Right Ventricular Diastolic Function and Right Atrial Function and Their Relation With Exercise Capacity in Ebstein Anomaly

BackgroundRight ventricular (RV) diastolic function and right atrial (RA) function are poorly characterized in patients with Ebstein anomaly (EA) but may influence functional capacity. We aimed to evaluate RV diastolic function and RA function in EA and study their relationship with biventricular systolic function and exercise capacity.MethodsSeventy-two patients with EA and 69 controls prospectively underwent echocardiography, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing to investigate RV systolic and diastolic function, RA function, and exercise capacity.ResultsAltered RV diastolic function was indicated by the reduced tricuspid valve E/A ratio, percentage RV filling time, and early and late diastolic strain rate; and by the increased tricuspid valve E/E′, isovolumic relaxation time, and RV myocardial performance index. The average of 6-RV-segment early diastolic strain rate correlated modestly with peak VO₂ (r = 0.38, P < 0.01), RV ejection fraction (r = 0.41, P < 0.01), and left ventricular ejection fraction (r = 0.33, P < 0.05). Patients with EA had impaired RA reservoir, conduit, and pump function, which were associated with peak VO₂ (r = 0.54, P < 0.001 for reservoir function).ConclusionsAltered RV diastolic function and RA function in patients with EA are associated with impaired biventricular systolic function and exercise capacity. The stronger correlation of RA vs RV function with exercise capacity suggests that it may be important to evaluate RA function in this population.