Left ventricular noncompaction and orthodromic atrioventricular tachycardia observed in a patient with neurofibromatosis type 1

Isolated left ventricular noncompaction (LVNC) was described for the first time in 1984. It is a rare congenital disease, characterized by prominent trabecular meshwork pattern and deep intertrabecular recesses, communicated with the left ventricular chamber. Clinical presentation varies from asymptomatic patients, to those developing supraventricular and ventricular arrhythmias, thromboembolism, heart failure and sudden cardiac death. We present an unusual case, where the patient with Neurofibromatosis type 1 presented with a wide complex orthodromic atrioventricular reentrant tachycardia (AVRT) and a diagnosis of left posterior paraseptal accessory pathway in association with LVNC.