Guidelines for the diagnosis and treatment of pulmonary hypertension

Hoeper, Marius M.; Humbert, Marc; Torbicki, Adam; Vachiéry, Jean‐Luc; Barberà, Joan Albert; Beghetti, Maurice; Corris, Paul A.; Gaine, Seán; Gibbs, J. Simon R.; Gómez-Sánchez, Miguel Ángel; Jondeau, Guillaume; Klepetko, Walter; Peacock, Andrew J.; Rubin, Lewis J.; Zellweger, M.; Simonneau, Gérald; Auricchio, Angelo; Bax, Jeroen J.; Ceconi, Claudio; Dean, Veronica; Filippatos, Gerasimos; Funck‐Brentano, Christian; Hobbs, Richard; Kearney, Peter; McDonagh, Theresa A.; McGregor, Keith; Popescu, Bogdan A.; Reiner, Željko; Sechtem, Udo; Tendera, Michał; Vardas, Panos; Widimský, Petr; Andreotti, Felicita; Aschermann, Michael; Asteggiano, Riccardo; Benza, Ray; Berger, Rolf M. F.; Bonnet, Damien; Delcroix, Marion; Howard, Luke; Kitsiou, Anastasia; Lang, Irene; Maggioni, Aldo; Nielsen‐Kudsk, Jens Erik; Park, Myung; Perrone‐Filardi, Pasquale; Domenech, María Teresa Subirana; Vonk‐Noordegraaf, Anton; Zamorano, José Luís

doi:10.1183/09031936.00139009

Cited by 1,081 publications

(531 citation statements)

References 274 publications

(200 reference statements)

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“…CHD‐APAH patients were more breathless with higher MRC dyspnea scores and higher Borg scores during the 6‐minute walk test, and these patients were confirmed to be more functionally limited with higher WHO‐FC and reduced 6MWD 48. This present study demonstrates that lung restriction correlates with 6MWD and Borg score during the 6‐minute walk test, which is in conflict with a previous study that did not demonstrate such a relationship 42.…”

Section: Discussioncontrasting

confidence: 99%

Lung Function, Inflammation, and Endothelin‐1 in Congenital Heart Disease–Associated Pulmonary Arterial Hypertension

Low

George

Howard

et al. 2018

JAHA

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BackgroundBreathlessness is the most common symptom in people with pulmonary arterial hypertension and congenital heart disease (CHD‐APAH), previously thought to be caused by worsening PAH, but perhaps also by inflammation and abnormalities of lung function. We studied lung function and airway inflammation in patients with CHD‐APAH and compared the results with controls.Methods and ResultsSixty people were recruited into the study: 20 CHD‐APAH, 20 CHD controls, and 20 healthy controls. Spirometry, gas transfer, whole body plethysmography and lung clearance index, 6‐minute walk distance, and medical research council dyspnea scoring were performed. Inflammatory markers and endothelin‐1 levels were determined in blood and induced sputum. The CHD‐APAH group had abnormal lung function with lung restriction, airway obstruction, and ventilation heterogeneity. Inverse correlations were shown for CHD‐APAH between medical research council dyspnea score and percent predicted peak expiratory flow (r=−0.5383, P=0.0174), percent predicted forced expiratory flow rate at 50% of forced vital capacity (r=−0.5316, P=0.0192), as well as for percent predicted forced expiratory volume in 1 s (r=−0.6662, P=0.0018) and percent predicted forced vital capacity (r=−0.5536, P=0.0186). The CHD‐APAH patients were more breathless with lower 6‐minute walk distance (360 m versus 558 m versus 622 m, P=0.00001). Endothelin‐1, interleukin (IL)‐β, IL‐6, IL‐8, tumor necrosis factor α, and vascular endothelial growth factor were significantly higher in CHD‐APAH than controls. Serum endothelin‐1 for CHD‐APAH correlated with airflow obstruction with significant negative correlations with percent predicted forced expiratory flow rate at 75% of forced vital capacity (r=−0.5858, P=0.0135).ConclusionsRaised biomarkers for inflammation were found in CHD‐APAH. Significant abnormalities in airway physiology may contribute to the dyspnea but are not driven by inflammation as assessed by circulating and sputum cytokines. A relationship between increased serum endothelin‐1 and airway dysfunction may relate to its bronchoconstrictive properties.

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Section: Discussioncontrasting

confidence: 99%

Lung Function, Inflammation, and Endothelin‐1 in Congenital Heart Disease–Associated Pulmonary Arterial Hypertension

Low

George

Howard

et al. 2018

JAHA

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“…In the current guidelines for the management of PH, TAPSE greater than 20 mm is stated to predict favorable outcome and, conversely, that less than 15 mm is stated to indicate poor outcome [1]. Moreover, in a recent echocardiographic study, TAPSE less than 15 mm was associated with poor prognosis in patients with idiopathic pulmonary arterial hypertension [15].…”

Section: Discussionmentioning

confidence: 99%

“…We then used receiver operating characteristic (ROC) curve analysis to calculate the cut-off value of TAPSE that distinguishes between PH patients with and without RV EF reduction. The suitability of the TAPSE values that are currently used in the guidelines, i.e., 20 and 15 mm [1], were also evaluated by calculating their sensitivity, specificity, positive predictive value, and negative predictive value for the prediction of preserved or reduced RV EF.…”

Section: Discussionmentioning

confidence: 99%

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Simple prediction of right ventricular ejection fraction using tricuspid annular plane systolic excursion in pulmonary hypertension

Sato

Tsujino

Oyama‐Manabe

et al. 2013

Int J Cardiovasc Imaging

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Purpose: The present study examined whether tricuspid annular plane systolic excursion (TAPSE) can simply predict right ventricular ejection fraction (RVEF) in patients with pulmonary hypertension (PH). The TAPSE cut-off value to predict reduced RV EF was also evaluated. Methods and results:The association between TAPSE and cardiac magnetic resonance imaging (CMRI)-derived RVEF was examined in 53 PH patients. The accuracy of the prediction equation to calculate RVEF using TAPSE was also evaluated. In PH patients, TAPSE was strongly correlated with CMRI-derived RVEF in PH patients (r=0.86, p<0.0001). We then examined the accuracy of the two equations: the original regression equation (RVEF = 2.01 x TAPSE + 0.6) and the simplified prediction equation (RV EF = 2 x TAPSE). Bland-Altman plot showed that the mean difference ± limits of agreement was 0.0 ± 10.6 for the original equation and −0.6 ± 10.6 for the simplified equation. Intraclass correlation coefficient was 0.84 for the original and 0.82 for the simplified equation. Normal RV EF was considered to be ≥ 40% based on the data from 53 matched controls, and the best TAPSE cut-off value to determine reduced RV EF (< 40%) was calculated to be 19.7 mm (sensitivity 88.9%, specificity 84.6%). Conclusion:A simple equation of RV EF = 2 x TAPSE enables easy prediction of RV 3 EF using TAPSE, an easily measurable M-mode index of echocardiography. TAPSE of 19.7 mm predicts reduced RV EF in PH patients with clinically acceptable sensitivity and specificity.4

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“…[1] PH is defined as an increase in mean pulmonary arterial pressure of ≥25 mmHg at rest, assessed by right heart catheterisation (RHC). [2] Based on shared pathological and haemodynamic characteristics and therapeutic approaches, PH is currently classified into five subtypes, namely PH groups 1 -5. [3] Epidemiological studies from Western countries suggest that the prevalence of PH among African Americans is higher than that among Caucasians, [4] while in a community-based echocardiographic cohort study in Armadale, Australia, a prevalence of 9.1% and high mortality were observed.…”

Section: Researchmentioning

confidence: 99%

Gender disparities in pulmonary hypertension at a tertiary centre in Cameroon

et al. 2017

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Background. Pulmonary hypertension (PH) is a potent cause of heart failure and has been little investigated in the African setting. Objective. To investigate the effects of gender on the clinical presentation, echocardiographic features and outcomes of patients with PH in Douala, Cameroon. Methods. A prospective cohort study was conducted from March 2012 to December 2013 as part of the Pan African Pulmonary Hypertension Cohort study. PH was diagnosed by echocardiography and defined as a right ventricular systolic pressure >35 mmHg in the absence of acute right heart failure. Patients were followed up for a maximum of 12 months for primary endpoint mortality. Results. In total, 130 patients with PH were recruited; 71 (54.6%) were women. The median age was 59.2 years for men and 58.3 years for women (p=0.76). Active smoking and alcohol use were more frequent in men than women (both p<0.001), but women had greater exposure to indoor cooking fumes than men (p<0.001). Previous tuberculosis infection (11.3% v. 1.7%) and S3 gallop rhythm (30.9% v. 11.9%) were more common in women (both p<0.03). Women had a significantly higher mean systolic blood pressure (134 mmHg v. 125 mmHg; p=0.04) and pulse pressure (53.8 mmHg v. 44.9 mmHg; p=0.01) and a lower mean haemoglobin concentration (10.4 g/dL v. 12.4 g/dL; p<0.05) compared with men. Echocardiographic left ventricular (LV) systolic dysfunction was more frequent in men: mean LV ejection fraction 42.6% v. 51.5% (p=0.01) and mean fractional shortening 21.4% v. 28.6% (p=0.01). The overall mortality rate was 20.3%, and rates were similar in the two groups (Kaplan-Meier log rank 1.1; p=0.30). Conclusions. Despite differences in baseline characteristics including cardiovascular risk factors, mortality rates on follow-up were similar in men and women in this study. However, these different baseline characteristics probably suggest differences in the pathogenesis of PH in men and women in our setting that need further investigation.

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Guidelines for the diagnosis and treatment of pulmonary hypertension

Cited by 1,081 publications

References 274 publications

Lung Function, Inflammation, and Endothelin‐1 in Congenital Heart Disease–Associated Pulmonary Arterial Hypertension

Lung Function, Inflammation, and Endothelin‐1 in Congenital Heart Disease–Associated Pulmonary Arterial Hypertension

Simple prediction of right ventricular ejection fraction using tricuspid annular plane systolic excursion in pulmonary hypertension

Gender disparities in pulmonary hypertension at a tertiary centre in Cameroon

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