Cardiomyopathy

Jens Bremerich

RadiologyUniversity Hospital Basel

Cardiomyopathies

Contemporary Definitions and Classification of the Cardiomyopathies. Circulation 2006;113:1807-1816

VENC Cine (5m/s)

Vmax= 4.2 m/s,∆P = 70mm Hg

Modified Bernoulli Equation:∆P (in mmHg) = 4 x (Vmax)2

Cine

Hypertrophic Obstructive Cardiomyopathy

Hydrodynamica 1738

HOCMSystolic Anterior Movement (SAM)

of Mitral Valve (Venturi Effect)

Cine

Adeno

Rest

HOCMTake home points: H(O)CM

• HCM most frequent cardiomyopathy in US (1:500)

• Autosomal dominant• Most frequent cause of death in young adults in

US• MR features

– Elevated mass & EF– Systolic obstruction of LVOT in HOCM

Cardiomyopathies

Contemporary Definitions and Classification of the Cardiomyopathies. Circulation 2006;113:1807-1816 Cine TrueFisp

Arrhythmogenic Right VentricularCardiomyopathy (ARVC)

T1-TSE

Arrhythmogenic Right VentricularCardiomyopathy

T1-TSE T1-TSET1-TSE-FS

Arrhythmogenic Right VentricularCardiomyopathy

Haverkamp et al. Herz 2005; 30: 565-70

McKenna et al. Br Heart J 71: 215, 1994

ARVC• Diagnostic criteria on MRI

– Major• Severe dilatation RV,

(almost) normal LV.

• Aneurysm RV with bulging.

• Fatty infiltration.

– Minor• Mild dilatation RV.

• Regional RV hypokinesia.

Arrhythmogenic right ventricular cardiomypathy: diagnostic and prognostic value of the cardiac MRI in

relation to arrhythmia-free survival.

Keller DI, Osswald S, Bremerich J, Bongartz G, Cron TA, Hilti P, Pfisterer ME, Buser PT.

Int J Cardiovasc Imag 2003 19: 537-543

0 12 24 36

CMR neg.

CMR pos.

0

.2

.4

.6

.8

1

Follow up (5-53 months)

Eve

nt fr

ee

surv

iva

l

Arrhythmic events

MR neg:• Symptomatic VT 1

MR pos:• Symptomatic VT 1• „Sudden Death“ 1• Appropriate ICD Shock 4

ARVCTo predict arrhythmia free survival

Bomma C et al. Evolving role of MDCT in evaluation of ARVC.

Am J Cardiol 2007

Arrhythmogenic Right VentricularCardiomyopathy Uhl‘s disease

Take home points: ARVC

• Uncommen (1:5000)• 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 1952 by Uhl in an infant with severeRV dysfunction and total absence of RV myocardium.

• MR is a piece of a diagnostic puzzle with minor/major features• MR can exclude ARVC

Cardiomyopathies

Contemporary Definitions and Classification of the Cardiomyopathies. Circulation 2006;113:1807-1816

Non Compaction

• 19 year old man.

• Heart failure NYHA II.

• Echocardiography– Trabeculations in LV.

– EF 32%

Non Compaction

Non Compaction Non-Compaction

Vanderdood et al. ECR 2003

~ 4 w ~ 5 w ~ 12 w normal non-comp

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.

Cardiomyopathies

Contemporary Definitions and Classification of the Cardiomyopathies. Circulation 2006;113:1807-1816

Dilated Cardiomyopathy

• 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%

Cine TrueFisp Late Enhancement

Dilated Cardiomyopathy T1 mapping to quantify fibrosis

Iles RG, et al. Evaluation of diffuse myocardial fibrosis in heart

failure with cardiac MR contrast enhanced T1-mapping. JACC 2008

• 23 year old male hockeyplayer

• Increasing weakness and fatigue

• Sudden onset of left hand palsis

• Elevated markers:– Inflammation

– Myocardial damage

Dilated Cardiomyopathy DCM

Take home points: DCM

• Prevalence 1:2500• Most frequent cause of transplantation• Etiology:

– ~ 30% genetic– Toxic– Postmyocarditis….

• MR features:– Increased EDV, ESV, poor LVEF– Late enhancement mittmyocardial >4.8% poor prognosis

Cardiomyopathies

Contemporary Definitions and Classification of the Cardiomyopathies. Circulation 2006;113:1807-1816

Takotsubo

CineTrueFisp T2w-TSE Late Enhancement

3 m

onth

sB

ase

line

Takotsubo Cardiomyopathy

Hara T et al. Noninvasive detection of Takotsubo cardiomyopathy

using multidetector row CT. Int Heart J 2007

• 60 yo woman

• Acute chest pain

• Chorus singer

• Cardiac enzymes slightly el.

• ST elevation V3-V5

Conclusion• Echocardiography 1st 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