Dr Nguyen Thi Kim SangPhD Dr Nguyen Tuan Vu

MEDIC HCM

HISTORYPt TP, 33 Y.O, F, presented by severe heart

failure for 1 yearPrevious diagnosis : dilated cardiomyopathyShe was sent to MEDIC for cardiac MSCT to

rule out Coronary artery disease.Decreased S1. audible S3, 2/6 apical systolic

murmur.

ECG

Short PR, delta wavs, QS in V2-V6, D1 aVL

Echocardiography

Decreased LV systolic function

Echocardiography

LV diastolic dysfunction

Decreased RV function

Echocardiography

Prominent trabeculae, spongyformed LV

Echocardiography

Decreased LV systolic function

Diagnostic criteria NC/C leyer > 2

Echocardiography

TDI and 3 D Echocardiography

ECHOCARDIOGRAPHYLAX4C colorApex 3c color

Noncompaction Cardiomyopathy

Intertrabecular RecessesMultiple TrabeculaePredominant location at Apex, mid lateral, mid inferior

Diagnostic Criteria

NC layer/ C layer > 2,2Involving >2 segmentsSens. 100%, Spec. 95%( Tomography, volume 6, Issue 5, Sept.-Oct. 2012, pp346-354 )

MSCT 640: 3D Imaging

Trabeculated and spongiformed LV

Coronary MSCT-angiography

LM, LAD and CX are normal

Noncompacted cardiomyopathyEchocardiography

Apical 4 C view and Parasternal short axis viewProminent trabeculations and spongiformed myocardium of LV

SummaryReported by Engberding and Benber in 1984Mutation in LDB3, genetic cardiomyopathyMyocardial sinusoidsSevere heart failure, Arrhythmias, Thrombus

formation, sudden deathDiagnosis: Echocardiography, MRI, MSCTMedical treatment ( ACEI, Betabloker, Aspirin,

Anticoagulant ), ICD, heart transplant.Long term prognosis is unknownValue of cardiac MSCT in Pt with heart

failure.

Thank you so much!