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Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: a retrospective cohort study.

Clin Respir J. 2019 Aug 16;:

Authors: Deng X, Jin B, Li S, Li Y, Zhou H, Wu Y, Yan M, Hu Y, Qiu Q, Zhang G, Zheng X

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 ESC guidelines and the 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-hospitalization due to the PAH worsening. Among the low-risk criteria, WHO/NYHA functional class, 6 MWD, NT-proBNP, and SvO2 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).
CONCLUSION: Among the low-risk criteria proposed by current guidelines, 6 MWD 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. This article is protected by copyright. All rights reserved.

PMID: 31419027 [PubMed – as supplied by publisher]

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