Prevalence and progression of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy

Anna Axelsson Raja, Hoshang Farhad, Anne Marie Valente, John Paul Couce, John Jefferies, Henning Bundgaard, Kenneth Zahka, Harry Lever, Anne M. Murphy, Euan Ashley, Sharlene M. Day, Mark V. Sherrid, Ling Shi, David A. Bluemke, Charles E. Canter, Steven D. Colan, Carolyn Y. Ho

Research output: Contribution to journalArticle

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Abstract

BACKGROUND: Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ≈60% of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. In addition, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM. METHODS: CMR scans from 195 patients ≤21 years of age were analyzed in an observational, retrospective study, including 155 patients with overt HCM and 40 sarcomere mutation carriers without left ventricular (LV) hypertrophy. The extent of LGE was quantified by measuring regions with signal intensity >6 SD above nulled remote myocardium. RESULTS: Patients were 14.3±4.5 years of age at baseline and 68% were male. LGE was present in 70 (46%) patients with overt HCM (median extent, 3.3%; interquartile range, 0.8-7.1%), but absent in mutation carriers without LV hypertrophy. Thirty-one patients had >1 CMR (median interval between studies, 2.4 years; interquartile range, 1.5-3.2 years). LGE was detected in 13 patients (42%) at baseline and in 16 patients (52%) at follow-up CMR. The median extent of LGE increased by 2.4 g/y (range, 0-13.2 g/y) from 2.9% (interquartile range, 0.8-3.2%) of LV mass to 4.3% (interquartile range, 2.9-6.8%) (P=0.02). In addition to LGE, LV mass and left atrial volume, indexed to body surface area, and z score for LV mass, as well, increased significantly from first to most recent CMR. CONCLUSIONS: LGE was present in 46% of children and adolescents with overt HCM, in contrast to ≈60% typically reported in adult HCM. In the subset of patients with serial imaging, statistically significant increases in LGE, LV mass, and left atrial size were detected over 2.5 years, indicating disease progression over time. Further prospective studies are required to confirm these findings and to better understand the clinical implications of LGE in pediatric HCM.

Original languageEnglish (US)
Pages (from-to)782-792
Number of pages11
JournalCirculation
Volume138
Issue number8
DOIs
StatePublished - Jan 1 2018

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Hypertrophic Cardiomyopathy
Gadolinium
Magnetic Resonance Imaging
Left Ventricular Hypertrophy
Pediatrics
Sarcomeres
Mutation
Body Surface Area
Observational Studies
Longitudinal Studies
Disease Progression
Myocardium
Fibrosis
Retrospective Studies
Cross-Sectional Studies
Prospective Studies

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Prevalence and progression of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy. / Raja, Anna Axelsson; Farhad, Hoshang; Valente, Anne Marie; Couce, John Paul; Jefferies, John; Bundgaard, Henning; Zahka, Kenneth; Lever, Harry; Murphy, Anne M.; Ashley, Euan; Day, Sharlene M.; Sherrid, Mark V.; Shi, Ling; Bluemke, David A.; Canter, Charles E.; Colan, Steven D.; Ho, Carolyn Y.

In: Circulation, Vol. 138, No. 8, 01.01.2018, p. 782-792.

Research output: Contribution to journalArticle

Raja, AA, Farhad, H, Valente, AM, Couce, JP, Jefferies, J, Bundgaard, H, Zahka, K, Lever, H, Murphy, AM, Ashley, E, Day, SM, Sherrid, MV, Shi, L, Bluemke, DA, Canter, CE, Colan, SD & Ho, CY 2018, 'Prevalence and progression of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy', Circulation, vol. 138, no. 8, pp. 782-792. https://doi.org/10.1161/CIRCULATIONAHA.117.032966
Raja, Anna Axelsson ; Farhad, Hoshang ; Valente, Anne Marie ; Couce, John Paul ; Jefferies, John ; Bundgaard, Henning ; Zahka, Kenneth ; Lever, Harry ; Murphy, Anne M. ; Ashley, Euan ; Day, Sharlene M. ; Sherrid, Mark V. ; Shi, Ling ; Bluemke, David A. ; Canter, Charles E. ; Colan, Steven D. ; Ho, Carolyn Y. / Prevalence and progression of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy. In: Circulation. 2018 ; Vol. 138, No. 8. pp. 782-792.
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title = "Prevalence and progression of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy",
abstract = "BACKGROUND: Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ≈60{\%} of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. In addition, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM. METHODS: CMR scans from 195 patients ≤21 years of age were analyzed in an observational, retrospective study, including 155 patients with overt HCM and 40 sarcomere mutation carriers without left ventricular (LV) hypertrophy. The extent of LGE was quantified by measuring regions with signal intensity >6 SD above nulled remote myocardium. RESULTS: Patients were 14.3±4.5 years of age at baseline and 68{\%} were male. LGE was present in 70 (46{\%}) patients with overt HCM (median extent, 3.3{\%}; interquartile range, 0.8-7.1{\%}), but absent in mutation carriers without LV hypertrophy. Thirty-one patients had >1 CMR (median interval between studies, 2.4 years; interquartile range, 1.5-3.2 years). LGE was detected in 13 patients (42{\%}) at baseline and in 16 patients (52{\%}) at follow-up CMR. The median extent of LGE increased by 2.4 g/y (range, 0-13.2 g/y) from 2.9{\%} (interquartile range, 0.8-3.2{\%}) of LV mass to 4.3{\%} (interquartile range, 2.9-6.8{\%}) (P=0.02). In addition to LGE, LV mass and left atrial volume, indexed to body surface area, and z score for LV mass, as well, increased significantly from first to most recent CMR. CONCLUSIONS: LGE was present in 46{\%} of children and adolescents with overt HCM, in contrast to ≈60{\%} typically reported in adult HCM. In the subset of patients with serial imaging, statistically significant increases in LGE, LV mass, and left atrial size were detected over 2.5 years, indicating disease progression over time. Further prospective studies are required to confirm these findings and to better understand the clinical implications of LGE in pediatric HCM.",
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TY - JOUR

T1 - Prevalence and progression of late gadolinium enhancement in children and adolescents with hypertrophic cardiomyopathy

AU - Raja, Anna Axelsson

AU - Farhad, Hoshang

AU - Valente, Anne Marie

AU - Couce, John Paul

AU - Jefferies, John

AU - Bundgaard, Henning

AU - Zahka, Kenneth

AU - Lever, Harry

AU - Murphy, Anne M.

AU - Ashley, Euan

AU - Day, Sharlene M.

AU - Sherrid, Mark V.

AU - Shi, Ling

AU - Bluemke, David A.

AU - Canter, Charles E.

AU - Colan, Steven D.

AU - Ho, Carolyn Y.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - BACKGROUND: Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ≈60% of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. In addition, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM. METHODS: CMR scans from 195 patients ≤21 years of age were analyzed in an observational, retrospective study, including 155 patients with overt HCM and 40 sarcomere mutation carriers without left ventricular (LV) hypertrophy. The extent of LGE was quantified by measuring regions with signal intensity >6 SD above nulled remote myocardium. RESULTS: Patients were 14.3±4.5 years of age at baseline and 68% were male. LGE was present in 70 (46%) patients with overt HCM (median extent, 3.3%; interquartile range, 0.8-7.1%), but absent in mutation carriers without LV hypertrophy. Thirty-one patients had >1 CMR (median interval between studies, 2.4 years; interquartile range, 1.5-3.2 years). LGE was detected in 13 patients (42%) at baseline and in 16 patients (52%) at follow-up CMR. The median extent of LGE increased by 2.4 g/y (range, 0-13.2 g/y) from 2.9% (interquartile range, 0.8-3.2%) of LV mass to 4.3% (interquartile range, 2.9-6.8%) (P=0.02). In addition to LGE, LV mass and left atrial volume, indexed to body surface area, and z score for LV mass, as well, increased significantly from first to most recent CMR. CONCLUSIONS: LGE was present in 46% of children and adolescents with overt HCM, in contrast to ≈60% typically reported in adult HCM. In the subset of patients with serial imaging, statistically significant increases in LGE, LV mass, and left atrial size were detected over 2.5 years, indicating disease progression over time. Further prospective studies are required to confirm these findings and to better understand the clinical implications of LGE in pediatric HCM.

AB - BACKGROUND: Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ≈60% of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. In addition, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM. METHODS: CMR scans from 195 patients ≤21 years of age were analyzed in an observational, retrospective study, including 155 patients with overt HCM and 40 sarcomere mutation carriers without left ventricular (LV) hypertrophy. The extent of LGE was quantified by measuring regions with signal intensity >6 SD above nulled remote myocardium. RESULTS: Patients were 14.3±4.5 years of age at baseline and 68% were male. LGE was present in 70 (46%) patients with overt HCM (median extent, 3.3%; interquartile range, 0.8-7.1%), but absent in mutation carriers without LV hypertrophy. Thirty-one patients had >1 CMR (median interval between studies, 2.4 years; interquartile range, 1.5-3.2 years). LGE was detected in 13 patients (42%) at baseline and in 16 patients (52%) at follow-up CMR. The median extent of LGE increased by 2.4 g/y (range, 0-13.2 g/y) from 2.9% (interquartile range, 0.8-3.2%) of LV mass to 4.3% (interquartile range, 2.9-6.8%) (P=0.02). In addition to LGE, LV mass and left atrial volume, indexed to body surface area, and z score for LV mass, as well, increased significantly from first to most recent CMR. CONCLUSIONS: LGE was present in 46% of children and adolescents with overt HCM, in contrast to ≈60% typically reported in adult HCM. In the subset of patients with serial imaging, statistically significant increases in LGE, LV mass, and left atrial size were detected over 2.5 years, indicating disease progression over time. Further prospective studies are required to confirm these findings and to better understand the clinical implications of LGE in pediatric HCM.

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U2 - 10.1161/CIRCULATIONAHA.117.032966

DO - 10.1161/CIRCULATIONAHA.117.032966

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JO - Circulation

JF - Circulation

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