| Sumario: | Background: Double‐chambered left ventricle (DCLV) is extremely rare. Challenges remain in accurate diagnosis of DCLV by echocardiography, because it is easily confused with left ventricular diverticulum, left ventricular aneurysm, ventricular septal defect, etc. Herein, we reviewed echocardiographic characteristics of DCLV and evaluated the accuracy of echocardiographic diagnosis. Methods: Clinical and echocardiographical databases of two heart centers were reviewed. Nine children with DCLV of superior–inferior arrangement were studied retrospectively. Results: Normal elliptical geometry of LV cavity is preserved in DCLV. Fibromuscluar bundles separate LV into the main chamber and the accessory chamber. The associated malformations included ventricular septal defects, mitral regurgitation, mitral valve stenosis, pulmonary stenosis, and left ventricular noncompaction in our study. Eight of nine subjects with DCLV of superior–inferior arrangement were diagnosed correctly by initial echocardiographic examination, and one case was mistaken as left ventricular diverticulum. Conclusions: Double‐chambered left ventricle could be diagnosed and followed up by echocardiography. The morphological classification is a simplified way to improve diagnostic accuracy.
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