CONTENT
A 28-year-old man with acute exacerbation of dyspnea was referred to our hospital. A 12-lead electrocardiography revealed sinus rhythm with left bundle branch block. Transthoracic echocardiography showed that the dilated left ventricle (LV) was divided into 2 separate chambers by hypertrophied muscle bundles with a reduced left ventricular ejection fraction (35%). The septal chamber (main chamber, LV1) communicated with the mitral and aortic valves, measuring 3.8 × 7.5 cm. The lateral chamber (accessory chamber, LV2) was located in the lateral posterior wall with excessive trabeculation, measuring 4.5 × 8.4 cm ( (A): Transthoracic echocardiography shows that the dilated LV is divided into two separate chambers by hypertrophied muscle bundles (white arrows). (B): Color Doppler echocardiography shows no flow acceleration between the 2 chambers. (C and D): Three-dimensional transthoracic echocardiogram reveals abnormal muscle bundles that obliquely divided the LV into a septal chamber and a lateral chamber. (E and F): Contrast-enhanced echocardiography demonstrates the contrast agent filling well into LV1 and LV2. (G and H): Cardiac magnetic resonance imaging confirms the abnormal muscle bundles that divide the LV cavity into main and accessory chambers. (I): Cardiac magnetic resonance imaging shows interspersed late gadolinium enhancement of LV2. LV, left ventricle; LV1, main chamber; LV2, accessory chamber.
Double-chambered left ventricle is an extremely rare congenital anomaly characterized by the division of the LV into 2 chambers by an abnormal septum or by muscle bundles. Our case highlights that multimodality imaging plays a vital role in the diagnosis of this condition.