title: Left Ventricular Remodeling and Function in Children with Biventricular Circulation After Fetal Aortic Valvuloplasty.
abstract: Fetal aortic valvuloplasty (FAV) has shown promise in averting the progression of fetal aortic stenosis to hypoplastic left-heart syndrome. Altered loading conditions due to valvar disease, intrinsic endomyocardial abnormalities, and procedures that alter endomyocardial mechanics may place patients with biventricular circulation (BiV) after FAV at risk of abnormal LV remodeling and function. Using the most recent echo data on BiV patients after technically successful FAV (n = 34), we evaluated LV remodeling pattern, risk factors for pathologic LV remodeling, and the association between LV remodeling pattern and LV function. Median age at follow-up was 4.7 years (range 1.0-12.5). Cardiac interventions were common. At latest follow-up, no patient had hypoplastic LV. Nineteen patients (55 %) had dilated LV, and five (16 %) patients had severely dilated LV. LV remodeling patterns were as follows: 12 (35 %) normal ventricle, 11 (32 %) mixed hypertrophy, 8 (24 %) eccentric hypertrophy or remodeling, and 3 (9 %) concentric hypertrophy. Univariate factors associated with pathologic LV remodeling were long-standing AR, ≥2 cardiac interventions, EFE resection, and aortic or mitral regurgitation ≥ moderate at most recent follow-up. In multivariate analysis, only long-standing AR fraction remained associated with pathologic remodeling. Pathologic LV remodeling was associated with depressed ejection fraction, lower septal E´, and higher E/E´. Pathologic LV remodeling, primarily eccentric or mixed hypertrophy, is common in BiV patients after FAV and is related to LV loading conditions imposed by valvar disease. Pathologic remodeling is associated with both systolic and diastolic dysfunction in this population.
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA. Kevin.Friedman@cardio.chboston.org.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA. Kevin.Friedman@cardio.chboston.org.
authors.forename: Kevin G
authors.initials: KG
authors.lastname: Friedman
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
authors.forename: Lindsay
authors.initials: L
authors.lastname: Freud
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
authors.forename: Maria
authors.initials: M
authors.lastname: Escobar-Diaz
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
authors.forename: Puja
authors.initials: P
authors.lastname: Banka
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
authors.forename: Sitaram
authors.initials: S
authors.lastname: Emani
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
authors.forename: Wayne
authors.initials: W
authors.lastname: Tworetzky
mesh_terms: D001021:Aortic Valve / Q000000981:diagnostic imaging; D001024:Aortic Valve Stenosis / Q000000981:diagnostic imaging*; D006348:Cardiac Surgical Procedures / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003971:Diastole / Q000502:physiology; D015150:Echocardiography, Doppler; D005260:Female; D006352:Heart Ventricles / Q000503:physiopathology; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery*; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011183:Postoperative Complications / Q000000981:diagnostic imaging*; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging*; D020257:Ventricular Remodeling
keywords: Aortic stenosis; Diastolic dysfunction; Fetal cardiology; Left ventricular remodeling