Abstract 18017: The Modified History in Young Patients With Repaired Tetralogy of Fallot: A Longitudinal Magnetic Resonance Study
Introduction: No large scale longitudinal information is available on the change of ventricular size and function as well as of exercise tolerance in survivors after Tetralogy of Fallot (TOF) repair during childhood and adolescence.
Objective: The objective of this study was to assess the rate of progression of right ventricular (RV) size and pulmonary regurgitation (PR) in relationship to clinical status and outcomes in pediatric TOF patients from a contemporary surgical era.
Methods: We analyzed 429 cardiac magnetic resonance (CMR) studies (from 2001-2014) of 267 patients (age at CMR 13.5 +/-2.8 years) after TOF repair prior to any pulmonary valve replacement (PVR), including 96 patients with serial CMRs. Measurements of biventricular volumes, mass, function, and PR were performed by a single observer. Serial ECGs, Holters, echocardiograms, and exercise tests were reviewed.
Results: No deaths occurred in our cohort. Three patients (1.1%) had non-sustained ventricular tachycardia and 4 (1.5%) had supraventricular tachycardia. Sixty seven (25%) patients underwent PVR at a mean age15.5 +/- 2.3 years.There was a linear progression of BSA-indexed RV end-diastolic volume (EDVi) at 3 ml/m2 per year (p.a., p<0.001). PR volume changed only minimally over time while PR fraction progressed at a rate of 0.7% p.a. (p<0.001). RV and LV ejection fractions (EFs) deteriorated over time (0.5% p.a., p<0.001 and 0.4% p.a., p=0.001, respectively). RV and LV EDVi increased (p=0.02 and p=0.001, respectively) and RV EF (p<0.001) decreased more rapidly in boys than in girls. Right atrial (RA) length correlated inversely with VO2max (% predicted, p=0.02). In a multivariable analysis, worsening PR fraction, RV EF, RA length, and LV EDVi were all predictors of PVR in the future.
Conclusions: This study describes the rate and dynamic of progression of biventricular size and function in children after TOF repair in the hitherto largest cohort, with apparent differences between boys and girls. RA length was inversely correlated with exercise intolerance.The need for PVR is predicted by the degree of RV dysfunction as well as LV and RA enlargement. The latter may deserve routine assessment in patients after TOF repair.
Author Disclosures: T. Gazzaz: None. B. Elders: None. C. Manlhiot: None. S. Yoo: None. M. Seed: None. B. Mccrindle: None. L. Grosse-Wortmann: None.
- © 2015 by American Heart Association, Inc.