CASE 205: MEDIC RADIOLOGY CASE 9= Noncompacted Cardiomyopathy on MSCT 640, NGUYEN TUAN VU, NGUYEN THI KIM SANG, DUONG PHI SON, PHAN THANH HAI, MEDIC MEDICAL CENTER, HCMC , VIETNAM

HISTORY

Female patient , 33 yo, presented by severe heart failure for  1 year, previous diagnosis : dilated cardiomyopathy . Decreased S1. audible S3,  2/6 apical systolic murmur.  She was sent to MEDIC for cardiac MSCT to rule out Coronary artery disease.

EKG

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

ECHOCARDIO+ TDI and 3 D Echocardiography

Decreased LV systolic function , LV diastolic dysfunction , LV diastolic dysfunction

Prominent trabeculae, spongiformed LV , Diagnostic criteria NC/C leyer > 2

Noncompacted cardiomyopathy Echocardiography: Apical 4 C view and Parasternal short axis view

Prominent trabeculations and spongiformed myocardium of LV 

MS CT 640               

Intertrabecular Recesses, Multiple Trabeculae , Predominant location at Apex, mid lateral, mid inferior . NC layer/ C layer > 2,2, Involving >2 segments, Sens. 100%, Spec. 95% (Tomography, volume 6, Issue 5, Sept.-Oct. 2012, pp 346-354)

MSCT 640: 3D Imaging =Trabeculated and spongiformed LV 

 Video clip from apex view

Summary

—  Reported by Engberding and Benber in 1984 :Mutation in LDB3, genetic cardiomyopathy

—  Myocardial sinusoids

—  Severe heart failure, arrhythmias, thrombus formation, sudden death

—  Diagnosis by  Echocardiography, MRI, MSCT

—  Medical treatment ( ACEI, Betabloker, Aspirin, Anticoagulant ), ICD, heart transplant.

—  Long term prognosis is unknown

—  Value of cardiac MSCT in patient with heart failure.