1. Introduction BACKGROUND  N on-sustained VT (NSVT) is a known risk factor for poor outcomes in adults with HCM and diastolic dysfunction is linked to poor outcomes in children with HCM  Cardiac Magnetic Resonance (CMR) is the gold standard for assessment of LV mass and can detect late gadolinium enhancement (LGE), which represents myocardial fibrosis  LGE is common in adults with HCM  Incidence of LGE and the clinical utility of CMR and LGE evaluation in children with HCM are unknown OBJECTIVE  Describe CMR characteristics in children with HCM  Determine the association of LGE and LV mass with NSVT and diastolic parameters in children with HCM Utility of Late Gadolinium Enhancement and Left Ventricular Mass as Assessed by Cardiac Magnetic Resonance in Children With Hypertrophic Cardiomyopathy Joseph A Spinner MD, Cory V Noel MD, Susan W Denfield MD, Rajesh Krishnamurthy MD, Aamir Jeewa MD, William J Dreyer MD, Shiraz A Maskatia MD Resident – Baylor College of Medicine  39 patients with CMR and echo images  LGE assessed in 33/39 patients (85%)  LGE was present in 17/33 (52%) with a predilection for the interventricular septum  Presence of LGE associated with greater LV mass, maximum LV thickness, and number of LV segments with hypertrophy  Each additional segment of LGE increased the odds of having NSVT (OR 1.4; 95% CI 1.1-1.9)  LGE < 5 segments had NPV of 89% for NSVT  LGE in specific LV segments associated with NSVT  Greater maximum LV thickness was associated with NSVT (OR 2.9; 95% CI 1.2-6.8)  LV Mass correlated with a higher Mitral E/e’ ratio (Rp 0.71, p<0.01) Results Abstract Cardiac magnetic resonance (CMR) has emerged as a tool to risk- stratify adult patients with hypertrophic cardiomyopathy (HCM). The incidence of late gadolinium enhancement (LGE) and the prognostic value of CMR in children with HCM are unknown. The purpose of this study was to determine the association between the extent of LGE and LV mass index (LVMI) as evaluated by CMR with known risk factors for poor outcomes in children with HCM. We retrospectively reviewed CMR and echocardiography studies, and a blinded observer reviewed studies for the number of LV segments with LGE. We compared the CMR findings to known risk factors for poor outcomes. CMR imaging was performed in 39 patients with HCM; evaluation of LGE was performed in 33 (85%) patients of which 17 (52 %) displayed LGE. NSVT was present in 7 patients of which 5 (71%) had LGE (OR 2.92; 95% CI 0.48 –17.9). LGE in specific segments was associated with NSVT. Furthermore, each additional segment of LGE increased the odds of NSVT (OR 1.4; 95% CI 1.1-1.9) and having fewer than 5 segments with LGE had a strong negative predictive value of 89% for the presence of NSVT (OR 0.06; 95% CI 0.01-0.5). The average LVMI in patients with NSVT was 76.4 ± 40.4 g/m 2.7 compared with 50.9 ± 24.3 g/m 2.7 in those without NSVT (p=0.03). One patient in our cohort underwent heart transplant, one patient experienced aborted sudden death, and no patients died. In conclusion, LGE was common in children with HCM who underwent CMR at our institution. The extent of LGE, assessed by the number of involved LV segments, was associated with NSVT, a known risk factor for sudden cardiac death in HCM. Increased LVMI calculated by CMR was also associated with NSVT. Furthermore, increased LVMI was associated with an elevated mitral annular E/e’ ratio assessed by echocardiography, which is a marker of diastolic dysfunction and known predictor of poor outcomes in children with HCM. While clinical events were rare in our cohort, our data demonstrates the clinical utility of CMR in pediatric patients with HCM and suggests new ways to risk stratify pediatric patients with HCM. Description of intervention/study Conclusions  LGE was common in children with HCM who underwent CMR at our institution  LGE was present in many LV segments without hypertrophy  Pattern of LGE was associated with NSVT  LGE in specific segments was associated with NSVT  Extent of LGE increased odds of having NSVT  Each additional LV segment with LGE increased the odds of NSVT  Fewer than 5 segments with LGE had strong NPV  Increasing LVMI was associated with NSVT  Increasing LVMI was associated with markers of diastolic dysfunction  These data suggest important roles of LGE assessment and the determination of LV mass in the care of children with HCM  Prospective study is necessary to assess the incidence of LGE in children with HCM and associated outcomes References 1.Maron JAMA 2002 2.Cannon RO III, N Engl J Med 2003 3.McMahon et al. Circulation 2004 4.Maskatia et al. Pediatr Cardiol 2012 5.Olivotto, et al. J Am Coll Cardiol, 2008 6.Green et al. JACC Cardiovascular Imaging, 2012 7.Moon et al. J Am Coll Cardiol, 2008 8.Adabag, et al. J Am Coll Cardiol 2008 Texas Pediatric Society Electronic Poster Contest Retrospective review of CMR and echo studies of patients with HCM performed at our center from 2005 – 2013 as part of routine clinical care. All echos reviewed were performed within one year of CMR A blinded expert reviewed all CMR studies and assessed for maximal LV thickness of any segment and all 17 LV segments for presence of hypertrophy and LGE. CMR reports reviewed for: LVEDV, LVEF, and LV mass indexed to height 2.7 (LVMI) We evaluated the association of the above measurements to non-sustained VT and diastolic parameters by echo Primary Outcome: Non-sustained VT Secondary Outcome: Parameters of diastolic function assessed by echo Pre-Gadolinium LGE Distribution of LGE vs LVH NO LGE LGE Always with Hypertrophy LGE ± Hypertrophy Distribution of LGE N% 00 1-53-15 6-1018-30 11-1533-45 LGE vs NO LGE CMR Measure LGE (n=17) No LGE (n=16) p-value LVEDV/m 2 87.7 (±22.0)82.8 (±19.2)0.50 LVEF (%)65.6 (±14.3)71.4 (±6.3)0.15 LVMI (g/m 2.7 )67.2 (±33.5)42.3 (±15.1)0.01 Max Thickness (cm)2.5 (±1.1)1.5 (±0.3)< 0.01 # Segments with Hypertrophy 10 (7-11.5)7 (5-9)0.02 Echo Parameters of Diastolic Function Mitral E/e’10.4 (±3.4)8.2 (±2.8)0.06 Mitral E/A1.6 (±0.4)1.9 (±0.5)0.11 Non-Sustained VT vs no NSVT CMR Measure NSVT (n=7) No NSVT (n=26) Odds Ratio / p-value Presence of LGE (# of patients, %) 5 (71.4%)12 (46.2%) OR = 2.92 (0.48 –17.9) LGE < 5 segments2 (29%)24 (92%) OR = 0.06 (0.01-0.5) LV Mass Index (g/m 2.7 )76.4 (±40.4)50.9 (±24.3)p = 0.03