Fig. 27.1
Single frontal view of the chest in a 7-year-old patient with noncompaction cardiomyopathy shows cardiomegaly and interstitial perihilar edema. Notice the interstitial lines in the periphery (arrows) from the interstitial edema
27.2.2 ECG Findings
Resting ECG findings are abnormal in most patients. Abnormalities include left ventricular hypertrophy, ST segment changes, intraventricular conduction abnormalities, atrioventricular block, and left bundle branch block.
27.2.3 Echocardiographic Findings
Primary diagnostic modality
Multiple prominent ventricular trabeculation s with deep intratrabecular recesses
Ratio of maximal thickness, noncompacted myocardium to the compacted myocardium greater than 2, measured at end systole in a parasternal short-axis view
Blood flow within the recesses, confirming communication with the ventricular cavity
Coexisting cardiac defects excluded
Evaluation for systolic hypokinesis and calculation of ejection fraction
27.2.4 MRI Findings
Left ventricle cavity normal in size or dilated
Localize and determine extent of noncompaction
Differences in myocardial signal intensity in noncompacted myocardium may help identify potential foci of lethal arrhythmia initiation
Steady-state free precession (SSFP) cine MRI
Used to evaluate for associated mitral regurgitation
Used to calculate end-systolic noncompacted-to-compacted ratio (ESNCCR) and end-diastolic noncompacted-to-compacted ratio (EDNCCR)
Ratio > 1.4 in pediatrics and >2 in adults is diagnostic (Figs. 27.2 and 27.3)
Fig. 27.2
Gradient echo (GRE) cine short-axis images from a cardiac MRI in an 8-year-old patient show prominent trabeculations (arrows). The ratio of noncompacted myocardium (2.13 cm) of the left ventricle to compacted myocardium (0.40 cm) is high. The ratio in this patient is more than 5:1
Fig. 27.3
GRE cine four-chamber images from a cardiac MRI during end systole (left) and end diastole (right) show the poor function in an 8-year-old patient with noncompaction cardiomyopathy. Notice the noncompacted lateral wall of the left ventricle (arrow)
ESNCCR has a stronger relationship to congestive heart failure, systolic dysfunction, and other cardiac events than EDNCCR
Delayed gadolinium enhancement
Identified in 60 % of patients
Associated with systolic dysfunction
27.2.5 Imaging Recommendations
Echocardiography is used for screening and is diagnostic, but cardiac MRI provides the greatest myocardial detail and is the best modality for localization and evaluation of disease extent.
27.3 Differential Diagnosis
Normal variant prominent trabeculations
At least 3 trabeculations
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