2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension

Galiè, Nazzareno; Humbert, Marc; Vachiéry, Jean‐Luc; Gibbs, Simon; Lang, Irene; Torbicki, Adam; Simonneau, Gérald; Peacock, Andrew J.; Noordegraaf, Anton Vonk; Beghetti, Maurice; Ghofrani, Hossein Ardeschir; Sánchez, Miguel; Hansmann, Georg; Klepetko, Walter; Lancellotti, Patrizio; Matucci, Marzia; McDonagh, Theresa A.; Piérard, Luc; Hoeper, Marius M.

doi:10.1016/j.rec.2016.01.002

Cited by 406 publications

(855 citation statements)

References 257 publications

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“…Nevertheless, we measured serum NP concentration which reflects an upregulated inflammatory response in the whole circulatory system; therefore, we can only hypothesize that production of NP locally in lung tissue, where specific inflammatory infiltrates in the perivascular space are found, is significantly elevated as well. According to the guidelines of the European Society of Cardiology, each patient with PAH or inoperable CTEPH should be assessed every 3–6 months to check whether the patient fulfils the criteria of disease stability [23]. Thus, we investigated NP serum concentration with regard to these validated parameters (clinical, functional, hemodynamic as well as biomarker: NT-proBNP) and we report a positive correlation of NP with NT-proBNP, and right atrium area and an inverse correlation with 6MWT and peakVO 2 assessed in CPET.…”

Section: Discussionmentioning

confidence: 99%

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Neopterin as a Biomarker in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

et al. 2018

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Background: Upregulation of the immune system is regarded to play an important role in the etiopathobiology of pulmonary arterial hypertension (PAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH). To the best of our knowledge, neopterin (NP) has never been investigated in patients with PAH and CTEPH. Objectives: The aim of the study was to evaluate the concentration of NP in blood in order to examine its impact on outcome and relationship with disease severity in that population. Methods: Serum concentration of NP was analysed prospectively in 50 patients (36 with PAH and 14 with CTEPH vs. 31 healthy controls) and assessed in relation to clinical parameters and outcome. Results: NP concentration in the PAH and CTEPH groups combined was significantly higher than in the control group (8.68, 6.39–15.03 vs. 5.14, 4.16–5.98 nmol/L, p < 0.0000001). During 9 months of follow-up, clinical deterioration occurred in 18 patients (including 8 deaths), and NP concentration in this group was higher when compared to stable patients (15.6, 8.52–25.13 vs. 7.87, 6.18–9.89, p = 0.002). The cutoff value of NP derived from ROC curve analysis was 15.3 nmol/L (p = 0.002, AUC 0.77, p = 0.0004, HR = 4.35, 95% CI 1.43–13.18, log-rank test). On Cox regression analysis, NP predicted clinical deterioration (p = 0.009, 95% CI 1.01–1.06). NP correlated positively with NT-proBNP (p < 0.001), red blood cell distribution width (p < 0.001), and right atrium area (p = 0.002) and inversely with 6-min walking test (p = 0.002) and peak oxygen consumption (p = 0.001). Conclusions: NP concentration is increased in patients with PAH and inoperable CTEPH. Elevated NP concentration is associated with adverse clinical outcomes and correlates with clinical parameters.

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Section: Discussionmentioning

confidence: 99%

“…The diagnosis of PAH and CTEPH was based on standard criteria [23] and conﬁrmed by right heart catheterization. Hemodynamic data and targeted treatment are presented in Table 2.…”

Section: Methodsmentioning

confidence: 99%

Neopterin as a Biomarker in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

et al. 2018

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“…PAH and CTEPH were diagnosed and classified according to the latest guidelines [12]. PAH subgroups with idiopathic/heritable PAH (IPAH) and associated with connective tissue disease, human immunodeficiency virus, portal hypertension, and congenital heart disease were eligible.…”

Section: Methodsmentioning

confidence: 99%

“…The following three thresholds of these outcome parameters were used to assess the achievement of treatment goals during routine follow-up: NYHA class ≤II, NTproBNP < 300 ng/L, and 6MWD > 440 m. These three parameters are, amongst others, associated with a 1-year mortality risk < 5% [12]. Patients whose parameters were available at least at the time of diagnosis and the last prevalent visit were included (main group).…”

Section: Methodsmentioning

confidence: 99%

“…More than half of CTEPH patients can be treated and substantially ameliorated with pulmonary endarterectomy [7-9] and recently, significant functional improvements have been demonstrated with balloon pulmonary angioplasty in a further subgroup [10, 11]. The remaining patients with surgically inaccessible or post-interventional residual PH due to additional distal involvement (summarised as distal CTEPH) show similarities with PAH and are accordingly treated with drug therapy [12-15]. Combination drug therapy has been recommended in the latest guidelines for patients with PAH who do not achieve therapeutic goals in terms of symptoms, exercise capacity, and haemodynamics, and studies have shown an additional benefit of combination compared to a single drug therapy [16, 17].…”

Section: Introductionmentioning

confidence: 99%

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Risk Factor Profiles Achieved with Medical Therapy in Prevalent Patients with Pulmonary Arterial and Distal Chronic Thromboembolic Pulmonary Hypertension

Bartenstein¹,

Saxer²,

Appenzeller³

et al. 2018

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Background: The latest pulmonary hypertension (PH) guidelines define therapeutic goals in terms of symptoms, exercise capacity, and haemodynamics for patients with pulmonary arterial hypertension (PAH) and recommend advanced combined medical therapy. For inoperable or post-surgical residual distal chronic thromboembolic PH (CTEPH) medical treatment is similarly advised. Objectives: We analysed whether risk factor goals are achieved and combination therapy is used in prevalent patients with PAH or distal CTEPH. Methods: PAH or distal CTEPH patients who were seen at the University Hospital Zurich during the last year were analysed in terms of demography, clinical data, medication, and therapeutic goals. Achievement of therapeutic goals was defined as New York Heart Association (NYHA) class ≤II, N-terminal pro-brain natriuretic peptide (NTproBNP) < 300 ng/L, and 6-min walking distance (6MWD) > 440 m. Results: A total of 108 PAH patients (age 59 ± 18 years, 62% female, 64% idiopathic, 36% associated) and 38 distal CTEPH patients (age 69 ± 14 years, 55% female) were included. They had been diagnosed on average 66 ± 48 months (±SD) previously. The percentage of PAH/CTEPH patients with NYHA ≤II was 52/53, respectfully, the percentage of those with NTproBNP < 300 ng/L was 61/52, and with 6MWD > 440 m 63/50. Overall, 33/31% fulfilled 3 and 29/35% fulfilled 2 of these goals. Regarding therapy, 43% of PAH patients were on double and 10% on triple combination therapy, whereas 16% of distal CTEPH patients were on double and 3% on triple combination therapy. Conclusions: In this real-life cohort of prevalent patients with PAH or distal CTEPH, targeted drug therapy resulted in an achievement of ≥2/3 predefined therapeutic goals in 2/3 of patients. Patients with PAH were more likely to receive combination therapy compared to CTEPH patients.

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Phosphodiesterase 5 inhibitors for pulmonary hypertension

Barnes

Brown

Burns

et al. 2019

Cochrane Database of Systematic Reviews

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2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension

Abstract: The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style. Either citation can be used when citing this article.

Cited by 406 publications

References 257 publications

Neopterin as a Biomarker in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Neopterin as a Biomarker in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Risk Factor Profiles Achieved with Medical Therapy in Prevalent Patients with Pulmonary Arterial and Distal Chronic Thromboembolic Pulmonary Hypertension

Phosphodiesterase 5 inhibitors for pulmonary hypertension

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