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Background:  The purpose of this study was to investigate whether patients with adult congenital heart disease (ACHD) benefit from exercise-based cardiac rehabilitation (CR) short- and long-term with regard to improvement of cardiorespiratory fitness.  Methods:  Cardiopulmonary exercise tests (CPET) completed by ACHD patients between January 2000 and October 2019 were analysed retrospectively. Linear mixed models were performed for peak oxygen consumption (VO 2 ) with patients as random effect and age, sex, disease classification, preceding surgery (≤3 months) and preceding CR (≤4 weeks for short term and &gt;4 weeks for long term) as fixed effects.  Results:  1056 CPETs of 311 ACHD patients with simple (7), moderate (188) or great (116) complexity heart defects were analysed. The 59 patients who completed a CR (median age 27 yrs, 38% females) increased peak VO 2  from before to after CR by a median of 2.7 (IQR –0.6 to 5.5) ml/kg/min. However, in the multivariate mixed model, peak VO 2  was non-significantly increased short-term after CR (β 0.8, 95%CI –0.7 to 2.4), not maintained long-term after CR (β 0.0, 95%CI –1.7 to 1.6) but significantly reduced after surgery (β –5.1, 95%CI –7.1 to –3.1). The 20 CR patients after surgery increased their peak VO 2  by 6.2 (IQR 3.6–9.5) ml/kg/min, while the 39 CR patients without preceding surgery increased it by 0.9 (IQR –1.5 to 3.1) ml/kg/min.  Conclusions:  The increase in peak VO 2  with CR was mainly due to recovery from surgical intervention. The small independent benefit from CR was not maintained long-term, highlighting the potential to improve current CR concepts in ACHD populations.

Effect of Exercise-Based Cardiac Rehabilitation on Cardiorespiratory Fitness in Adults with Congenital Heart Disease