Cardiomyopathy. Cardiomyopathies HOCM. Hypertrophic Obstructive Cardiomyopathy. Systolic Anterior Movement (SAM) of Mitral Valve (Venturi Effect) Cine

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1 Jens Bremerich Radiology University Hospital Basel Hypertrophic Obstructive Cine VENC Cine (5m/s) Modified Bernoulli Equation: P (in mmhg) = 4 x (Vmax)2 Vmax= 4.2 m/s, P = 70mm Hg Hydrodynamica 738 HOCM Systolic Anterior Movement (SAM) of Mitral Valve (Venturi Effect)

2 Cine Adeno Rest HOCM Take home points: H(O)CM HCM most frequent cardiomyopathy in US (:500) Autosomal dominant Most frequent cause of death in young adults in US MR features Elevated mass & EF Systolic obstruction of LVOT in HOCM (ARVC) Cine TrueFisp T-TSE T-TSE T-TSE-FS T-TSE

3 ARVC ARVC To predict arrhythmia free survival Diagnostic criteria on MRI Arrhythmic events Severe dilatation RV, (almost) normal LV. Aneurysm RV with bulging. Fatty infiltration. Minor Mild dilatation RV. Regional RV hypokinesia. Event free survival Major CMR neg..8.6 CMR pos MR neg: Symptomatic VT MR pos: Symptomatic VT Sudden Death Appropriate ICD Shock 4 36 Follow up (5-53 months) Arrhythmogenic right ventricular cardiomypathy: diagnostic and prognostic value of the cardiac MRI in relation to arrhythmia-free survival. Haverkamp et al. Herz 2005; 30: McKenna et al. Br Heart J 7: 25, 994 Keller DI, Osswald S, Bremerich J, Bongartz G, Cron TA, Hilti P, Pfisterer ME, Buser PT. Int J Cardiovasc Imag : Uhl s disease Bomma C et al. Evolving role of MDCT in evaluation of ARVC. Am J Cardiol 2007 Take home points: ARVC Uncommen (:5000) More frequent in Italy and Greece Spectrum: RVOT Tachycardia to Uhl disease. Uhl s disease: Dilated congestive cardiomyopathy limited to the RV. Initially discribed in 952 by Uhl in an infant with severe RV dysfunction and total absence of RV myocardium. MR is a piece of a diagnostic puzzle with minor/major features MR can exclude ARVC

4 Non Compaction Non Compaction 9 year old man. Heart failure NYHA II. Echocardiography Trabeculations in LV. EF 32% Non Compaction Non-Compaction ~ 4 w ~ 5 w ~ 2 w normal non-comp Vanderdood et al. ECR 2003 Non Compaction Take home points: Non-Compaction Imaging features: No coexisting cardiac abnormaly. Non-Compact inner layer. Compact outer layer. Non-Compact/Compact > 2. Complications: Heart failure with focal or global motion abnormalities. Ventricular tachyarrhythmia. Systemic thrombembolism.

5 Dilated 53 year old man Palpitations, non-sustained ventricular tachycardia, peristent atrial fibrillation. Dyspnea NYHA II; Nycturia (2x) Coronary angiography (2003): normal Father died at age 45 sudden cardiac death Echocardiography (referring physician) inconclusive Non compaction / ARVC / Dilatation / LVEF 50% Dilated T mapping to quantify fibrosis Cine TrueFisp Late Enhancement Iles RG, et al. Evaluation of diffuse myocardial fibrosis in heart failure with cardiac MR contrast enhanced T-mapping. JACC 2008 Dilated 23 year old male hockeyplayer Increasing weakness and fatigue Sudden onset of left hand palsis Elevated markers: Inflammation Myocardial damage DCM

6 Take home points: DCM Prevalence :2500 Most frequent cause of transplantation Etiology: ~ 30% genetic Toxic Postmyocarditis. MR features: Increased EDV, ESV, poor LVEF Late enhancement mittmyocardial >4.8% poor prognosis 3 months Baseline Takotsubo CineTrueFisp T2w-TSE Late Enhancement Takotsubo 60 yo woman Acute chest pain Chorus singer Cardiac enzymes slightly el. ST elevation V 3 -V 5 Hara T et al. Noninvasive detection of Takotsubo cardiomyopathy using multidetector row CT. Int Heart J 2007 Conclusion Echocardiography st line-modality. Added value of MR: Fokal Hypertrophy Identify fatty infiltration Goldstandard Volumes/Mass Risk Stratification Added value of CT: Coronary angiography Calcification / Fat Short examination time Contraindication for MR