title: Predictors of Change in Functional Health Status in Adults with Repaired Tetralogy of Fallot.
abstract: Left ventricular (LV) ejection fraction (EF) and right ventricular (RV) ejection fraction by cardiovascular magnetic resonance (CMR) are associated with functional health status in patients with repaired tetralogy of Fallot (TOF) in cross-sectional studies, but few longitudinal data are available. This study aimed to determine predictors of subsequent decrease in functional health status in midterm follow-up. Patients with repaired TOF who had previously completed CMR and assessment with the Short Form 36 version 2 (SF-36) were recruited for repeat CMR, SF-36, and exercise test, if they had not had interval pulmonary valve replacement (PVR). Patients from the same cohort who had undergone PVR were recruited for repeat SF-36. A total of 19 patients (median 33.5 years old, interquartile range [IQR] 26-42 years, 53 % male) had not undergone PVR and were enrolled at a median of 5.0 years (IQR 4.8-5.3) since prior CMR and SF-36. LVEF and RVEF did not change from baseline, while RV end-diastolic volume increased (138 ± 34 vs. 126 ± 31 ml/m(2), p = 0.02). In the overall cohort, SF-36 scores remained stable. However, higher baseline RV end-systolic volume and pulmonary regurgitant fraction correlated with subsequent decreases in SF-36 scores. In 9 patients post-PVR (median 35.9 years old, IQR 24-43), physical functioning increased compared to those without PVR (change in z-score +0.59 ± 0.59 vs. -0.26 ± 0.72, p = 0.005). In adults with repaired TOF who do not undergo PVR, LVEF, RVEF, and functional health status remain stable in midterm follow-up. However, baseline RV end-systolic volume correlates with subsequent change in functional health status, underscoring its importance in prognostication and timing of intervention in this population.
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, USA. jimmyL@umich.edu.|Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, MI, USA. jimmyL@umich.edu.
authors.forename: Jimmy C
authors.identifier: http://orcid.org/0000-0002-2544-7402
authors.initials: JC
authors.lastname: Lu
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Sunkyung
authors.initials: S
authors.lastname: Yu
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Ray
authors.initials: R
authors.lastname: Lowery
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Janaki
authors.initials: J
authors.lastname: Sagi
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Amanda C
authors.initials: AC
authors.lastname: Delong
authors.affiliation: Division of Cardiothoracic Radiology, Department of Radiology, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Prachi P
authors.initials: PP
authors.lastname: Agarwal
authors.affiliation: Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Maryam Ghadimi
authors.initials: MG
authors.lastname: Mahani
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, MI, USA.|Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Adam L
authors.initials: AL
authors.lastname: Dorfman
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006304:Health Status; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011665:Pulmonary Valve Insufficiency; D013771:Tetralogy of Fallot; D016278:Ventricular Function, Right
keywords: Cardiac magnetic resonance; Congenital heart disease; Functional health status; Tetralogy of Fallot