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Heart Online First, published on May 1, 2014 as 10.1136/heartjnl-2014-305494 Pulmonary vascular disease

ORIGINAL ARTICLE

C-reactive protein in adults with pulmonary arterial hypertension associated with congenital heart disease and its prognostic value Giancarlo Scognamiglio,1,2 Aleksander Kempny,1,3,4 Laura C Price,1,5 Rafael Alonso-Gonzalez,1,3 Philip Marino,1,3 Lorna Swan,1,3 Michele D’ Alto,2 James Hooper,1 Michael A Gatzoulis,1,3 Konstantinos Dimopoulos,1,3 Stephen J Wort1,3,5 1

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK 2 Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy 3 NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, London, UK 4 Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany 5 National Heart and Lung Institute, Imperial College School of Medicine, London, UK Correspondence to Dr Stephen Wort, Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, UK; [email protected] GS and AK contributed equally. KD and SJW are equal last authors. Received 5 January 2014 Revised 28 March 2014 Accepted 8 April 2014

ABSTRACT Objectives To assess the relationship of C-reactive protein (CRP) to clinical outcome and mortality in adults with pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). Background Approximately 5–10% of adults with congenital heart disease (ACHD) develop PAH, which in turn is associated with substantial morbidity and mortality. Although CRP is known to predict outcome in idiopathic PAH, little is known regarding its prognostic value in CHD-PAH. Methods We obtained and analysed 1936 CRP values in a total of 225 adults with CHD-PAH (median age at study entry 34.0 years (27.0–41.7); 32.9% male, 35% with Down syndrome), performed over a 12-year period. High CRP values related to infection or blood transfusions were excluded from the analysis. Results During a median follow-up of 4.8 years (1149 patients-years), 50 patients died. The median CRP concentration on the last assessment was 5.0 mg/L (IQR 2.0–10.0), higher in deceased patients compared with survivors (11.5 mg/L (6.0–23.0) vs 4.0 mg/L (1.5–8.0), p

C-reactive protein in adults with pulmonary arterial hypertension associated with congenital heart disease and its prognostic value.

To assess the relationship of C-reactive protein (CRP) to clinical outcome and mortality in adults with pulmonary arterial hypertension (PAH) associat...
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