PMID- 32088002
OWN - NLM
STAT- MEDLINE
DCOM- 20200803
LR  - 20200803
IS  - 1879-1913 (Electronic)
IS  - 0002-9149 (Linking)
VI  - 125
IP  - 8
DP  - 2020 Apr 15
TI  - Cardiac Magnetic Resonance Imaging Features in Hypertrophic Cardiomyopathy 
      Diagnosed at <21 Years of Age.
PG  - 1249-1255
LID - S0002-9149(20)30068-0 [pii]
LID - 10.1016/j.amjcard.2020.01.027 [doi]
AB  - Hypertrophic cardiomyopathy (HC) is the most common inherited cardiomyopathy, 
      with varied timing of phenotypic and clinical presentation. Literature describing 
      cardiac magnetic resonance (CMR) imaging and late gadolinium enhancement (LGE) in 
      young patients with HC is limited. This study included patients diagnosed with HC 
      at young age (<21 years) between January 1990 and January 2015 who underwent 
      transthoracic echocardiography and CMR with assessment of LGE at a single 
      tertiary referral center. LGE was quantified via a method of 6 standard 
      deviations and patients were grouped based upon presence or absence of LGE (</=1% 
      and >1% LGE, respectively). Sudden cardiac death (SCD) risk was assessed in 
      patients >16 years of age using the European SCD risk score. A composite outcome 
      of New York Heart Association class III-IV symptoms, aborted SCD, heart 
      transplantation, and all-cause mortality was assessed via Kaplan-Meier curves 
      with log-rank analysis. Overall, 126 patients were included (78 male; 62%). 
      Median age of diagnosis was 15 (12 to 18) years. LGE was present in 81 (64%) 
      patients, although only 4 (3%) patients had LGE >15%. Median age at CMR imaging 
      was 19 (15 to 23) years. Patients with LGE had greater wall thickness (25 +/- 8 mm 
      vs 22 +/- 7 mm, p = 0.01). Median European SCD risk score was 4.7 (2.9 to 6.5). 
      Median follow-up was 6.5 (2.5 to 13) years with 26 patients (21%) meeting the 
      composite outcome. There were no significant differences in composite outcome 
      since age of diagnosis when stratified by presence/absence of LGE (p = 1.0). The 
      presence of LGE in young HC patients was not an independent risk factor for 
      cardiovascular morbidity and mortality. Wall thickness was greater in patients 
      with LGE. There remains a need for further evaluation of this unique HC cohort.
CI  - Copyright (c) 2020 Elsevier Inc. All rights reserved.
FAU - Bonura, Erica D
AU  - Bonura ED
AD  - Division of Pediatric Cardiology, Department of Pediatric and Adolescent 
      Medicine, Mayo Clinic, Rochester, Minnesota.
FAU - Bos, J Martijn
AU  - Bos JM
AD  - Division of Pediatric Cardiology, Department of Pediatric and Adolescent 
      Medicine, Mayo Clinic, Rochester, Minnesota; Department of Cardiovascular 
      Medicine, Mayo Clinic, Rochester, Minnesota; Department of Molecular Pharmacology 
      & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics 
      Laboratory, Mayo Clinic, Rochester, Minnesota.
FAU - Abdelsalam, Mahmoud A
AU  - Abdelsalam MA
AD  - Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
FAU - Araoz, Philip A
AU  - Araoz PA
AD  - Department of Radiology, Mayo Clinic, Rochester, Minnesota.
FAU - Ommen, Steve R
AU  - Ommen SR
AD  - Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
FAU - Ackerman, Michael J
AU  - Ackerman MJ
AD  - Division of Pediatric Cardiology, Department of Pediatric and Adolescent 
      Medicine, Mayo Clinic, Rochester, Minnesota; Department of Cardiovascular 
      Medicine, Mayo Clinic, Rochester, Minnesota; Department of Molecular Pharmacology 
      & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics 
      Laboratory, Mayo Clinic, Rochester, Minnesota.
FAU - Geske, Jeffrey B
AU  - Geske JB
AD  - Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota. 
      Electronic address: geske.jeffrey@mayo.edu.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20200128
PL  - United States
TA  - Am J Cardiol
JT  - The American journal of cardiology
JID - 0207277
RN  - 0 (Contrast Media)
RN  - K2I13DR72L (Gadolinium DTPA)
SB  - IM
MH  - Adolescent
MH  - Age Factors
MH  - Cardiomyopathy, Hypertrophic/*diagnostic imaging
MH  - Cardiomyopathy, Hypertrophic, Familial/diagnostic imaging
MH  - Child
MH  - Contrast Media
MH  - Death, Sudden, Cardiac
MH  - Echocardiography
MH  - Female
MH  - Gadolinium DTPA
MH  - Heart Ventricles/*diagnostic imaging/pathology
MH  - Humans
MH  - Magnetic Resonance Imaging/methods
MH  - Magnetic Resonance Imaging, Cine/*methods
MH  - Male
MH  - Organ Size
MH  - Proportional Hazards Models
MH  - Risk Assessment
MH  - Ventricular Septum/*diagnostic imaging
MH  - Young Adult
EDAT- 2020/02/24 06:00
MHDA- 2020/08/04 06:00
CRDT- 2020/02/24 06:00
PHST- 2019/11/14 00:00 [received]
PHST- 2020/01/13 00:00 [revised]
PHST- 2020/01/17 00:00 [accepted]
PHST- 2020/02/24 06:00 [pubmed]
PHST- 2020/08/04 06:00 [medline]
PHST- 2020/02/24 06:00 [entrez]
AID - S0002-9149(20)30068-0 [pii]
AID - 10.1016/j.amjcard.2020.01.027 [doi]
PST - ppublish
SO  - Am J Cardiol. 2020 Apr 15;125(8):1249-1255. doi: 10.1016/j.amjcard.2020.01.027. 
      Epub 2020 Jan 28.