Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review

Abstract

ObjectiveExercise impairment is common in Fontan patients. Our aim is to systematically review previous literature to determine the prognostic value of exercise capacity in older adolescent and adult Fontan patients with respect to late outcome. Additionally, we reviewed the determinants of exercise capacity in Fontan patients and changes in exercise capacity over time.MethodsPubMed, CINAHL, Embase, The Cochrane Library and Scopus were searched systematically for studies reporting exercise capacity and late outcome such as mortality, cardiac transplantation and hospitalisation. Studies were eligible for inclusion if more than 30 patients were included and mean age was ≥16 years.ResultsFour thousand and seven hundred and twenty-two studies were identified by the systematic search. Seven studies fulfilled the inclusion and exclusion criteria. The total number of patients was 1664 adult Fontan patients. There were 149 deaths and 35 heart transplantations. All eligible studies were retrospective cohort studies. The correlation between exercise capacity and late outcome was identified, and HRs were reported.ConclusionIn Fontan patients, the best predictors of death and transplantation were a decline in peak VO2, heart rate variables and exercise oscillatory ventilation. Peak VO2 was not strongly predictive of mortality or hospitalisation in Fontan patients. Several variables were strong and independent predictors of hospitalisation and morbidity.

Chronotropic incompetence causes multiple organ complications in adults after the Fontan procedure

“…Of the patients in the FO group, 98% had an increased VE/VCO2. These results concur with the previous studies and show that exercise limitation is common in this special group of patients [2,15,16]. The reasons for the reduced exercise performance in patients after FO seem to be associated with a combination of different mechanisms.…”

“…CPET is a widely acknowledged tool as it provides information about not only exercise capacity but also prognosis, including the need for heart transplantation [15]. The results of the present study corroborate with a well-known observation that patients with Fontan physiology have reduced exercise capacity.…”

The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity

“…The physiological mechanisms of this limitation includes inadequate preload augmentation to the single ventricle (14), inadequate heart rate reserve (15), and poor conditioning with relative skeletal muscle weakness (16). Poor exercise performance is associated with acute cardiovascular decompensation, need for heart transplantation, and early mortality (17). There is evidence however that a few Fontan patients achieve normal exercise performance (6).…”

Maximal versus sub-maximal effort during cardiopulmonary exercise testing in adults with congenital heart disease: outcome analysis of short-term cardiac-related events

“…A number of studies have found that the peak oxygen consumption is one of the best predictors of morbidity or mortality in a variety of cardiovascular conditions including congestive heart failure, 19,20 pulmonary hypertension, 21 tetralogy of Fallot, 22 transposition of the great arteries, 23 and Fontan circulations. 3 The study by Inuzuka et al 1 found that peak oxygen consumption and heart rate reserve data can be used to generate estimates for 5-year survival in many adults with CHD. However, in their study a majority of patients had low peak respiratory exchange ratio values <1.1, suggesting that a large fraction of the patients did not expend adequate effort and terminated exercise before reaching their cardiovascular limit.…”