Wendy Wai Man Lam1, Xue Cun Liang2, Eddie Cheung2, Alice KP Wu, Sophia J. Wong2, Yiu fai Cheung2
1Department of Radiology, Queen Mary Hospital, Hong Kong SAR, China; 2Division of Paediatric Cardiology, Queen Mary Hospital
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.