Left ventricular noncompaction associated with Ebstein’s anomaly

Left ventricular (LV) noncompaction is a congenital dysfunction of endomyocardial morphogenesis characterized by excessively prominent trabeculations and deep intratrabecular recesses of the myocardium. Its association with Ebstein’s anomaly is very rare. A 13-year-old boy presented to the outpatient clinic for a cardiac evaluation before enrolling in a sports activity. On physical examination, there was a grade 1/6 systolic murmur at the tricuspid valve location. His electrocardiogram was normal. Transthoracic echocardiography revealed numerous large trabeculations and deep intratrabecular recesses at the apex, lateral wall, and the apical part of the interventricular septum. Color flow Doppler examination confirmed the presence of blood flow within the trabeculae. The size and ejection fraction of the LV was normal (65%). The attached margins of the septal and inferior leaflets of the tricuspid valve were apically displaced and there was mild tricuspid regurgitation. The distance between the mitral and tricuspid annuli was 20 mm. The right ventricle showed no dilatation and had normal systolic function, but the right atrium was moderately enlarged. As the patient was asymptomatic and the LV systolic function was preserved, he was scheduled for regular follow-up without medication except for low-dose aspirin (100 mg/day). After five years of follow-up, he was still asymptomatic with normal size and ejection fraction of both ventricles.