Objective: To probe into the clinical characteristics of left ventricular non-compaction cardiomyopathy (LVNC) associated with long QT syndrome (LQTS) and review the literature. Methods: Clinical data of a child with LVNC associated with LQTS was retrospectively analyzed

including clinical symptoms

assistant examinations and treatment process. Results: Adams-Strokes syndrome (ASS) was the primary symptom in this child. During the onset of ECG monitoring

ventricular tachycardia (VT) was detected

including Torsades de pointes (Tdp). LVNC was observed in the echocardiography in the late stage of the disease

persistently with left ventricular dysfunction (LVEF 35%

LVFS 17%). After hospitalization

isoproterenol intravenous maintenance failed to control the onset of ASS

and temporary cardiac pacemaker combined with lidocaine intravenous maintenance treatment was given. Implantable cardioverter defibrillator (ICD) was implanted at the third week of admission. Conclusion: LVNC manifests as an undulating phenotype and associated with LQTS is relatively rare in clinic. Tdp induced by prolonged QT interval of secondary LVNC is deadly

and ecurrent Tdp is difficult to be controlled by antiarrhythmic agent

while ICD is an effective treatment.