◽  
S Kriechbaum ◽  
A Rieth ◽  
H A Ghofrani ◽  
M Haas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Walking Distance ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Mean

Abstract Background Balloon pulmonary angioplasty (BPA) is an evolving treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). The main indicator for success is improvement in pulmonary hemodynamics, but outcome data are heterogeneous. Purpose The aim of the present study was to evaluate pulmonary hemodynamics not only at rest, but also during exercise before and 6 months after BPA. Methods We report a prospective series of 64 consecutive patients with inoperable CTEPH who were treated interventionally with BPA. All patients underwent standardized assessment prior to the first BPA and 6 months after the last intervention. Assessment included WHO FC, Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), 6-minute walking distance (6MWD), serum levels of the N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP), and exercise RHC. Results The mean number of sessions per patient was 5.6 (± 1.3) and the mean number of pulmonary segments targeted in all interventions was 11 (± 3). BPA treatment led to improvements in pulmonary hemodynamics and exercise capacity (6MWD: 416±94 m vs. 463±96 m; p<0.0001) except for CO and CI during RHC at rest; these parameters showed improvements only during exercise RHC. MPAP at rest showed a reduction from 41±9 to 31±9 mmHg (p<0.0001) and PVR at rest decreased from 6.8±2.3 WU to 4.3±1.9 WU (p<0.0001). Further decreases were observed for systolic pulmonary arterial pressure, TPG, PVR, and TPR. Cardiac output (7.0±2.0 L/min vs. 8.3±2.0 L/min; p<0.0001) and cardiac index during exercise RHC (3.8±1.1 L/min/m2 vs. 4.4±1.1 L/min/m2; p<0.0001) improved significantly. Median NT-proBNP concentrations decreased from 741 ng/L (IQR 192–1425 ng/L) to 139 ng/L (IQR 60–266 ng/L) during BPA treatment (p<0.0001). Results from the CAMPHOR questionnaire showed significant improvements in symptoms (11±5.8 vs. 5.5±4.9, p<0.0001), activity limitations (9.2±5.6 vs. 5.2±4.5, p<0.0001), and quality of life (6.4±5.7 vs. 3.5±3.7, p<0.0001). Conclusion Significant improvements in pulmonary hemodynamics at rest and during exercise were observed 6 months after BPA. Exercise right heart catheterization offers a more discriminating evaluation of the changes in pulmonary hemodynamics after BPA.

Pulmonary hypertension

2019 ◽  
pp. 289-314
Author(s):  
James Tonkin ◽  
Kate Ryan ◽  
Brendan Madden
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Options ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Phosphodiesterase 5 ◽  
Group 1

Pulmonary hypertension is characterized by a mean pulmonary artery pressure greater than 25mmHg at rest. Pulmonary hypertension is divided into groups 1 to 5, according to the underlying cause. Group 1 diseases are characterized by plexogenic pulmonary arteriopathy, which occurs through nitric oxide, endothelin, and prostacyclin pathways. Investigations for diagnosis include echocardiography, computed tomography pulmonary angiography, and right heart catheterization. Treatment options for group 1 pulmonary hypertension include phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogues, and novel therapies such as selexipag and riociguat. However, chronic thromboembolic pulmonary hypertension is often treated surgically with pulmonary thromboendarterectomy.

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.

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