PMID- 24632273 OWN - NLM STAT- MEDLINE DCOM- 20140624 LR - 20161125 IS - 1558-3597 (Electronic) IS - 0735-1097 (Linking) VI - 63 IP - 17 DP - 2014 May 6 TI - Myocardial extracellular remodeling is associated with ventricular diastolic dysfunction in children and young adults with congenital aortic stenosis. PG - 1778-85 LID - S0735-1097(14)01304-7 [pii] LID - 10.1016/j.jacc.2013.11.066 [doi] AB - OBJECTIVES: This study sought to analyze cardiac magnetic resonance (CMR) measurements of myocardial extracellular volume fraction (ECV) and late gadolinium enhancement (LGE) in children and young adults with congenital aortic stenosis (AS) to determine the extent of fibrosis and examine their association with aortic valve and ventricular function. BACKGROUND: Patients with congenital AS frequently have impaired diastolic ventricular function and exercise capacity that may be related to myocardial fibrosis. METHODS: A total of 35 patients with congenital AS (median age 16 years) and 27 normal control subjects (median age 16 years) were evaluated by CMR. ECV was calculated from pre- and post-gadolinium contrast T1 measurements of blood and myocardium, and the hematocrit. RESULTS: ECV was significantly higher in AS patients than in normal subjects (median 0.27 [range 0.22 to 0.42] vs. 0.25 [range 0.18 to 0.27], p = 0.001). LGE was present in 8 (24%) of the AS patients. A higher ECV was correlated with echocardiographic indexes of diastolic dysfunction including a higher mitral E-wave z-score (r = 0.58, p = 0.002), E/septal E' z-score (r = 0.56, p = 0.003), E/mean E' z-score (r = 0.55, p = 0.003), and indexed left atrial volume (r = 0.56, p = 0.001). Other factors associated with an elevated ECV (>0.28) included a greater number of aortic valve interventions (p = 0.004) and a greater number of aortic valve balloon valvuloplasties (p = 0.003). ECV was not significantly associated with AS gradient, left ventricular mass, mass/volume ratio, or ejection fraction. CONCLUSIONS: In young patients with AS, myocardial ECV is significantly elevated compared with control subjects and is associated with echocardiographic indexes of diastolic dysfunction. ECV measured by CMR may be a useful method for risk stratification and monitoring therapies targeting fibrosis. CI - Copyright (c) 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. FAU - Dusenbery, Susan M AU - Dusenbery SM AD - Department of Cardiology, Boston Children's Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts. FAU - Jerosch-Herold, Michael AU - Jerosch-Herold M AD - Department of Radiology, Brigham and Women's Hospital, and the Department of Radiology, Harvard Medical School, Boston, Massachusetts. FAU - Rickers, Carsten AU - Rickers C AD - Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany. FAU - Colan, Steven D AU - Colan SD AD - Department of Cardiology, Boston Children's Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts. FAU - Geva, Tal AU - Geva T AD - Department of Cardiology, Boston Children's Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts. FAU - Newburger, Jane W AU - Newburger JW AD - Department of Cardiology, Boston Children's Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts. FAU - Powell, Andrew J AU - Powell AJ AD - Department of Cardiology, Boston Children's Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts. Electronic address: andrew.powell@cardio.chboston.org. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140313 PL - United States TA - J Am Coll Cardiol JT - Journal of the American College of Cardiology JID - 8301365 SB - IM CIN - J Am Coll Cardiol. 2014 May 6;63(17):1786-7. PMID: 24632277 MH - Adolescent MH - Adult MH - Aortic Valve Stenosis/complications/*congenital/physiopathology MH - Child MH - Child, Preschool MH - Diastole MH - Disease Progression MH - Echocardiography MH - Exercise Test MH - Female MH - Fibrosis/complications/diagnosis MH - Follow-Up Studies MH - Heart Ventricles/diagnostic imaging/pathology/*physiopathology MH - Humans MH - Infant MH - Magnetic Resonance Imaging, Cine MH - Male MH - Myocardial Contraction/*physiology MH - Myocardium/*pathology MH - Prognosis MH - Retrospective Studies MH - Ventricular Dysfunction/diagnosis/etiology/*physiopathology MH - *Ventricular Remodeling MH - Young Adult OTO - NOTNLM OT - congenital aortic stenosis OT - magnetic resonance imaging OT - myocardial fibrosis EDAT- 2014/03/19 06:00 MHDA- 2014/06/25 06:00 CRDT- 2014/03/18 06:00 PHST- 2013/11/04 00:00 [received] PHST- 2013/11/05 00:00 [accepted] PHST- 2014/03/18 06:00 [entrez] PHST- 2014/03/19 06:00 [pubmed] PHST- 2014/06/25 06:00 [medline] AID - S0735-1097(14)01304-7 [pii] AID - 10.1016/j.jacc.2013.11.066 [doi] PST - ppublish SO - J Am Coll Cardiol. 2014 May 6;63(17):1778-85. doi: 10.1016/j.jacc.2013.11.066. Epub 2014 Mar 13.