Cardiopulmonary exercise testing in adults with congenital heart disease

Abstract

Adults with congenital heart disease (CHD) are at high risk for morbidity and mortality. Identifying those who are at highest risk of complications can be challenging, in part because self-reported functional status is not a reliable indicator of physiological status. Individuals with CHD may present with exercise limitation due to a variety of cardiac and non-cardiac causes. Cardiopulmonary exercise testing (CPET) provides an integrated assessment of cardiac, pulmonary, and metabolic function and can identify the source of exercise limitation in many patients. Because CPET parameters have also been associated with outcome in adults with CHD, CPET has recently emerged as an important prognostic indicator in this population.

Keywords::

• cardiopulmonary exercise testing
• congenital heart disease
• exercise capacity
• heart failure
• pediatrics

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Cardiopulmonary exercise testing (CPET) provides an integrated assessment of the cardiac, pulmonary and metabolic response to exercise.

• In contrast to NYHA class, CPET allows for the objective assessment of functional capacity.

• Adults living with congenital heart disease (ACHD) have a consistently lower objective exercise capacity than normal individuals despite a lack of self-reported symptoms.

• CPET is a helpful tool to identify cardiac sources of dyspnea in ACHD.

• Chronotropic and ventilatory abnormalities are common in this population and can be detected by CPET.

• The degree of impairment in exercise capacity is correlated with the type of the congenital heart disease, with the worst exercise capacity seen in those with Eisenmenger syndrome and complex disease.

• Lower peak oxygen consumption has been correlated with cardiac morbidity and mortality.

• Chronotropic and ventilatory abnormalities have also been correlated with adverse outcome in some studies.

• CPET may be used to identify ACHD patients who are at higher risk of adverse outcomes during pregnancy.