Table 1. Definitions of cardiac abnormalities found in NMDs.
dCMP | Combination of abnormal LV function (LVFS Z score or LVEF Z score<-2) and LV dilation (LV EDD Z score>2); or the presence of at least moderately depressed LV function, defined as LV fractional shortening Z score<-3, or LV ejection fraction Z score<-3, regardless of LV sizep |
hCMP | Either echocardiographic evidence of localized ventricular hypertrophy or a posterior wall or septal thickness Z score>3, normal coronary angiography, normal or decreased systolic function, normal dimensions or dilation of cardiac cavities184) |
rCMP | One or both atria enlarged relative to ventricles of normal or small size with evidence of impaired diastolic filling in the absence of significant valvular heart disease |
LVHT | Two-layered structure of myocardium comprising a non-compacted thick layer and compacted thin layer with intertrabecular spaces perfused from the ventricular side. Systolic function may be normal or reduced |
TTS | Segmental hypokinesia or akinesia (stunning) and reduced systolic function and compensatory hyperkinesia of segments not affected by the stunning. Most frequently, stunning affects the apical and midventricular segments of the left ventricular myocardium (classical type, apical ballooning), but rarely also the midventricular segments (midventricular type), the basal and midventricular segments (inverted type), or all segments (global type) |
Myocardial thickening | Posterior wall thickness, septum thickness>11 mm |
Aortic root ectasia | Aortic root diameter 41-50 mm |
Sudden cardiac death | Unexpected death occurring<1 hour after the onset of a symptomatic cardiac event |
Heart failure | Exertional or resting dyspnea, edema, neck vein distension, and echocardiographically EF<50% or FS<25% |
NMDs: neuromuscular disorders, dCMP: dilated cardiomyopathy, LV: left ventricular, LVFS: left ventricular fractional shortening, LVEF: left ventricular ejection fraction, EDD: end-diastolic diameter, hCMP: hypertrophic cardiomyopathy, rCMP: restrictive cardiomyopathy, LVHT: left ventricular hypertrabeculation/noncompaction, TTS: Takotsubo syndrome, EF: ejection fraction, FS: fractional shortening