Ecg contest Response to ECG, December 2019

This patient has a marked QT prolongation (≈ 520 ms) with T-wave macroalternans, and he presented with a cardiorespiratory arrest from which he receoved,¹ and so answer 1 is incorrect. This is an autosomal-recessive condition with a poor prognosis, which is more frequent when consanguinity is present, and which characteristically includes congenital deafness² (answer 2, correct). Given the family history of sudden cardiac death in early childhood, and the electrocardiographic abnormalities observed, diagnosis of obstructive hypertrophic cardiomyopathy is unlikely (answer 4, incorrect). Timothy syndrome, although a congenital long QT syndrome, is an autosomal-dominant form that is associated with other disorders, such as mental retardation, autism, dysmorphy, calcium metabolism disorders, and immunodeficiency, and so answer 3 is also incorrect.