title: Clinical significance of late gadolinium enhancement in patients<20 years of age with hypertrophic cardiomyopathy.
abstract: Late gadolinium enhancement (LGE) on cardiovascular magnetic resonance imaging is associated with adverse events in adults with hypertrophic cardiomyopathy (HC). However, limited data exist on the extent and clinical significance of LGE in the pediatric population. In 30 patients (aged 14.1±3.2 years) with clinically diagnosed HC who underwent cardiovascular magnetic resonance imaging from 2007 to 2012, segments with hypertrophy and LGE were identified by 2 experienced readers blinded to outcome. Radial, circumferential, and longitudinal strains were evaluated using feature tracking software. The composite outcome was defined as cardiac death, nonsustained ventricular tachycardia, ventricular fibrillation, or appropriate implantable cardioverter-defibrillator discharge. LGE was present in 17 of 30 patients (57%), all in a midmyocardial pattern, with median 3 segments per patient (interquartile range [IQR] 2 to 5). No LGE was detected in patients without phenotypic hypertrophy. Segments with LGE had decreased radial (basal segments 20.7% vs 70.9%, p=0.01), circumferential (basal segments -23.2% vs -29.3%, p=0.04), and longitudinal strains (basal segments -13.8% vs -20.9%, p=0.04). After median follow-up of 26.9 months (IQR 7.5 to 34.3), 7 patients who had an adverse outcome (5 ventricular tachycardia, 1 appropriate implantable cardioverter-defibrillator discharge, and 1 death) had more segments of LGE (median 4, IQR 2 to 7 vs 0, IQR 0 to 2, p=0.01). One patient without LGE had ventricular tachycardia on exercise test. In conclusion, LGE occurs in a similar pattern in pediatric patients with HC as in adults, associated with hypertrophy, decreased myocardial strain, and adverse clinical outcomes. Further longitudinal studies are necessary to evaluate the rate of development of LGE and relation to outcomes in a larger cohort.
authors: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan. Electronic address: bsmith@neomed.edu.
authors: Brandon M
authors: BM
authors: Smith
authors: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan; Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors: Adam L
authors: AL
authors: Dorfman
authors: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan.
authors: Sunkyung
authors: S
authors: Yu
authors: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan.
authors: Mark W
authors: MW
authors: Russell
authors: Division of Cardiothoracic Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors: Prachi P
authors: PP
authors: Agarwal
authors: Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors: Maryam Ghadimi
authors: MG
authors: Mahani
authors: Division of Pediatric Cardiology, Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, Michigan; Section of Pediatric Radiology, Department of Radiology, University of Michigan, Ann Arbor, Michigan.
authors: Jimmy C
authors: JC
authors: Lu
mesh_terms: D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis* / Q000503:physiopathology; D002648:Child; D003287:Contrast Media; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D019786:Gadolinium DTPA; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D009206:Myocardium / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013318:Stroke Volume; D013997:Time Factors; D016277:Ventricular Function, Left; D055815:Young Adult
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA