Long-Term Reduced Exercise Capacity After Total Cavopulmonary Connection

Researchers sought to determine decades-long exercise performance in patients with congenital heart disease after total cavopulmonary connection.

After total cavopulmonary connection (TCPC), patients were found to have long-term reduced exercise capacity, and as patients aged, the decline in exercise capacity paralleled the general population, according to findings from a retrospective study published in ESC Heart Failure.

Patients (N=288) who underwent TCPC at the Charles University in Prague and Motol University Hospital in the Czech Republic between 1992 and 2016 were assessed for clinical characteristics and underwent cardiopulmonary exercise testing and semi-quantitative echocardiographic evaluation.

TCPC was performed primarily due to tricuspid atresia (23.3%), single ventricle/double-inlet left ventricle (18.1%), and double-outlet right ventricle (17.7%). Patients had a median age of 4.7 (IQR, 3.5-6.4) years at the time of the procedure. Patients had a mean age of 19.0±6.9 at the time of exercise testing.

Among the patient group, peak oxygen uptake was highest among boys in early childhood (aged 6-10 years), declining linearly with age. Women and girls had a steeper decline in exercise capacity compared with men and boys. Compared with reference values for the general population, the patients who underwent TCPC did not exhibit a peak in exercise capacity during puberty.

A subset of patients with sufficient data (n=54) had a mean oxygen uptake efficiency slope/kg of 32.8±8.3 mL/min/kg, minute ventilation/carbon dioxide production slope of 32.9±4.0 and oxygen uptake to work rate slope of 9.4±1.7 mL/min/W. Peak oxygen uptake was correlated with oxygen uptake efficiency slope/kg (r, 0.77; P <.001), oxygen uptake to work rate slope (r, 0.35; P =.04), and minute ventilation/carbon dioxide production (r, -0.48; P =.003).

Compared with healthy values, the increase in oxygen pulse from low to maximal effort was decreased among women (mean, 1.8 vs 2.3 mL; P =.03) and men (mean, 2.9 vs 4.4 mL; P =.0007) in the TCPC cohort.

Among individuals with longitudinal data (n=206), the mean decline in peak oxygen uptake was 36.6 mL/min/kg – 0.41 ´ age, which did not differ significantly from the decline observed among the general population (P =.47). However, the TCPC cohort had a lower exercise capacity, given their age (P <.00001).

Sex was a significant predictor of decline in exercise capacity over time (P =.016). Peak oxygen uptake was 3.81 mL/min/kg – 0.65 ´ age among women and 36.2 mL/min/kg – 0.26 ´ age among men (P =.008).

There was little evidence of an effect of ventricular morphology (P =.23) or preoperative pulmonary dimensions (Nakata index: P =.64; McGoon ratio: P =.50). There was a significant negative correlation between peak oxygen uptake and body mass index (BMI) (P =006).

This study may have been limited by not taking atrioventricular valve regurgitation into consideration.

“[TCPC] patients have significantly reduced exercise capacity with a linear decline from adolescence to third decade of life,” the study authors said. “The rate of decline is faster in women and is not significantly affected by ventricular morphology or pre-TCPC pulmonary artery size.”


Illinger V, Materna O, Slabý K, et al. Exercise capacity after total cavopulmonary anastomosis: a longitudinal paediatric and adult study. ESC Heart Fail. Published online December 10, 2021. doi:10.1002/ehf2.13747

This article originally appeared on The Cardiology Advisor