Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000222648 | SCV000271262 | likely pathogenic | Catecholaminergic polymorphic ventricular tachycardia | 2015-10-12 | criteria provided, single submitter | clinical testing | The c.(?_169)_(273_?)del variant in RYR2 results in a deletion of exon 3, which has been reported in >10 individuals with CPVT +/- LVNC, segregated with disease in >20 affected relatives from 5 families, and occurred de novo in 1 individual (Bhuiyan 2007, Medeiros-Domingo 2009, Marjamaa 2009, Szentpali 2013, Ohno 2014, Campbell 2015, Leong 2015). However, its frequency in healthy controls or the g eneral population is not available. In summary, this variant is very likely path ogenic, but the frequency information for controls is needed to determine this w ith certainty. |