Exercise capacity after total cavopulmonary anastomosis: a longitudinal paediatric and adult study

Abstract

Aims Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long‐term survival is negatively affected by the absence of sub‐pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O2peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease‐specific reference V̇O2peak dataset. Methods and results A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O2peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter‐quartile range) of 3.0 (2.0–5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O2peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O2peak decline correlated negatively with the trend of body mass index z‐score (P = 0.006) and was faster in women than men (P = 0.008). Conclusions Total cavopulmonary connection patients have significantly reduced exercise capacity. The age‐related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O2peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

Exercise capacity in a cohort of children with congenital heart disease

“…In adults with a univentricular circulation, a decrease in VO 2peak as percentage of predicted of > 3% per year has been shown to be a predictor for death or cardiac surgery [ 30 ]. In the present study, we describe a similar decrease in VO 2 peak/kg in patients with a univentricular circulation, and recently, Janousek et al described a similar decrease in a group of patients ranging from 10 to 30 years of age [ 31 ]. Follow-up studies in this cohort are necessary to determine the effects of attrition on outcome.…”

“…In the present study, we describe a similar decrease in VO 2 peak/kg in patients with a univentricular circulation, and recently, Janousek et al described a similar decrease in a group of patients ranging from 10 to 30 years of age [31]. Follow-up studies in this cohort are necessary to determine the effects of attrition on outcome.…”

Association of Body Mass Index With Clinical Features and Outcomes in Adults With Fontan Palliation

“… 20 , 21 This explains our and prior investigators’ findings of the association of lower VO 2 with increasing BMI among our study patients. 22 However, the lack of this association after adjusting for the covariates underscores the impact of other factors (such as age, sex, NYHA class, or type of Fontan) on VO 2 in these patients. Nevertheless, it is well established that a low level of VO 2 is an independent risk factor for cardiovascular mortality 23 , 24 and, hence, measures to improve this may improve clinical outcomes in patients.…”

Impact of liver fibrosis and nodules formation on hemodynamics in young adults after total cavopulmonary connection. A magnetic resonance study

“…Breath by breath measurement of gas exchange and ventilation was monitored by a calibrated gas exchange analyzer (Oxycon Pro with the electrochemical oxygen sensor, Jaeger, Germany). Each test result was checked for consistency in the physiological response of the data by an exercise physiologist ( 8 ).…”