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Table 1 Detailed description of all cases of spontaneous sudden death. *: all animals that died suddenly and spontaneously were found in rigor, some within 2 h of last being seen alive. Post-mortem necropsy did not reveal any anatomical or toxicological cause of death in any case of sudden death. SCD: sudden cardiac death

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