Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension

S. C. Mathai; M. Bueso; L. K. Hummers; D. Boyce; N. Lechtzin; J. Le Pavec; A. Campo; H. C. Champion; T. Housten; P. R. Forfia; A. L. Zaiman; F. M. Wigley; R. E. Girgis; P. M. Hassoun

doi:10.1183/09031936.00074309

Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension

S. C. Mathai, M. Bueso, L. K. Hummers, D. Boyce, N. Lechtzin, J. Le Pavec, A. Campo, H. C. Champion, T. Housten, P. R. Forfia, A. L. Zaiman, F. M. Wigley, R. E. Girgis, P. M. Hassoun

School of Medicine

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95 Scopus citations

Abstract

N-terminal pro-brain natriuretic peptide (NT-proBNP) is a marker of neurohormonal activation that is useful in the diagnosis and prognosis of various forms of pulmonary arterial hypertension (PAH). We sought to characterise and compare NT-proBNP in a cohort of PAH related to systemic sclerosis (PAH-SSc) and idiopathic PAH (IPAH) patients. NT-proBNP levels, collected from PAH-SSc and IPAH patients followed prospectively, were compared and correlated with haemodynamic variables. Cox proportional hazard models were created to assess the predictive value of NT-proBNP. 98 patients (55 PAH-SSc, 43 IPAH) were included. Haemodynamics were similar, except for lower mean pulmonary arterial pressure in PAH-SSc. NT-proBNP levels were significantly higher in PAH-SSc (3,419±3,784 versus 1,393±1,633 pg?mL ^-1; p<0.01) and were more closely related to haemodynamics in PAH-SSc than IPAH. 28 patients died. NT-proBNP predicted survival (hazard ratio (HR) 3.18; p<0.01) in the overall cohort; however, when stratified by group, predicted survival only in PAH-SSc (HR 3.07, p<0.01 versus 2.02, p=0.29 in IPAH). This is the first description showing NT-proBNP levels are 1) significantly higher in PAH-SSc than IPAH despite less severe haemodynamic perturbations, and 2) stronger predictors of survival in PAH-SSc, suggesting that neurohormonal regulation may differ between PAH-SSc and IPAH. Future studies to define pertinent mechanisms are warranted. Copyright

Original language	English (US)
Pages (from-to)	95-104
Number of pages	10
Journal	European Respiratory Journal
Volume	35
Issue number	1
DOIs	https://doi.org/10.1183/09031936.00074309
State	Published - Jan 2010

Keywords

Prognostic markers
Pulmonary hypertension
Scleroderma

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine

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10.1183/09031936.00074309

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Mathai, S. C., Bueso, M., Hummers, L. K., Boyce, D., Lechtzin, N., Le Pavec, J., Campo, A., Champion, H. C., Housten, T., Forfia, P. R., Zaiman, A. L., Wigley, F. M., Girgis, R. E., & Hassoun, P. M. (2010). Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension. European Respiratory Journal, 35(1), 95-104. https://doi.org/10.1183/09031936.00074309

Mathai, SC, Bueso, M, Hummers, LK, Boyce, D, Lechtzin, N, Le Pavec, J, Campo, A, Champion, HC, Housten, T, Forfia, PR, Zaiman, AL, Wigley, FM, Girgis, RE & Hassoun, PM 2010, 'Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension', European Respiratory Journal, vol. 35, no. 1, pp. 95-104. https://doi.org/10.1183/09031936.00074309

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abstract = "N-terminal pro-brain natriuretic peptide (NT-proBNP) is a marker of neurohormonal activation that is useful in the diagnosis and prognosis of various forms of pulmonary arterial hypertension (PAH). We sought to characterise and compare NT-proBNP in a cohort of PAH related to systemic sclerosis (PAH-SSc) and idiopathic PAH (IPAH) patients. NT-proBNP levels, collected from PAH-SSc and IPAH patients followed prospectively, were compared and correlated with haemodynamic variables. Cox proportional hazard models were created to assess the predictive value of NT-proBNP. 98 patients (55 PAH-SSc, 43 IPAH) were included. Haemodynamics were similar, except for lower mean pulmonary arterial pressure in PAH-SSc. NT-proBNP levels were significantly higher in PAH-SSc (3,419±3,784 versus 1,393±1,633 pg?mL -1; p<0.01) and were more closely related to haemodynamics in PAH-SSc than IPAH. 28 patients died. NT-proBNP predicted survival (hazard ratio (HR) 3.18; p<0.01) in the overall cohort; however, when stratified by group, predicted survival only in PAH-SSc (HR 3.07, p<0.01 versus 2.02, p=0.29 in IPAH). This is the first description showing NT-proBNP levels are 1) significantly higher in PAH-SSc than IPAH despite less severe haemodynamic perturbations, and 2) stronger predictors of survival in PAH-SSc, suggesting that neurohormonal regulation may differ between PAH-SSc and IPAH. Future studies to define pertinent mechanisms are warranted. Copyright",

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AU - Hummers, L. K.

AU - Boyce, D.

AU - Lechtzin, N.

AU - Le Pavec, J.

AU - Campo, A.

AU - Champion, H. C.

AU - Housten, T.

AU - Forfia, P. R.

AU - Zaiman, A. L.

AU - Wigley, F. M.

AU - Girgis, R. E.

AU - Hassoun, P. M.

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Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension

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