Top Things to Know: Cardiopulmonary Exercise Testing Across the Lifespan in Congenital Heart Disease
Published: January 09, 2025
Prepared by Adam W. Powell, MD, MS
- Patients with congenital heart disease congenital heart disease (HD) are living longer secondary to medical and surgical advances; however, there remain significant quality of life and functional status concerns.
- Cardiopulmonary exercise testing (CPET) is a tool that qualitatively evaluates dynamic cardiovascular function, but many health care professionals who care for these patients need more confidence in interpreting these findings. This document focuses on the key CPET findings in those with congenital HD across their lifespan.
- CPET can give robust prognostic information in congenital HD, particularly in those with a Fontan circulation, Ebstein anomaly, and tetralogy of Fallot.
- In addition to measuring fitness, CPET can provide data on the pulmonary responses to exercise and can be used to assess for ischemia, the presence of arrhythmias, and to evaluate chronotropy.
- Patients with prior atrial and ventricular septal defects and patent ductus arteriosus have slightly lower fitness than those without congenital HD.
- CPET is used in patients with D-transposition of the great vessels (D-TGA) repaired via a Mustard or Senning operation to evaluate for oxygen desaturation secondary to baffle leaks, conduction and rhythm abnormalities, and diminished peak oxygen consumption (VO2 peak) due to impaired stroke volume and blunted heart rate response. In patients with D-TGA repaired via an arterial switch, CPET can help assess for ischemia in those with exertional symptoms, assess chronotropy, and can be used for surveillance over time.
- In patients with tetralogy of Fallot, CPET is a vital tool to assist with decision-making for pulmonary valve replacement. Additionally, a VO2 peak <65% and the slope of minute ventilation/expired carbon dioxide (VE/VCO2 slope) ≥31 are associated with greater reintervention rates, cardiac-related events, and death.
- Patients with a Fontan circulation tend to have markedly reduced VO2 peak, chronotropic incompetence, a reduced oxygen pulse reflecting attenuated stroke volume, elevated VE/VCO2 slope, and exertional hypoxemia. Information from CPET can assist with clinical decision-making regarding cardiac transplantation, valve interventions, and elimination of right-to-left shunts.
- Patients with Ebstein anomaly often have markedly abnormal VO2 peak with <60% of predicted being associated with adverse clinical outcomes, including death and unplanned hospitalization.
- CPET can often unmask exercise-induced hypertension in patients with repaired coarctation of the aorta, which can result in earlier initiation of antihypertensive treatment.
Citation
Cifra B, Cordina RL, Gauthier N, Murphy LC, Pham TD, Veldtman GR, Ward K, White DA, Paridon SM, Powell AW; on behalf of the American Heart Association Council on Lifelong Congenital Heart Disease and Heart Health in the Young, Congenital Cardiac Defect Committee; the Council on Cardiovascular Radiology and Intervention; the Council on Clinical Cardiology; and the Council on Cardiovascular and Stroke Nursing. Cardiopulmonary exercise testinterpretation across the lifespan in congenital heart disease: a scientific statement from the American HeartAssociation. J Am Heart Assoc. January 9, 2025. doi: 10.1161/JAHA.124.038200