PMID- 31419027 OWN - NLM STAT- MEDLINE DCOM- 20200316 LR - 20210110 IS - 1752-699X (Electronic) IS - 1752-6981 (Print) IS - 1752-6981 (Linking) VI - 13 IP - 11 DP - 2019 Nov TI - Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study. PG - 693-699 LID - 10.1111/crj.13076 [doi] AB - INTRODUCTION: Current guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH-CHD). OBJECTIVES: The aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH-CHD patients during 12-month follow-up. METHODS: We reviewed 288 Chinese PAH-CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low-risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow-up were collected. The association between low-risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed. RESULTS: There were 105 PAH-CHD patients included in the final analysis. Twenty-nine patients had AEs defined as death, initiation of new or combined medication treatment, or re-hospitalisation because of the PAH worsening. Among the low-risk criteria, WHO/NYHA functional class, 6-minute walking distance (6MWD), NT-proBNP and SvO(2) were significantly different between AE and AE-free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03-0.19, P < 0.001) and NT-proBNP (HR = 0.35, 95% CI: 0.16-0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low-risk criteria at diagnosis discriminated the risk of AEs (P < 0.001). CONCLUSIONS: Among the low-risk criteria proposed by current guidelines, 6MWD and NT-proBNP predicted AEs independently for PAH-CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH-CHD patients. CI - (c) 2019 The Authors. The Clinical Respiratory Journal published by John Wiley & Sons Ltd. FAU - Deng, Xiaoxian AU - Deng X AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Jin, Bowen AU - Jin B AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Li, Shanshan AU - Li S AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Li, Yaping AU - Li Y AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Zhou, Hongmei AU - Zhou H AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Wu, Yang AU - Wu Y AD - Imaging Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Yan, Menghuan AU - Yan M AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Hu, Yuanping AU - Hu Y AD - Laboratory of Molecular Cardiology, Wuhan Asia Heart hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Qiu, Qiu AU - Qiu Q AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Zhang, Gangcheng AU - Zhang G AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. FAU - Zheng, Xuan AU - Zheng X AUID- ORCID: 0000-0002-8433-6198 AD - Congenital Heart Disease Center, Wuhan Asia Heart Hospital, Wuhan University of Science and Technology, Wuhan, China. AD - Laboratory of Molecular Cardiology, Wuhan Asia Heart hospital, Wuhan University of Science and Technology, Wuhan, China. LA - eng GR - 2017CX4-B01/Innovation funding of Wuhan Asia Heart hospital/ GR - WJ2018H0045/Hubei Province Health and Family Planning Scientific Research Project/ GR - WX15D10/Wuhan Innovational funding/ PT - Journal Article DEP - 20190829 PL - England TA - Clin Respir J JT - The clinical respiratory journal JID - 101315570 RN - 0 (Peptide Fragments) RN - 0 (pro-brain natriuretic peptide (1-76)) RN - 114471-18-0 (Natriuretic Peptide, Brain) RN - S88TT14065 (Oxygen) SB - IM MH - Adult MH - China/epidemiology MH - Eisenmenger Complex/diagnosis/epidemiology MH - Female MH - Health Plan Implementation/*methods MH - Heart Defects, Congenital/complications/*epidemiology/metabolism/mortality MH - Humans MH - Incidence MH - Infant MH - Male MH - Middle Aged MH - Natriuretic Peptide, Brain/blood MH - Oxygen/blood MH - Peptide Fragments/blood MH - Practice Guidelines as Topic MH - Predictive Value of Tests MH - Prognosis MH - Pulmonary Arterial Hypertension/*epidemiology/etiology/mortality/physiopathology MH - Retrospective Studies MH - Risk Assessment/*methods MH - Walk Test/methods PMC - PMC6899897 OTO - NOTNLM OT - congenital heart disease OT - guidelines OT - prognosis OT - pulmonary arterial hypertension OT - risk stratification COIS- The authors declared that they have no conflicts of interest with the contents of this article. EDAT- 2019/08/17 06:00 MHDA- 2020/03/17 06:00 PMCR- 2019/12/08 CRDT- 2019/08/17 06:00 PHST- 2019/02/05 00:00 [received] PHST- 2019/06/28 00:00 [revised] PHST- 2019/07/29 00:00 [accepted] PHST- 2019/08/17 06:00 [pubmed] PHST- 2020/03/17 06:00 [medline] PHST- 2019/08/17 06:00 [entrez] PHST- 2019/12/08 00:00 [pmc-release] AID - CRJ13076 [pii] AID - 10.1111/crj.13076 [doi] PST - ppublish SO - Clin Respir J. 2019 Nov;13(11):693-699. doi: 10.1111/crj.13076. Epub 2019 Aug 29.