Abstract
AIMS: Inefficient ventricular-arterial (V-A) coupling has been described in Fontan patients and may result in adverse haemodynamics. A varying amount of aortopulmonary collateral (APC) flow is also frequently present that increases volume load of the single ventricle. The aim of the study was to assess changes in V-A coupling and APC flow during exercise CMR. METHODS AND RESULTS: Eighteen Fontan patients (age 24 ± 3 years) and 14 controls (age 23 ± 4 years) underwent exercise CMR using a cycle ergometer. Ventricular volumetry and flow measurements in the ascending aorta (AAO), inferior (IVC), and superior (SVC) vena cava were assessed using real-time sequences during stepwise increases in work load. Measures of systemic arterial elastance Ea, ventricular elastance Ees, and V-A coupling (Ea/Ees) were assessed. APC flow was quantified as AAO - (SVC + IVC). Ea remained unchanged during all levels of exercise in both groups (P = 0.39 and P = 0.11). Ees increased in both groups (P = 0.001 and P < 0.001) with exercise but was lower in the Fontan group (P = 0.04). V-A coupling was impaired in Fontan patients at baseline (P = 0.04). Despite improvement during exercise (P = 0.002) V-A coupling remained impaired compared with controls (P = 0.001). Absolute APC flow in Fontan patients did not change during exercise even at maximum work load (P = 0.98). CONCLUSIONS: Inefficient V-A coupling was already present at rest in Fontan patients and aggravated during exercise due to a limited increase in ventricular contractility which demonstrates the importance of a limited functional reserve of the single ventricle. APC flow remained unchanged suggesting no further increase in volume load during exercise.