Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

Boucly, Athénaïs; Weatherald, Jason; Savale, Laurent; Jaïs, Xavier; Cottin, Vincent; Prévôt, Grégoire; Picard, François; Groote, Pascal de; Jevnikar, Mitja; Bergot, Emmanuel; Chaouat, Ari; Chabanne, Céline; Bourdin, Arnaud; Parent, Florence; Montani, David; Simonneau, Gérald; Humbert, Marc; Sitbon, Olivier

doi:10.1183/13993003.00889-2017

Cited by 587 publications

(779 citation statements)

References 22 publications

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“…A study from the French registry demonstrated clearly that in order to achieve a better long-term prognosis, patients need to have as many parameters as possible in the low-risk category. In this study, PAH patients who achieved only one or two low-risk criteria during the first year of PAH treatment had worse outcomes than those who achieved three or four low-risk criteria [18].…”

Section: Role Of Risk Assessment In Pahmentioning

confidence: 85%

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Gaine

McLaughlin

2017

Eur Respir Rev

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Recent advances in the treatment of pulmonary arterial hypertension (PAH) have led to improved patient outcomes. Multiple PAH therapies are now available and optimising the use of these drugs in clinical practice is vital. In this review, we discuss the management of PAH patients in the context of current treatment guidelines and supporting clinical evidence. In clinical practice, considerable emphasis is placed on the importance of making treatment decisions guided by each patient's risk status, which should be assessed using multiple prognostic parameters. As PAH is a progressive disease, regular assessments are essential to ensure that any change in risk is detected in a timely manner and treatment is adjusted accordingly. With the availability of therapies that target three different pathogenic pathways, combination therapy is now the standard of care. For most patients, this involves dual combination therapy with agents targeting the endothelin and nitric oxide pathways. Therapies targeting the prostacyclin pathway should be added for patients receiving dual combination therapy who do not achieve a low-risk status. There is also a need for a holistic approach to treatment beyond pharmacological therapies. Implementation of all these approaches will ensure that PAH patients receive maximal benefit from currently available therapies.

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Section: Role Of Risk Assessment In Pahmentioning

confidence: 85%

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Gaine

McLaughlin

2017

Eur Respir Rev

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“…Since then, the age of IPAH patients has increased considerably, at least in Western countries [4,[8][9][10][11]. In Germany, the mean age of patients newly diagnosed with IPAH in 2014 was 65 years [12].…”

Section: Increasing Age and Mortality In Ipahmentioning

confidence: 99%

“…Recent studies from the French PH registry and from the European PH registry COMPERA have confirmed that IPAH patients who meet 3-4 distinct low risk criteria with targeted therapies have 5-year survival rates of 95% or higher [3,4]. Unfortunately, this was accomplished only in 19% of the patients in the French series and 9% of the patients in COMPERA [4,5]. The reasons why the majority of patients fail to reach a low risk profile with targeted therapies have not been fully explored.…”

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confidence: 99%

Age, risk and outcomes in idiopathic pulmonary arterial hypertension

2018

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The current European pulmonary hypertension (PH) guidelines recommend a risk-based therapeutic approach to patients with idiopathic pulmonary arterial hypertension (IPAH) [1,2]. The goal is reaching and maintaining a low risk profile. Recent studies from the French PH registry and from the European PH registry COMPERA have confirmed that IPAH patients who meet 3-4 distinct low risk criteria with targeted therapies have 5-year survival rates of 95% or higher [3,4]. Unfortunately, this was accomplished only in 19% of the patients in the French series and 9% of the patients in COMPERA [4,5]. The reasons why the majority of patients fail to reach a low risk profile with targeted therapies have not been fully explored.In this issue of the European Respiratory Journal, HJALMARSSON et al.[6] present data from the Swedish PH registry showing that the likelihood that IPAH patients meet a low risk profile with targeted therapies diminishes with increasing age. Almost 80% of patients aged between 18 and 45 years reached the low risk category with PAH treatment, compared with approximately 20% of patients aged ⩾65 years and less than 10% of patients aged ⩾75 years. Improvements in risk category were rarely observed in patients aged ⩾65 years. Similar data have been recently reported from COMPERA [3]. In the present Swedish series, the 5-year transplantation-free survival decreased from 88% in patients aged 18-45 years to 36% in patients aged ⩾75 years.The high mortality risk of older IPAH patients is probably due to various reasons including comorbidities, unique disease phenotypes, different treatment patterns, and a diminished response to PAH targeted therapies. These factors are tightly interconnected but can be assessed separately. Increasing age and mortality in IPAHThe mean age of patients enrolled between 1981 and 1985 in the US National Institutes of Health (NIH) registry was 36 years at the time of diagnosis; only 9% of the patients were more than 60 years of age [7]. Since then, the age of IPAH patients has increased considerably, at least in Western countries [4,[8][9][10][11]. In Germany, the mean age of patients newly diagnosed with IPAH in 2014 was 65 years [12]. In a recent Swedish registry, the mean age at diagnosis was 69 years [13]. Naturally, older patients have a higher mortality risk in general, but it has already been shown that older patients (>65 years) diagnosed with IPAH have a significantly higher mortality risk than younger patients (18-65 years), even when adjusted for their respective statistical life expectancies [14].

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“…BOUCLY et al [15] concluded that the number of low-risk criteria present at diagnosis and at first re-evaluation (median 4.4 months) discriminated risk of death or lung transplantation.…”

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confidence: 99%

“…In the French registry (n=1017), BOUCLY et al [15] studied incident patients with idiopathic, heritable or drug-induced PAH. The number of low-risk criteria (FC I or II, 6MWD >440 m, mRAP <8 mmHg and CI ⩾2.5 L·min…”

mentioning

confidence: 99%

Assessing risk in pulmonary arterial hypertension: what we know, what we don't

et al. 2017

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Despite improvement in short-term survival, pulmonary arterial hypertension (PAH) remains an incurable disease with an unacceptable median survival of 7 years [1]. In the USA, the survival rates for PAH patients awaiting transplant continue to mirror the 2.5-year survival rate in the pretreatment era of the disease [2]. Despite treatment, PAH patients continue to experience disease progression and increased rates of hospitalisations due to right heart failure. Importantly, right heart failure hospitalisations in PAH occur at rates that are comparable to patients with left heart disease, particularly in those aged greater than 65 [3] More so, morbid events in PAH, notably hospitalisations, herald disease progression and early mortality [4,5]. Hence, along with advancing PAH treatment options, appropriate and accurate risk prediction is essential to halt disease progression and make individualised treatment decisions.In a progressive disease like PAH, early and accurate risk prediction allows for the identification of patients who are more likely to progress rapidly, "rapid progressors". Risk stratification is especially important in settings where clinical PAH experience is not available and could facilitate early referral to a PAH centre. A risk stratification algorithm could also offer a more individualised treatment strategy for PAH patients; by identifying risk stratum, guiding clinical decision making and informing treatment options and goals. Risk prediction modelling can help physicians allocate treatment resources in settings where they are scarce. They can also be used to inform patients of their prognosis thereby allowing them to make informed decisions about treatment options. If widely adopted, appropriate risk prediction provides an opportunity to learn about various risk phenotypes in PAH, enhance consistency of treatment approaches across practitioners and assist in the timely referral for lung transplantation. Lastly, risk model-derived equations can enhance clinical study design both by selecting the appropriate study cohort and serving as a study end-point.Statistical models are often used to predict the probability that an individual with a given set of risk factors will experience a health outcome, usually termed an "event" [6]. In PAH, it is widely agreed that a

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Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

Cited by 587 publications

References 22 publications

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Age, risk and outcomes in idiopathic pulmonary arterial hypertension

Assessing risk in pulmonary arterial hypertension: what we know, what we don't

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