Objective The prevalence of heart failure (HF) among adult patients with congenital heart disease (ACHD) is rising. Right ventricle (RV) exercise reserve and its relationship to outcomes have not been characterised. We aim to evaluate the prognostic impact of impaired RV reserve in an ACHD population referred for cardiopulmonary exercise testing (CPET).
Methods This retrospective study evaluates patients with ACHD who underwent CPET (n=147) with first-pass radionuclide ventriculography at a single tertiary care centre. RV reserve was categorised as normal, mild to moderately or severely impaired. The primary composite clinical outcome included clinical right HF, arrhythmia, transplantation or death.
Results Patients were median age 41±13 years, 50% were female and median follow-up was 1.1 (IQR: 0.7–2.0) years. Exercise RV reserve was impaired in 103 patients (70%), of whom 32% were asymptomatic. Resting RV systolic function poorly predicted RV reserve, with 52% of patients with severe impairment having a qualitatively normal echocardiographic assessment. The severely impaired reserve group had lower peak oxygen consumption (VO2)(17.2 vs 22.5 mL/kg/min, p<0.0001) compared with the normal reserve group, and was more likely to develop the composite outcome (48% vs 9%, log-rank p<0.001). Severely impaired RV reserve predicted event-free survival after adjusting for peak VO2, age, sex, RV pathology, QRS duration, New York Heart Association class, resting RV ejection fraction and RV dilation by echocardiography or MRI (HR 3.7, 95% CI 1.1 to 13.0, p=0.039).
Conclusion Impaired RV reserve, occurred in asymptomatic patients, was not well predicted by resting systolic function assessment, and strongly predicted adverse cardiovascular outcomes.
- congenital heart disease
- heart failure
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Contributors DDY and GDL contributed to the design of the study. DDY, ACSS and ABB helped conduct the study. All authors contributed equally to the drafting and revision of contents of the manuscript. All authors approved the final version of the manuscript.
Funding DDY is supported in this manuscript preparation by the Massachusetts General Hospital, Office of Diversity and Inclusion Clinician Teacher Development Award and ACSS was supported by the NIH T32HL007604 training grant in cardiovascular research. GDL was supported by NIH R01HL131029.
Competing interests None declared.
Patient consent Not required.
Ethics approval This study was approved by the institutional review board.
Provenance and peer review Not commissioned; externally peer reviewed.
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