Clinical Significance of Late Gadolinium Enhancement in Patients <20 Years of Age With Hypertrophic Cardiomyopathy

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 ye...

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Bibliographic Details
Published in:The American journal of cardiology 2014-04, Vol.113 (7), p.1234-1239
Main Authors: Smith, Brandon M., MD, Dorfman, Adam L., MD, Yu, Sunkyung, MS, Russell, Mark W., MD, Agarwal, Prachi P., MBBS, Mahani, Maryam Ghadimi, MD, Lu, Jimmy C., MD
Format: Article
Language:English
Subjects:
Adolescent
Cardiology
Cardiomyopathy
Cardiomyopathy, Hypertrophic - diagnosis
Cardiomyopathy, Hypertrophic - physiopathology
Cardiovascular
Cardiovascular disease
Child
Clinical outcomes
Confidence intervals
Contrast Media
Disease Progression
Female
Follow-Up Studies
Gadolinium DTPA
Heart
Heart attacks
Humans
Magnetic Resonance Imaging, Cine - methods
Male
Mutation
Myocardium - pathology
Prognosis
Retrospective Studies
Severity of Illness Index
Stroke Volume
Time Factors
Ventricular Function, Left
Young Adult
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Summary: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.
ISSN:0002-9149
1879-1913
DOI:10.1016/j.amjcard.2013.12.034