After Fontan operation, patients are limited in increasing cardiac output and in exercise capacity. This has been related to impaired preload or other factors leading to decreased global ventricular performance with stress. To study these factors, the stress responses of functionally univentricular hearts were assessed at rest and during low-dose dobutamine stress using cardiovascular magnetic resonance imaging. Thirty-two patients after Fontan completion at young age were included (27 with total cavopulmonary connection, 5 with atriopulmonary connection; mean age 13.3 years, range 7.5 to 22.2; 23 male patients; median follow-up after Fontan operation 8.1 years, range 5.2 to 17.8). A multiphase short-axis stack of 10 to 12 contiguous slices of the systemic ventricle was obtained at rest and during low-dose dobutamine stress cardiovascular magnetic resonance imaging (maximum 7.5 μg/kg/min). With stress-testing, heart rate, ejection fraction, and cardiac index increased adequately (p <0.001). There was an abnormal decrease in end-diastolic volume and an adequate decrease in end-systolic volume (p <0.001). Stroke volume did not change with stress testing (p = 0.15). At rest, dominant left ventricles had higher ejection fractions than dominant right ventricles (p = 0.01), but this difference disappeared with stress testing. In conclusion, a functionally univentricular heart after Fontan completion at young age has an adequate increase in ejection fraction with β-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate.

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Journal The American Journal of Cardiology
Robbers-Visser, D, Jan ten Harkel, D, Kapusta, L, Strengers, J.L.M, Dalinghaus, M, Meijboom, F.J, … Helbing, W.A. (2008). Usefulness of Cardiac Magnetic Resonance Imaging Combined With Low-Dose Dobutamine Stress to Detect an Abnormal Ventricular Stress Response in Children and Young Adults After Fontan Operation at Young Age. The American Journal of Cardiology, 101(11), 1657–1662. doi:10.1016/j.amjcard.2008.01.050