DPP6

A new arrhythmia gene on chromosome 7q36, DPP6, is linked to familial IVF and shows a very malignant phenotype. This phenotype consists of cardiac arrest by IVF at young age, which is elicited by short coupled extrasystoles. While this gene has not earlier been related to cardiac arrhythmias, we are now able, for the first time, to identify presymptomatic family members at risk for future fatal events solely by genetic analysis. Affected patients can be offered preventive treatment accordingly. Therefore, when there is a family history of unexplained sudden cardiac deaths, we make the reader aware of this particular link to the DPP6 gene as it may enable risk stratification of the remaining family members. In addition, when closely coupled extrasystoles initiate ventricular fibrillation in the absence of other identifiable causes, a link to the DPP6 gene should also be suspected.[1]

References

1. Postema PG, Christiaans I, Hofman N, Alders M, Koopmann TT, Bezzina CR, Loh P, Zeppenfeld K, Volders PG, and Wilde AA. Founder mutations in the Netherlands: familial idiopathic ventricular fibrillation and DPP6. Neth Heart J. 2011 Jun;19(6):290-6. DOI:10.1007/s12471-011-0102-8 |