Lung function and aerobic capacity in adult patients following modified Fontan procedure

Abstract

Objective-To examine cardiopulmonary performance in 52 adult patients with a Fontan circulation. Design-Retrospective cohort study. Values of maximum oxygen uptake (VO 2 max), maximum heart rate (HRmax), forced vital capacity (FVC), and forced expiratory volume in one second (FEV 1 ) were compared with predictive values for diVerent age groups. Patients were further subdivided into those with a pulmonary artery connection (RA-PA) or right atrium to right ventricle conduit (RA-RV). Results-At late follow up (median 10 years, range 1 to 26 years), patients with Fontan circulation had greatly diminished VO 2 max, HRmax, FVC, and FEV 1 compared with predicted values. Early age at surgery had a positive impact on aerobic capacity. The FEV 1 :FVC ratio indicated restrictive lung function. No diVerences were found with respect to any variable between patients with RA-PA connections and those with RA-RV connections. Conclusions-Patients with a Fontan circulation have greatly diminished values of aerobic capacity and a restrictive pattern of lung function. Patients with an early surgical procedure obtained higher values of VO 2 max. The theoretical benefits of including the right ventricle in a Fontan circulation were not apparent. (Heart 2001;85:295-299)

Congenital Heart Disease in the Older Adult

“…169,170 The estimated prevalence of RLD in adults with CHD has been reported to be as high as 44% to 56%, with an even higher prevalence in the setting of certain lesions, particularly patients with a history of Fontan palliation (prevalence of 89%) and TOF (prevalence of 76%). 170,171 These studies suggest that the prevalence of spirometry-diagnosed RLD in the ACHD population is markedly higher than in the general adult population, where it is reported to be ≈9.2%.…”

Exercise Intolerance in Adult Congenital Heart Disease

“…Not surprisingly, therefore, factors that impair chronotropic response to exercise such as ␤-blockade or abnormal cardiac rhythm were associated with a lower peak V O 2 . Previous studies have shown that some ACHD patients have impaired pulmonary function 15 and depressed peak heart rate. 5 The present study extends these findings by demonstrating that both heart rate response and pulmonary function correlate positively with peak V O 2 even after accounting for other physiological parameters.…”

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease

“…Serial evaluations facilitate detection of functional decline, although patients may report that they are "normal." One study 52 showed that peak oxygen uptake (V o 2 max) in a group of 41 patients with CCTGA ranged from 11 to 22 mL per kg per min, which is the equivalent of only 30% to 50% of normal control values.…”