Abstract

In 2000 the description of a family (a mother and three sons) with a typical long QT ECG and echocardiographic diagnosis of classical hypertrophic non-obstructive cardiomyopathy was published [1]. The ECG of three family members was characterised by typical long QT syndrome (QTc intervals between 460 up to nearly 500 msec), but without signs of left ventricular hypertrophy, pathologic Q-wave and T-wave abnormalities as shown in ECG figures [1]. The mother of the family developed atypical angina; coronary angiography revealed slight coronary abnormalities. One of her sons had pre-syncope. In the later follow-up the mother developed severe ventricular arrhythmias; the implantation of an ICD has been discussed.

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