DELAYED ENHANCEMENT IMAGING IN CHILDREN
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1 NASCI 38 TH ANNUAL MEENG, SEATLE October 3-5, DELAYED ENHANCEMENT IN CHILDREN Shi-Joon Yoo, MD Lars Grosse-Wortmann, MD University of Toronto Canada Magnitude image Magnitude images ISSUES IN CHILDREN A higher heart rate and a shorter RR Less cooperative Frequently, the RV is the primary target of imaging ARVC/D S/P TOF repair Inversion is different between RV & LV Desai, et al. J Cardiovasc Magn Reson 25;7: Inversion is not different between RV & LV Grosse-Wortmann, et al. J Cardiovasc Magn Reson 28;1:
2 1. 1. Magnitude images DELAYED ENHANCEMENT IN DIASTOLE VERSUS SYSTOLE In magnitude images: Normal myocardium = Normal myocardium < Fibrosis In PSIR images: Normal myocardium < Normal myocardium << Fibrosis -1. Phase-sensitive inversion recovery (PSIR) Diastole Systole
3 MID- DIASTOLIC Low HR Long RR MID- DIASTOLIC High HR Short RR TRIGGER DELAY TRIGGER DELAY END- SYSTOLIC Low HR Long RR END- SYSTOLIC High HR Short RR TD TD DIASTOLIC IMAGES Infarcted Myocardium Normal Myocardium Contrast IV is shortened by scanning earlier when normal myocardium has not reached an equilibrium state. SYSTOLIC IMAGES infarction myocarditis sarcoidosis DELAYED GAD ENHANCEMENT - PATTERNS - Transmural Sub-endo Sub-epi Mid-wall Multifocal Diffuse infarction sarcoidosis amyoid myocarditis pericarditis sarcoidosis DCM chronic myocarditis Courtesy to Dr. Lars Grosse-Wortmann HCM sarcoidosis PHT myocarditis amyloid Fabry s sarcoidosis Chagas DELAYED GAD ENHANCEMENT - PEDIATRIC APPLICAONS - Myocardial ischemic injury Endocardial fibroelastosis Cardiomyopathies: ARVC/D HCM Dilated cardiomyopathy Myocarditis Pulmonary hypertension Cardiac involvement of sarcoidosis Cardiac tumors
4 MYOCARDIAL INFARCON MYOCARDIAL INFARCON RETROAORC LEFT CORONARY ARTERY ALCAPA AFTER REPAIR MYOCARDIAL INFARCON MYOCARDIAL INFARCON AFTER KAWASAKI DISEASE RCA INJURY DURING AORC VALVE REPLACEMENT CRICAL AORC STENOSIS - ENDOCARDIAL FIBROELASTOSIS - Tworetzky et, al. Am J Cardiol 25;96:
5 MYOCARDIAL FIBROSIS IN ARVD/C MYOCARDIAL FIBROSIS IN ARVD/C Tandri H, et al. JACC 25:45: MR studies for ARVD/C 12 met Task Force Criteria for ARVD/C CRITERIA + (N=12) CRITERIA (N=18) DE + 8 (67%) None Biopsy + 4 (with DE+) / 9 None Inducible VT 6 / 8 with DE / 4 without DE SICKKIDS IN 28 1 CMR studies for evaluation for ARVD 34 showed 1 positive findings 65 showed no positive finding 1 failure due to sustained arrhythmia SICKKIDS IN 28 (N = 1) CMR Criterion Incidence RV dilatation 2 (1) Reduced RVEF 17 (3) Regional wall motion abnormality / 14 (4) myocardial thinning / aneurysm RVOT dilatation 2 (1) LV dilatation / reduced EF 9 () Myocardial fat Delayed Gad enhancement CASE. 11Y S/P Pulmonary valvotomy and ASD closure Hx of Cardiac Arrest and Ventricular fibrillation 11 years old History of open heart surgery No family history RBBB with anterolateral T-wave inversion Normal signal averaged ECG Can it still be ARVD/C?
6 MYOCARDILA FIBROSIS IN ARVD/C Summary Not pathognomonic for the disease. Rarely detectable in children. Almost always associated with other positive findings. DE imaging in systole might be more diagnostic than imaging in diastole. DEALYED ENHANCEMENT OR FIBROSIS IN HYPERTROPHIC CARDIOMYOPATHY DEALYED ENHANCEMENT OR FIBROSIS IN HYPERTROPHIC DEALYED ENHANCEMENT OR FIBROSIS IN HCM CARDIOMYOPATHY Rubinshtein R, et al. Circ Heart Fail 21;1: HCM patients (2-9 years, av 55) 236 (56%) showed fibrosis (.4-65% of LV) Gene positive cases: higher incidence of DE NY Heart Association class: no difference Positive DE patients are associated with higher frequency of: Nonsustained VT (27% vs 8.5%, 4.5 vs 1.1) Ventricular extrasystole (7 vs 13) Sudden cardiac death only in 4 +DE cases ICD discharge only in 4 +DE cases DEALYED ENHANCEMENT OR FIBROSIS IN HCM O Hanlon R, et al. JACC 21:56: consecutive HCM patients 136 (63%) showed fibrosis Combined primary end point* 34 (25%) of cases with fibrosis 6 (7.4%) of cases with no fibrosis DEALYED ENHANCEMENT OR FIBROSIS IN HCM Bruder A, et al. JACC 21:56: consecutive HCM patients 2 died (16 due to cardiac reasons) 2 survived sudden death Scar: Odd ratio 8.1 for cardiac mortality * Combined primary end point Cardiovascular death Unplanned cardiovascular admission Sustained ventricular tachycardia or fibrillation Appropriate implantable cardioverter-defibrillator discharge
7 DEALYED ENHANCEMENT OR FIBROSIS IN HCM Olivotto I, et al. Am J Cardiol 21:16: MYOCARDIS / CARDIOMYOPATHY IN BECKER S MUSCULODYSTROPHY 31 consecutive HCM patients Inverse relationship between extent of DE and LVEF 13-year male with chest pain, increased troponin level Endomyocardial biopsy: EB virus at PCR MYOCARDIS CHEST PAIN / + TROPONIN UNOBSTRUCTED CA Assomull RG, et al. Eur Heart J. 27 May;28(1): patients with + troponin episode of chest pain and unobstructed CA T2-weighted imaging and DE Underlying causes: Myocarditis: 5% Myocardial infarction: 11.6% Cardiomyopathy: 3.4% 15-year male with chest pain, ST segment elevation Increased troponin and CKMB levels DELAYED ENHANCEMENT IN MYOCARDIS Friedrich MG, et al. J Am Coll Cardiol 29;53: MYOCARDIAL FIBROSIS IN NON-ISCHEMIC DILATED CM Assomull RG, et al. J Am Coll Cardiol 26;48: Typically sub-epicardial LV: inferolateral and less often anteroseptal Can be multifocal or diffuse Guidance to endomyocardial biopsy Sensitivity: Active myocarditis: 84% Borderline myocarditis: 44% 11 dilated CM patients 35% showed mid-wall fibrosis: Higher rate of death Higher rate of hospitalization A predictor of sudden cardiac death / ventricular tachycardia
8 MYOCARDIAL FIBROSIS IN NON-ISCHEMIC DILATED CM Karaahmet T, et al. Echocardiography 21:May 1, Epub 65 Non-ischemic, non-hcm cardiomyopathies 38 (58%) 4 non-ischemic dilated CM patients 19 showed DE: Impaired diastolic function Higher functional class Higher N-terminal pro BNP levels Significant LV remodeling End-point of cardiac events: Hospitalization for heart failure Appropriate ICD firing Cardiac death 27 (42%) Wu KC, et al. J Am Coll Cardiol 28;51: REPAIRED TOF - MYOCARDIAL FIBROSIS - DELAYED ENHANCEMENT AFTER TOF REPAIR RV: 1% RVOT: 99% VSD patch: 98% Trabeculated myocardium: 24% LV : 53% Apical: 49% Inferior or lateral wall: 5% Other areas: 8% Babu-Narayan SV, et al. Circulation 26;113: RV insertion to septum: Superior: 11% Inferior : 8%? Babu-Narayan SV, et al. Circulation 26;113: Babu-Narayan SV, et al. Circulation 26;113:45-413
9 DELAYED ENHANCEMENT AFTER TOF REPAIR Wald R, et al. Circulation 29;119: Wald R, et al. Circulation 29;119: DELAYED ENHANCEMENT PULMONARY HYPERTENSION 23 of 25 patients Confine to insertion points in 7 Extended into septum in 16 Blyth K G et al. Eur Heart J 25;26: Blyth K G et al. Eur Heart J 25;26: McCann, G. P. et al. Am. J. Roentgenol. 27;188: McCann, G. P. et al. Am. J. Roentgenol. 27;188:
10 CARDIAC TUMOR -FIBROMA - 1. Tumor T1 DE Magnitude images T2 DE DELAYED GAD ENHANCEMENT PITFALLS High intensity signals can be from: Slow flow artifact Epicardial fat Myocardial fat Enhancement of so-called insertion points DELAYED GAD ENHANCEMENT PITFALLS
11 DELAYED GAD ENHANCEMENT So-called Insertion Points 17-year old with Marfan syndrome Babu-Narayan SV, et al. Pulmonary hypertension Circulation 26;113: Sen-Choudhry et al. ARVC Circulation 27;115: Blyth K G et al. Pulmonary hypertension Eur Heart J 25;26: DELAYED GAD ENHANCEMENT - PEDIATRIC APPLICAONS SUMMARY Diseases affecting the RV are common. A high heart rate leaves a short for imaging in diastole. Imaging in systole may allow better definition of DE of RV myocardium. Obvious DE is a bad prognostic sign of any pathologic conditions. Should be familiar with pitfalls.
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