From: Knock-in Kcnh2 rabbit model of long QT syndrome type-2, epilepsy, and sudden death
Case # | Genotype | Age (months) | Sex | Time from last seen alive | Rigor* | Necropsy Findings | History of Epileptiform Activity | Manifestations at Time of Death | Mechanism of Sudden Death |
---|---|---|---|---|---|---|---|---|---|
1 | Kcnh2(+/7bp−del) | 0.4 | Female | Witnessed & Recorded | Yes | None | Yes | Myoclonic jerks, GTCS, tonic limb/head extension, PGES, bradycardia, ECG T-wave inversion, 2nd & 3rd degree AV block, cardiac focal activity,& asystole | Status Epilepticus |
2 | Kcnh2(+/7bp−del) | 1.8 | Male | Witnessed & Recorded | Yes | None | No | Atrial bigeminy, bradycardia, ECG ST-elevation/depression (ischemia), PVCs, 2nd degree AV block, asystole | SCD |
3 | Kcnh2(+/7bp−del) | 11 | Female | Witnessed | Yes | None | N/A | Loss of consciousness with convulsions during routine blood draw | unknown |
4 | Kcnh2(+/7bp−del) | 13.4 | Female | < 24 h | Yes | None | No | Found in the cage in rigor | unknown |
5 | Kcnh2(+/7bp−del) | 21.7 | Female | 2 h | Yes | None | No | Found in the cage in rigor | unknown |
6 | Kcnh2(+/7bp−del) | 25.3 | Female | < 7 h | Yes | None | N/A | Found in the cage in rigor | unknown |
7 | Kcnh2(+/7bp−del) | 30 | Male | < 2 h | Yes | None | No | Found in the cage in rigor | unknown |
8 | Kcnh2(+/+) | 2 | Female | Video Recorded | Yes | None | Yes | Convulsive Syncope | SCD |