Improvement after bariatric surgery in chronic thromboembolic pulmonary hypertension: a novel treatment target

2021 ◽  
Vol 14 (3) ◽  
pp. e228358
Author(s):  
Anika Vaidy ◽  
Paul Forfia ◽  
Jeremy Mazurek ◽  
Anjali Vaidya
Keyword(s):  
Bariatric Surgery ◽  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Walk Distance ◽  
Right Heart ◽  
Exertional Dyspnea ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

A 66-year-old man with a medical history significant for multiple pulmonary embolism and morbid obesity was evaluated for chronic thromboembolic pulmonary hypertension. Echocardiogram and right heart catheterisation were significant for severe pulmonary hypertension. Therefore, he was started on pulmonary hypertension medical therapy with riociguat and ambrisentan, in addition to anticoagulation. He experienced a dramatic clinical response to medical therapy. Despite haemodynamic improvement, the patient remained symptomatic with significant fatigue, exertional dyspnea and poor functional status as highlighted by a 6 min walk distance of only 128 m. Patient was referred for bariatric surgery with a gastric sleeve, after which he successfully lost 95 lbs in 6 months. Postoperative right heart catheterisation demonstrated normal pulmonary  vascular resistance and cardiac output. His echocardiogram revealed normal right ventricular size and function. His 6 min walk distance also nearly quadrupled from 128 to 512 m, consistent with WHO Functional Class I.

scholarly journals Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature

2017 ◽  
Vol 49 (2) ◽  
pp. 1601792 ◽  
Author(s):  
Yvonne M. Ende-Verhaar ◽  
Suzanne C. Cannegieter ◽  
Anton Vonk Noordegraaf ◽  
Marion Delcroix ◽  
Piotr Pruszczyk ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Meta Analysis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Population Level ◽  
Right Heart ◽  
Random Effects Models ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is relevant for management decisions but is currently unknown.We performed a meta-analysis of studies including consecutive PE patients followed for CTEPH. Study cohorts were predefined as “all comers”, “survivors” or “survivors without major comorbidities”. CTEPH incidences were calculated using random effects models.We selected 16 studies totalling 4047 PE patients who were mostly followed up for >2-years. In 1186 all comers (two studies), the pooled CTEPH incidence was 0.56% (95% CI 0.1–1.0). In 999 survivors (four studies) CTEPH incidence was 3.2% (95% CI 2.0–4.4). In 1775 survivors without major comorbidities (nine studies), CTEPH incidence was 2.8% (95% CI 1.5–4.1). Both recurrent venous thromboembolism and unprovoked PE were significantly associated with a higher risk of CTEPH, with odds ratios of 3.2 (95% CI 1.7–5.9) and 4.1 (95% CI 2.1–8.2) respectively. The pooled CTEPH incidence in 12 studies that did not use right heart catheterisation as the diagnostic standard was 6.3% (95% CI 4.1–8.4).The 0.56% incidence in the all-comer group probably provides the best reflection of the incidence of CTEPH after PE on the population level. The ∼3% incidences in the survivor categories may be more relevant for daily clinical practice. Studies that assessed CTEPH diagnosis by tests other than right heart catheterisation provide overestimated CTEPH incidences.

scholarly journals Chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 53 (1) ◽  
pp. 1801915 ◽  
Author(s):  
Nick H. Kim ◽  
Marion Delcroix ◽  
Xavier Jais ◽  
Michael M. Madani ◽  
Hiromi Matsubara ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Anticoagulation Therapy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Treatment Decision ◽  
Right Heart Failure ◽  
Vascular Involvement ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Pulmonary Angiogram ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.

scholarly journals ERS Statement on Chronic Thromboembolic Pulmonary Hypertension

2020 ◽  
pp. 2002828
Author(s):  
Marion Delcroix ◽  
Adam Torbicki ◽  
Deepa Gopalan ◽  
Olivier Sitbon ◽  
Frederikus A. Klok ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Acute Pulmonary Embolism ◽  
Current Knowledge ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Treatment Modalities ◽  
Reference Network ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with a secondary microvasculopathy of vessels less than 500 µm, leads to increased pulmonary vascular resistance and progressive right heart failure. The mechanism responsible for the transformation of red clots into fibrotic material remnants has not yet been elucidated. In patients with pulmonary hypertension, the diagnosis is suspected when a ventilation/perfusion lung scan shows mismatched perfusion defects and confirmed by right heart catheterisation and vascular imaging. Today, in addition to lifelong anticoagulation, treatment modalities include surgery, angioplasty and medical treatment according to the localisation and characteristics of the lesions.This Statement outlines a review of the literature and current practice concerning diagnosis and management of CTEPH. It covers the definitions, diagnosis, epidemiology, follow up after acute pulmonary embolism, pathophysiology, treatment by pulmonary endarterectomy, balloon pulmonary angioplasty, drugs and their combination, rehabilitation and new lines of research in CTEPH.It represents the first collaboration of the European Respiratory Society (ERS), the International CTEPH Association (ICA) and the European Reference Network (ERN)-Lung in the pulmonary hypertension domain. The Statement summarises current knowledge but does not make formal recommendations for clinical practice.

scholarly journals Multicentre observational screening survey for the detection of CTEPH following pulmonary embolism

2018 ◽  
Vol 51 (4) ◽  
pp. 1702505 ◽  
Author(s):  
Nicolas Coquoz ◽  
Daniel Weilenmann ◽  
Daiana Stolz ◽  
Vladimir Popov ◽  
Andrea Azzola ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Observational Study ◽  
Acute Pulmonary Embolism ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Second Step ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension ◽  
Screening Survey

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe complication of pulmonary embolism. Its incidence following pulmonary embolism is debated. Active screening for CTEPH in patients with acute pulmonary embolism is yet to be recommended.This prospective, multicentre, observational study (Multicentre Observational Screening Survey for the Detection of Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Following Pulmonary Embolism (INPUT on PE); ISRCTN61417303) included patients with acute pulmonary embolism from 11 centres in Switzerland from March 2009 to November 2016. Screening for possible CTEPH was performed at 6, 12 and 24 months using a stepwise algorithm that included a dyspnoea phone-based survey, transthoracic echocardiography, right heart catheterisation and radiological confirmation of CTEPH.Out of 1699 patients with pulmonary embolism, 508 patients were assessed for CTEPH screening over 2 years. CTEPH incidence following pulmonary embolism was 3.7 per 1000 patient-years, with a 2-year cumulative incidence of 0.79%. The Swiss pulmonary hypertension registry consulted in December 2016 did not report additional CTEPH cases in these patients. The survey yielded 100% sensitivity and 81.6% specificity. The second step echocardiography in newly dyspnoeic patients showed a negative predictive value of 100%.CTEPH is a rare but treatable disease. A simple and sensitive way for CTEPH screening in patients with acute pulmonary embolism is recommended.

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

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.

scholarly journals Right heart catheterisation: best practice and pitfalls in pulmonary hypertension

2015 ◽  
Vol 24 (138) ◽  
pp. 642-652 ◽  
Author(s):  
Stephan Rosenkranz ◽  
Ioana R. Preston
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Best Practice ◽  
Data Interpretation ◽  
Volume Measurement ◽  
Right Heart ◽  
Heart Catheterisation ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial

Right heart catheterisation (RHC) plays a central role in identifying pulmonary hypertension (PH) disorders, and is required to definitively diagnose pulmonary arterial hypertension (PAH). Despite widespread acceptance, there is a lack of guidance regarding the best practice for performing RHC in clinical practice. In order to ensure the correct evaluation of haemodynamic parameters directly measured or calculated from RHC, attention should be drawn to standardising procedures such as the position of the pressure transducer and catheter balloon inflation volume. Measurement of pulmonary arterial wedge pressure, in particular, is vulnerable to over- or under-wedging, which can give rise to false readings. In turn, errors in RHC measurement and data interpretation can complicate the differentiation of PAH from other PH disorders and lead to misdiagnosis. In addition to diagnosis, the role of RHC in conjunction with noninvasive tests is widening rapidly to encompass monitoring of treatment response and establishing prognosis of patients diagnosed with PAH. However, further standardisation of RHC is warranted to ensure optimal use in routine clinical practice.

P2774Galectin-3, GDF-15, and ST2 in noninvasive assessment of myocardial remodelling in chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S D Kriechbaum ◽  
K Peters ◽  
R Ajnwojner ◽  
J S Wolter ◽  
M Haas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Disease Severity ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Control Group ◽  
Right Heart ◽  
Non Invasive ◽  
Galectin 3 ◽  
Myocardial Remodelling ◽  
Thromboembolic Pulmonary Hypertension

Abstract Background In chronic thromboembolic pulmonary hypertension (CTEPH), pulmonary artery obstruction leads to impaired pulmonary hemodynamics and secondary right heart failure, which is highly predictive of outcome. Thus, the extent of myocardial -especially right heart- remodelling is an indicator of disease severity. Purpose The aim of the present study was to assess growth differentiation factor-15 (GDF-15), galectin-3, and suppression of tumorigenicity 2 (ST2) as non-invasive biomarkers of myocardial remodelling in patients suffering from CTEPH. Methods We analysed the serum levels of GDF-15, galectin-3 and ST2 in a cohort of 64 CTEPH patients and in a control group of 25 patients without cardiovascular disease. The biomarker levels were further correlated with clinical, laboratory, and hemodynamic data, including 6-minute walking distance (6-MWD), N-terminal pro-brain natriuretic peptide (NT-proBNP), mean pulmonary artery pressure (meanPAP), pulmonary vascular resistance (PVR), and right atrial pressure (RAP). Results The biomarker levels in the control group were: galectin-3: 3.5 ng/l (IQR 2.7–4.0), GDF-15: 92.6 pg/ml (IQR 78.5–129.1), and ST2: 48.65 ng/l (IQR 35.5–57.0). CTEPH patients had higher levels of GDF-15 (196.7 pg/ml; IQR 128.4–302.8; p<0.001) and ST2 (52.6 ng/l; IQR 44.5–71.9; p=0.05) but not galectin-3 (3.4 ng/l; IQR 2.7–4.3; p=0.84). In the CTEPH cohort, patients with a meanPAP >35 mmHg (GDF-15: p=0.01; ST2: p=0.04) and patients with a PVR >500 dyn sec cm–5 (GDF-15: p=0.004; ST2: p=0.002) had significantly increased biomarker levels. For the detection of a meanPAP >35mmHg, ROC analysis revealed an AUC of 0.71 for GDF-15 and 0.67 for ST2. The level of GDF-15 correlated with the level of NT-proBNP (rrs=0.69; p≤0.001) and the RAP (rrs=0.54; p≤0.001) and inversely with the 6-MWD (rrs=−0.47; p≤0.001). The level of ST2 correlated with the level of NT-proBNP (rrs=0.67; p≤0.001) and the RAP (rrs=0.54; p≤0.001) and inversely with the 6-MWD (rrs=-0.31; p=0.02). Conclusion Our results demonstrate that GDF-15 and ST2, non-invasive biomarkers of myocardial remodelling, are significantly elevated in patients suffering from CTEPH. The correlation of biomarker levels with established outcome predictors suggests a use as indicators of disease severity.

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