The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial Hypertension

Benza, Raymond L.; Gomberg‐Maitland, Mardi; Miller, Dave P.; Frost, Adaani E.; Frantz, Robert P.; Foreman, Aimee J.; Badesch, David B.; McGoon, Michael D.

doi:10.1378/chest.11-0676

Cited by 463 publications

(493 citation statements)

References 20 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: 52%

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: 52%

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

et al. 2016

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“…Because of the lack of blood and urine samples, we could not explore the mechanisms that might have contributed to these higher rates of death in those patients with CKD. Furthermore, this study (similar to any observational study) was adjusted for potential confounders, but we lacked details relating to socioeconomic data, functional status of the patients, and left ventricular mass index, which are factors that may contribute to mortality in PH (29,30). The Kidney Disease Outcomes Quality Initiative definition was used to define stages 3 and 4 CKD for this study's purposes, and patients who could have had underlying longstanding cardiorenal syndrome (for .3 months) could not be excluded.…”

Section: Discussionmentioning

confidence: 99%

Presence and Outcomes of Kidney Disease in Patients with Pulmonary Hypertension

Navaneethan

Wehbe

Heresi³

et al. 2014

Clinical Journal of the American Society of Nephrology

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Background and objectives Pulmonary hypertension is associated with higher mortality rates. The associations of nondialysis-dependent CKD and all-cause mortality in patients with pulmonary hypertension were studied.Design, setting, participants, & measurements The study population included those patients who underwent right heart catheterization for confirmation of pulmonary hypertension between 1996 and January 2011. Pulmonary hypertension was defined as the presence of mean pulmonary artery pressure$25 mmHg at rest measured by right heart catheterization. CKD was defined as the presence of two measurements of eGFR,60 ml/min per 1.73 m 2 90 days apart. The risk factors associated with CKD as well as the association between CKD and death in those patients with pulmonary hypertension using logistic regression and Cox proportional hazard models were examined.Results Of 1088 patients with pulmonary hypertension, 388 (36%) patients had CKD: 340 patients had stage 3 CKD, and 48 (4%) patients had stage 4 CKD. In the multivariable analysis, older age, higher hemoglobin, and higher mean right atrial pressures were independently associated with CKD. During a median follow-up of 3.2 years (interquartile range=1.5-5.6 years), 559 patients died. After adjusting for relevant covariates, presence of stage 3 CKD (hazard ratio, 1.37; 95% confidence interval, 1.14 to 1.66) and stage 4 CKD (hazard ratio, 2.69; 95% confidence interval, 1.88 to 3.86) was associated with all-cause mortality in those patients with pulmonary hypertension. When eGFR was examined as a continuous measure, a 5 ml/min per 1.73 m 2 lower eGFR was associated with a 5% (95% confidence interval, 1.03 to 1.07) higher hazard for death. This higher risk with CKD was similar irrespective of demographics, left ventricular function, and pulmonary capillary wedge pressure. ConclusionIn a clinical population referred for right heart catheterization, presence of CKD was associated with higher all-cause mortality in those patients with pulmonary hypertension. Mechanisms that may underlie these associations warrant additional studies.

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“…Despite aggressive treatment, she still reported NYHA-III/IV symptoms, an elevated N-terminal-pro-B type natriuretic peptide, and a Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk score of 11, stratifying her as high risk with a potential 1-year mortality risk of 15-30% (3,15). She was listed for lung transplantation.…”

Section: Patientmentioning

confidence: 99%