Restrictive Right Ventricular Physiology, Right Ventricular Fibrosis as Suggested by Cardiac Magnetic Resonance, and Exercise Capacity After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum in Children

Wendy Wai Man Lam¹, Xue Cun Liang², Eddie Cheung², Alice KP Wu, Sophia J. Wong², Yiu fai Cheung²

We tested the hypothesis that RV restrictive physiology is prevalent and related to RV fibrosis and exercise capacity in patients after repair of pulmonary atresia and intact ventricular septum (PAIVS). Restrictive RV physiology was defined by presence of antegrade diastolic pulmonary flow and RV fibrosis assessed by late gadolinium enhancement (LGE) MR. They had higher RV LGE score. The score correlated negatively with exercise duration and with greater % predicted maximum oxygen consumption. In conclusion: restrictive RV physiology is prevalent in post-operative PAIVS patients. RV diastolic dysfunction is related to the magnitude of RV fibrosis and with better exercise capacity.