The role of cardiopulmonary exercise testing in predicting mortality and morbidity in people with congenital heart disease: a systematic review and meta-analysis.

Curtis A Wadey,Rod S Taylor,Guido E Pieles,Dan Mihai Dorobantu,Alan R Barker,Max E Weston,Craig A Williams,Graham Stuart

doi:10.1093/eurjpc/zwab125

Abstract

The role of cardiopulmonary exercise testing (CPET) in predicting major adverse cardiovascular events (MACE) in people with congenital heart disease (ConHD) is unknown. A systematic review with meta-analysis was conducted to report the associations between CPET parameters and MACE in people with ConHD. Electronic databases were systematically searched on 30 April 2020 for eligible publications. Two authors independently screened publications for inclusion, extracted study data, and performed risk of bias assessment. Primary meta-analysis pooled univariate hazard ratios across studies. A total of 34 studies (18 335 participants; 26.2 ± 10.1 years; 54% ± 16% male) were pooled into a meta-analysis. More than 20 different CPET prognostic factors were reported across 6 ConHD types. Of the 34 studies included in the meta-analysis, 10 (29%), 23 (68%), and 1 (3%) were judged as a low, medium, and high risk of bias, respectively. Primary univariate meta-analysis showed consistent evidence that improved peak and submaximal CPET measures are associated with a reduce risk of MACE. This association was supported by a secondary meta-analysis of multivariate estimates and individual studies that could not be numerically pooled. Various maximal and submaximal CPET measures are prognostic of MACE across a variety of ConHD diagnoses. Further well-conducted prospective multicentre cohort studies are needed to confirm these findings.

Highlights

Cardiopulmonary exercise testing is recommended as a part of routine care in people with congenital heart disease (ConHD) by the European Society of Cardiology.[3]
People with ConHD can have reduced Cardiorespiratory fitness (CRF) compared to healthy controls,[6,7,8] and a 2018 systematic review reported that reduced CRF is associated with major adverse cardiovascular events (MACE) in people with Fontan circulations.[9]
Peak V_O2 was inversely associated with MACE in all ConHD subtypes, with an 18% reduction in Fontan circulation, a 12% reduction in a mixed ConHD cohort, a 16% reduction in transposition of the great arteries (TGAs), and a 6% reduction in Tetralogy of Fallot (ToF) (0.89–0.99; 7 studies)

Summary

Introduction

Cardiopulmonary exercise testing is recommended as a part of routine care in people with congenital heart disease (ConHD) by the European Society of Cardiology.[3]. Congenital heart disease represents a spectrum of cardiac (and/or intrathoracic vessel) defects present at birth,[4] and is the most common birth defect with approximately 1% of the global population affected.[5] People with ConHD can have reduced CRF compared to healthy controls,[6,7,8] and a 2018 systematic review reported that reduced CRF is associated with major adverse cardiovascular events (MACE) in people with Fontan circulations.[9] Major adverse cardiovascular events is a widely used composite clinical endpoint in cardiovascular research, encompassing a range of morbidities and mortalities It does not have a standardized definition, it allows researchers to capture patient-important outcomes, which may vary due to specific populations or interventions

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