A 54-year-old male with severe aortic regurgitation (AR), aortic root aneurysm, left ventricular hyper-trabeculation/noncompaction (LVHT) and systolic dysfunction with a left ventricular ejection fraction (LVEF) of 52% underwent successful aortic root replacement. Intraoperative video-endoscopy confirmed LVHT. At 3-year follow-up, he remains in an excellent clinical condition and echocardiography shows an improvement of the systolic function, LVHT and LVEF of 66%. Timely surgical correction of severe AR may also lead to improvement of systolic function in a patient with LVHT.
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