The study of risk in pulmonary arterial hypertension

Rubin, Lewis J.; Simonneau, Gérald; Badesch, David B.; Galiè, Nazzareno; Humbert, Marc; Keogh, Anne; Massaro, Joseph; Cerinic, Marco Matucci; Sitbon, Olivier; Kymes, Steven M.

doi:10.1183/09059180.00003712

Cited by 15 publications

(12 citation statements)

References 22 publications

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“…Despite the several above-described limitations, we believe that this study is unique and that it would be difficult to perform such a study prospectively, as administering upfront combination therapy for PAH (24) and balloon pulmonary angioplasty (BPA) for CTEPH before clinical worsening develops has recently become prevalent, especially in Japan. However, limitations on access to PAH medications in patients with less severe disease, and those receiving combination therapy have previously been reported in the UK and Australia, as a consequence of the cost-effectiveness of these therapies (25). In our study, no patients with the TT genotype exhibited clinical worsening with monotherapy.…”

Section: Discussionmentioning

confidence: 42%

Polymorphism of the G Protein β3 Subunit Gene Influences the Efficacy of Sildenafil in Patients with Pulmonary Hypertension

et al. 2014

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Objective The C825T polymorphism in the G protein β3 subunit gene (GNB3) influences the efficacy of sildenafil in patients with erectile dysfunction. The effects of this polymorphism on the therapeutic response to sildenafil in patients with pulmonary hypertension remains unknown. To investigate whether the GNB3 C825T polymorphism is associated with the clinical efficacy of sildenafil in patients with pulmonary hypertension. Methods Fifty-nine patients (age: 55.6±13.3 [SD] yrs., mean pulmonary arterial pressure (Ppa): 52±11 mmHg) with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension were treated with sildenafil. The pre-and post-treatment parameters, including pulmonary hemodynamics measured using right heart catheterization, the systolic pulmonary arterial pressure estimated on Doppler echocardiography (sPA), the six-minute walk distance (6MWD) and freedom from clinical worsening, were compared between the patients with the TT and CT/CC genotypes. Results The pretreatment parameters were not significantly different between the two groups, with the exception of a lower mean Ppa in the TT group. The post-treatment World Health Organization (WHO) class was significantly better (p=0.03) and the 6MWD values trended toward improvement in the TT genotype patients compared with that observed in the CC/CT genotype patients (p=0.05). The time to clinical worsening was significantly longer in the TT genotype patients than in the CC/CT genotype patients (3-year freedom from clinical worsening: 83.1% vs. 46.0%, p=0.02), while the TT genotype was found to be a significant predictor of freedom from clinical worsening, even after adjusting for the baseline mean Ppa. Conclusion The GNB3 C825T polymorphism influences the efficacy of sildenafil in patients with pulmonary hypertension.

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Section: Discussionmentioning

confidence: 42%

Polymorphism of the G Protein β3 Subunit Gene Influences the Efficacy of Sildenafil in Patients with Pulmonary Hypertension

et al. 2014

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“…22 Thus, there is a "need for assessment at multiple follow-up time-points (dynamic assessment) to evaluate the impact of time-dependent variables on clinical outcomes." 26 The change in CO and PVR after 3-4 months may be one of these follow-up variables.…”

Section: Discussionmentioning

confidence: 99%

Short‐Term Improvement in Pulmonary Hemodynamics is Strongly Predictive of Long‐Term Survival in Patients with Pulmonary Arterial Hypertension

et al. 2013

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Hemodynamic measurements provide important parameters for determining prognosis and therapy in patients with pulmonary arterial hypertension (PAH). Current guidelines do not incorporate the possible predictive value of individual changes in hemodynamic variables during the disease time course, and there is no consensus about the time point for hemodynamic reevaluation. We aimed to assess the long-term prognostic value of shortterm changes in hemodynamic parameters. The study included 122 patients with PAH from the Giessen Pulmonary Hypertension Registry who underwent hemodynamic evaluation at baseline and at 16 weeks (±2.5 standard deviations [SDs]; range: 4 -29 weeks) after initial assessment. At baseline, mean pulmonary vascular resistance (PVR) was 1,109 dyn s cm -5 , and 82% of patients were in World Health Organization (WHO) functional class III or IV. Fifty patients died, and 2 underwent lung transplantation during long-term observation (≤10 years; mean: 4.7 years). Kaplan-Meier estimates for transplant-free survival were 93.3%, 76.1%, 57.8%, and 53.1% at 1, 3, 5, and 7 years, respectively. When assigned to prognostic groups, improvements in cardiac output of >0.22 L min -1 (hazard ratio [HR]: 2.05; P ¼ 0:015) and a decrease in PVR of >176 dyn s cm -5 (HR: 1.89; P ¼ 0:044) at 4-29 weeks were associated with long-term transplant-free survival. Changes in mean pulmonary arterial pressure did not predict long-term prognosis. Of 2 noninvasive parameters assessed in this selected patient group, change in WHO functional class, but not in 6-minute walk distance, predicted long-term prognosis. Short-term assessment of changes in hemodynamic parameters at 16 weeks AE 2:5 SDs after initial invasive evaluation is useful to determine long-term prognosis in patients with PAH.

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“…However, it should be noted that the tools and parameters discussed in this article have mainly been evaluated in the context of baseline risk assessment and very few data are available on their use in the on-going assessment of risk. Tools for dynamic assessment at multiple follow-up time-points would benefit evaluation of the impact of time-dependent variables on clinical outcomes [51].…”

Section: Practical Considerations For Risk Assessmentmentioning

confidence: 99%

Risk assessment in pulmonary arterial hypertension

Raina

Humbert

2016

Eur Respir Rev

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Regular patient assessment is essential for the management of chronic diseases, such as pulmonary arterial hypertension (PAH). Comprehensive patient assessment and risk stratification in PAH are important to guide treatment decisions and to monitor disease progression as well as patients' response to treatment. Approaches for assessing risk in PAH patients include the use of risk variables, as recommended in the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines, and the application of risk equations and scores, such as the French registry risk equation and the REVEAL registry risk score. Risk stratification and risk scores are both useful predictors of survival on a population basis, and provide an estimate for individual patients' risk. The 2015 ESC/ERS PH guidelines recommend regular assessment of multiple variables at an expert centre. The respective merits and limitations of different risk assessment methods in PAH are discussed in this article, as well as some considerations that can be taken into account in the future development of risk assessment tools.

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The study of risk in pulmonary arterial hypertension

Cited by 15 publications

References 22 publications

Polymorphism of the G Protein β3 Subunit Gene Influences the Efficacy of Sildenafil in Patients with Pulmonary Hypertension

Polymorphism of the G Protein β3 Subunit Gene Influences the Efficacy of Sildenafil in Patients with Pulmonary Hypertension

Short‐Term Improvement in Pulmonary Hemodynamics is Strongly Predictive of Long‐Term Survival in Patients with Pulmonary Arterial Hypertension

Risk assessment in pulmonary arterial hypertension

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