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Correlation With Andersen Syndrome
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There is a lot of confusion whether Andersen syndrome has something to do with long QT syndrome (LQTS) or not.

Andersen syndrome (AS) is defined as a rare, inherited disorder characterized by periodic paralysis, skeletal developmental abnormalities and a prolonged QT-interval with ventricular arrhythmias.

It recently has been established that AS is caused by mutations in the gene KCNJ2. Because these  findings support the notion that AS is a disorder of myocellular repolarization, it has been classified as LQT7. To date, five genes that cause the inherited forms of long QT syndrome have been identified: KCNQ1 (LQT1), hERG (KCNH2, LQT2), SCN5A (LQT3), KCNE1 (LQT5), and KCNE2 (LQT6).

Similar to the other forms of inherited LQTS, the primary cardiac manifestation of AS was a prolonged QT-interval, identified in 71% of all gene carriers. The mean corrected QT-interval (QTc) of male and female probands with AS was 479 and 493 ms, respectively, compared with 497 and 510 ms for males and females with other forms of LQTS.

While a prolonged QT-interval was present in most KCNJ2 mutation carriers, the ventricular arrhythmias manifested by these individuals were clearly distinct from the other forms of inherited LQTS.

Therefore, AS is considered a subtype of long QT syndrome. However, this proposal is controversial at present.

Source: Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen Syndrome): J. Clin. Invest. 110:381-388 (2002).

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