List 1.

Inclusionary echocardiographic criteria for the Pediatric Cardiomyopathy Registry

Measurements

• Left ventricular fractional shortening or ejection fraction >2 standard deviations below the normal mean for age. Left ventricular fractional shortening is acceptable in children with a normal ventricular configuration and without abnormal regional wall motion. Abnormal ejection fractions detected by echocardiography, radionuclide or contrast angiography, or MRI are acceptable alternatives but age-appropriate norms for the individual laboratory must be applied.

• Left ventricular posterior wall thickness at end-diastole >2 standard deviations above the normal mean for body-surface area.

• Left ventricular posterior wall thickness at end-systole >2 standard deviations below the normal mean for body-surface area.

• Left ventricular end-diastolic dimension or volume >2 standard deviations above the normal mean for body-surface area. Dimension data are acceptable under the conditions outlined for left ventricular fractional shortening above, and volume data may be derived from the imaging methods as above.

Patterns

• Localized ventricular hypertrophy: such as, septal thickness >1.5 × left ventricular posterior wall thickness with at least normal left ventricular posterior wall thickness, with or without dynamic outflow obstruction.

• Restrictive cardiomyopathy: one or both atria enlarged relative to the ventricles of normal or small size with evidence of impaired diastolic filling and in the absence of marked valvular heart disease.

• Contracted form of endocardial fibroelastosis: similar to restrictive cardiomyopathy plus an echo-dense endocardium.

• Ventricular dysplasia or Uhl's congenital anomaly: a very thin right ventricle with a dilated right atrium (usually better assessed by MRI than by echocardiography).

• Concentric hypertrophy in the absence of a hemodynamic cause: a single measurement of LV posterior wall thickness at end-diastole >2 standard deviations suffices.

• Left ventricular myocardial noncompaction: highly trabeculated spongiform left ventricle myocardium with multiple interstices.