CARDIAC MRI TISSUE CHARACTERIZATION AND MYOCARDIAL MECHANICS IN PEDIATRIC PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY

Introduction: Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) has been shown to be an independent predictor of sudden cardiac death (SCD) in adults with hypertrophic cardiomyopathy (HCM). The clinical significance of LGE in pediatric HCM patients is unknown. Hypothesis: LGE improves the SCD risk prediction in children with HCM. Methods: We retrospectively analyzed the CMR images and reviewed the outcomes pediatric HCM patients. Results: Amongst the 720 patients from 30 centers, 73% were male, with a mean age of 14.2±4.8 years. During a mean follow up of 2.6±2.7 years (range 0-14.8 years), 34 experienced an episode of SCD or equivalent. LGE (Figure 1A) was present in 34%, with a mean burden of 14±21g, or 2.5±8.2g/m2 (6.2±7.7% of LV myocardium). The presence of ≥1 adult traditional risk factor (family history of SCD, syncope, LV thickness >30mm, non-sustained ventricular tachycardia on Holter) was associated with an increased risk of SCD (HR=4.6, p<0.0001). The HCM Risk-Kids score predicted SCD (p=0.002). The presence of LGE was strongly associated with an increased risk (HR=3.8, p=0.0003), even after adjusting for traditional risk factors (HR adj =3.2, p=0.003) or the HCM Risk-Kids score (HR adj =3.5, p=0.003). Furthermore, the burden of LGE was associated with increased risk (HR=2.1/10% LGE, p<0.0001). LGE burden remained independently associated with an increased risk for SCD after adjusting for traditional risk factors (HRadj=1.5/10% LGE, p=0.04) or HCM Risk-Kids (HRadj=1.9/10% LGE, p=0.0018, Figure 1B). The addition of LGE burden improved the predictive model using traditional risk markers (C statistic 0.67 vs 0.77, p=0.003) and HCM Risk-Kids (C statistic 0.68 vs 0.74, p=0.045). Conclusions: Quantitative LGE is an independent risk factor for SCD in pediatric patients with HCM and improves the performance of traditional risk markers and the HCM Risk-Kids Score for SCD risk stratification in this population.

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Abstract 15632: Phenotypic Overlap Between Left Ventricular Noncompaction and Hypertrophic Cardiomyopathy: A Case Report

Circulation ◽

10.1161/circ.142.suppl_3.15632 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Ethan Senser ◽

Madison Hawkins ◽

Eric M Williams ◽

Lauren Gilstrap

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Cardiac Mri ◽

Genetic Diagnosis ◽

Lateral Wall ◽

Anterior Wall ◽

Left Ventricular ◽

Ventricular Noncompaction ◽

History Of ◽

Clinically Significant

Introduction: Left ventricular non-compaction (LVNC) is characterized by extensively trabeculaed myocardium adjacent to normal compacted myocardium of the left ventricle (LV). Hypertrophic cardiomyopathy (HCM) typically appears as diffuse or segmental LV hypertrophy, with or without outflow tract obstruction. Cardiac sarcomere mutations are present in most HCM cases and have also been identified in LVNC. Whether or not there is clinically significant phenotypic overlap between the two diseases is less well understood. We present a case of known HCM that met criteria for both LVNC and HCM by cardiac MRI. Case: A 49-year old man with HCM due to a c.3742_3759dup variant in MYBPC3 presented to clinic after an episode of syncope and ICD firing. In clinic, the device was interrogated and he was found to have had ventricular flutter which was successfully treated with one shock and a new, high (>20%) burden of premature ventricular beats. An echocardiogram showed a stable ejection fraction at 42%, mild concentric LV hypertrophy without obstruction and a newly dilated LV with an end diastolic diameter of 7.1cm (previously 6.2cm). A cardiac MRI was performed ( Figure ) and showed LV noncompaction and diffuse transmural and mid myocardial hyperenhancement/fibrosis of the septum, basilar lateral wall, anterior wall, and distal right ventricle consistent with patient's long-standing history of hypertrophic cardiomyopathy. Discussion: This case highlights the phenotypic overlap between HCM and LVNC by cardiac MRI. Had this patient not already carried a genetic diagnosis of HCM, he would likely have been diagnosed with LVNC based on this cardiac MRI. The phenotypic overlap in these diseases raises questions about ICD guidelines, the role of anticoagulation and prognosis.

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CHARACTERISTICS AND LONGER-TERM OUTCOMES OF PEDIATRIC PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY: A TERTIARY CARE CENTER EXPERIENCE

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(19)31485-8 ◽

2019 ◽

Vol 73 (9) ◽

pp. 878

Author(s):

Alaa Alashi ◽

Laurence Svensson ◽

Jared Klein ◽

Kenneth Zahka ◽

Nicholas Smedira ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Pediatric Patients ◽

Care Center ◽

Tertiary Care ◽

Tertiary Care Center

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Myocardial Mechanics Explains the Time Course of Benefit for Septal Ethanol Ablation for Hypertrophic Cardiomyopathy

Journal of the American Society of Echocardiography ◽

10.1016/j.echo.2007.08.020 ◽

2008 ◽

Vol 21 (5) ◽

pp. 493-499 ◽

Cited By ~ 35

Author(s):

Shemy Carasso ◽

Anna Woo ◽

Hua Yang ◽

Leonard Schwartz ◽

Mani A. Vannan ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Time Course ◽

Ethanol Ablation ◽

Myocardial Mechanics

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Predicting Risk Stratification In Hypertrophic Cardiomyopathy With Fibrosis Burden And Scar Index Score With Cardiac Mri

International Journal of Pharmaceutical Research ◽

10.31838/ijpr/2021.13.03.182 ◽

2021 ◽

Vol 13 (03) ◽

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Risk Stratification ◽

Cardiac Mri ◽

Index Score

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Speckle tracking deformation imaging to detect regional fibrosis in hypertrophic cardiomyopathy: a comparison between 2D and 3D echo modalities

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa057 ◽

2020 ◽

Vol 21 (11) ◽

pp. 1262-1272 ◽

Cited By ~ 1

Author(s):

Efstathios D Pagourelias ◽

Oana Mirea ◽

Jürgen Duchenne ◽

Serkan Unlu ◽

Johan Van Cleemput ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Speckle Tracking ◽

Tissue Characterization ◽

Three Dimensional ◽

Area Under The Curve ◽

Deformation Parameters ◽

Maximum Wall Thickness ◽

3D Deformation ◽

2D And 3D ◽

Lge Cmr

Abstract Aims We aimed at directly comparing three-dimensional (3D) and two-dimensional (2D) deformation parameters in hypertrophic hearts and depict which may best reflect underlying fibrosis in hypertrophic cardiomyopathy (HCM), defined by late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR). Methods and results We included 40 HCM [54.1 ± 14.3 years, 82.5% male, maximum wall thickness (MWT) 19.3 ± 4.8 mm] and 15 hypertensive (HTN) patients showing myocardial hypertrophy (58.1 ± 15.6 years, 80% male, MWT 12.8 ± 1.4 mm) who have consecutively undergone 2D-, 3D-speckle tracking echocardiography and LGE CMR. Deformation parameters (2D and 3D) presented overall poor to moderate correlations, with 3D_longitudinal strain (LS) and 3D_circumferential strain (CS) values being constantly higher compared to 2D derivatives. By regression analysis, hypertrophy substrate (HCM vs. hypertension) and hypertrophy magnitude were the parameters to influence 2D–3D LS and CS strain correlations (R2 = 0.66, P < 0.001 and R2 = 0.5, P = 0.001 accordingly). Among segmental deformation indices, 2D_LS showed the best area under the curve [AUC = 0.78, 95% confidence intervals (CI) (0.75–0.81), P < 0.0005] to detect fibrosis, with 3D deformation parameters showing similar AUC (0.65) and 3D_LS presenting the highest specificity [93.1%, 95% CI (90.6–95.1)]. Conclusions In hypertrophic hearts, 2D and 3D deformation parameters are not interchangeable, showing modest correlations. Thickness, substrate, and tracking algorithm calculating assumptions seem to induce this variability. Nevertheless, among HCM patients 2D_peak segmental longitudinal strain remains the best strain parameter for tissue characterization and fibrosis detection.

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