Predicting survival in pulmonary arterial hypertension in the UK

Lee, Wai-Ting Nicola; Liu, Yi; Sheares, Karen; Pepke‐Zaba, Joanna; Peacock, Andrew J.; Johnson, Martin K.

doi:10.1183/09031936.00196611

Cited by 111 publications

(82 citation statements)

References 36 publications

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“…In multifactorial analysis the only significant factor was right atrial pressure, which reveals the important role played by the right ventricle in predicting prognosis [20]. This was confirmed in numerous publications [16,21,22], and right atrial pressure was incorporated to guidelines as a one of the haemodynamic determinants of prognosis [1]. Noteworthy is the assessment of cardiac output, which, from the pathophysiological point of view, should be a significant independent risk factor for mortality, but this has been demonstrated only in one out of four analyses.…”

Section: Risk Factorssupporting

confidence: 59%

“…Therefore, in assessing of the prognosis the often-repeated non-invasive measurement of cardiac output would be of greater importance than the initial catheterisation diagnostic test. Survival analyses based on data from large registries for adults with PAH allowed the creation of risk calculators [21,22]. They include various parameters -the United Kingdom [21]: age, gender, aetiology, 6MWT, and haemodynamic studies (right atrial pressure and the cardiac output); United States [22]: aetiology, age, sex and co-morbidities, the WHO-FC, blood pressure and heart function, 6MWT, BNP or NT-proBNP, pericardial effusion in echo, pulmonary function tests (DLCO), and haemodynamic studies (right atrial pressure and pulmonary resistance).…”

Section: Risk Factorsmentioning

confidence: 99%

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Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience

et al. 2016

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A b s t r a c tBackground: Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfavourable prognosis. Aim:To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004-2013. Methods:The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH -n = 23 (42%), PAH associated with systemic-to-pulmonary shunts -n = 17 (31%), and PAH after corrective cardiac surgery -n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy.Results: During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03).Conclusions: Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.

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Section: Risk Factorssupporting

confidence: 59%

Section: Risk Factorsmentioning

confidence: 99%

Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience

et al. 2016

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confidence: 99%

Registries for paediatric pulmonary hypertension

Hansmann

Hoeper

2013

Eur Respir J

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@ERSpublications Paediatric pulmonary arterial hypertension should be treated specifically and differently from adult pulmonary arterial hypertension http://ow.ly/mzPDT Pulmonary arterial hypertension (PAH) is a progressive, angio-obliterative disease leading to increased pulmonary vascular resistance, right heart failure, and death in ,25-60% of PAH patients 5 years after diagnosis [1][2][3]. The estimated prevalence of PAH is 15-50 cases per million adults [4-6] and 2-16 cases per million children [7][8][9]. In certain at-risk groups, however, the occurrence of PAH is substantially higher. For example, the prevalence is 0.5% in HIV-infected patients [10] and 4-6% in schistosomiasis [11]. These diseases are far more common in developing countries with limited health care and, thus, the real burden of PAH worldwide is probably underestimated.Historically, extrapolation from adult pulmonary hypertension studies has been used to make assumptions on the disease in children; however, this approach is neither validated nor appropriate [12]. The recent comprehensive analysis of paediatric registries allowed predictions on the epidemiology, clinical practice and outcome of PAH in childhood. In France, for example, the prevalence of paediatric PAH was estimated to be 2.2 cases per million children [7]. In the UK, the incidence and prevalence of idiopathic PAH (IPAH) was 0.48 and 2.1 per million children, respectively [8]. In the Netherlands, the incidence and prevalence of IPAH was 0.7 and 4.4 per million children, whereas PAH associated with congenital heart disease (PAH-CHD) had an incidence of 2.2 and prevalence of 15.6 per million [9]. Of note, the PAH disease spectrum may somewhat differ in the individual tertiary centre and in multicentre registries [13]. In a British cohort study, the 5-year survival for children with IPAH was only 75% with a freedom from death or transplantation of only 57% (1986 and 2000 with follow-up to 2007) [8].Patients, family members and healthcare providers should be aware of the unique features of paediatric pulmonary hypertensive vascular disease (PPHVD) [14,15]. These include the fetal origins, developmental and adaptive aspects of pulmonary vascular disease (PVD) and right ventricular dysfunction (RVD) in both childhood-and adult-onset disease. The proposal for a new classification of PPHVD (Panama, 2011 [14]) aims to incorporate such specific paediatric aspects that are not adequately addressed in the current classification of pulmonary hypertension (Dana Point, CA, USA, 2008) [16]. The Panama proposal of PPHVD is still very new, not broadly (internationally) accepted yet, and probably will undergo further modifications, according to international meetings (e.g., the PH World Symposium, Nice, France, 2013) and feedback from healthcare providers.

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“…The major strength of the 6MWT is the ability of a baseline measurement to predict prognosis in IPAH which has resulted in its inclusion in several PH registry models for predicting survival including the French PH registry [138], the risk calculator derived from REVEAL (the North American database) [139] and the Scottish Composite Score [140]. This was also demonstrable in a meta-analysis of the many treatment trials in PAH [141].…”

Section: Field Exercise Testsmentioning

confidence: 84%

“…This was also demonstrable in a meta-analysis of the many treatment trials in PAH [141]. It retains significance in multivariate analyses even when other strong predictors are included such as patient demographics [127,140], baseline haemodynamics from right heart catheterisation or CMR [140,142,143], CPET variables [92,113] and presence of pericardial effusion [127]. Absolute values of 6MWD measured following PAH treatment retain prognostic power but do not improve on the baseline measurement [144].…”

Section: Field Exercise Testsmentioning

confidence: 90%

The Role of Exercise Testing in the Modern Management of Pulmonary Arterial Hypertension

Johnson

Thomson

2014

Diseases

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A culture of exercise testing is firmly embedded in the management of pulmonary arterial hypertension (PAH) but its clinical relevance and utility have recently been under some debate. The six minute walk test (6MWT) has been used as a primary outcome measure to enable the licensing of many of the medications used for this condition. Recent reviews have questioned the validity of this test as a surrogate of clinical outcomes. At the same time, other questions are emerging where exercise testing may be the solution. With the rise in understanding of genetic markers of idiopathic PAH (IPAH), the screening of an otherwise healthy population for incipient pulmonary hypertension (PH) will be required. The proliferation in treatment choices and identification of populations with PH where PAH treatment is not indicated, such as left heart and lung disease, requires more definitive differentiation from patients with PAH. There is a continuing question about the existence and clinical relevance of exercise induced PAH as a cause of unexplained dyspnoea and fatigue and as a latent phase of resting PH. This review presents a summary and critical analysis of the current role of exercise testing in PAH and speculates on future trends.

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Predicting survival in pulmonary arterial hypertension in the UK

Cited by 111 publications

References 36 publications

Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience

Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience

Registries for paediatric pulmonary hypertension

The Role of Exercise Testing in the Modern Management of Pulmonary Arterial Hypertension

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