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1 Supplementary Online Content Tseng ZH, Hayward RM, Clark NM, et al. Sudden death in patients with cardiac implantable electronic devices. JAMA Intern Med. Published online June 22, doi: /jamainternmed etable. Details of Investigation for SCDs With Cardiovascular Implantable Electronic Devices efigure 1. Case 5 efigure 2. Case 1 efigure 3. Case 2 efigure 4. Case 3 efigure 5. Case 4 efigure 6. Case 15 efigure 7. Case 18 efigure 8. Case 19 efigure 9. Case 20 efigure 10. Case 21 This supplementary material has been provided by the authors to give readers additional information about their work.

2 etable 1. Details of Investigation for SCDs with Cardiovascular Implantable Electronic Devices Case Patient Details Device Autopsy Findings Device Interrogation Terminal Rhythm Adjudicated COD Device Concern Sudden battery depletion from 2.62V (5 weeks prior to death) to 2.17V Presumed profound Yes (Battery; Hardware 1 70 with CHB, AF DC PPM No MI or other acute COD Arrhythmic (day of death). See efigure 2. bradycardia/asystole failure) Yes (ICD was indicated at 42 with CHB, HCM, Hypertrophic CM with 3.0 cm 2 DC PPM Normal PPM function with VF at time of death. See efigure 3. VF Arrhythmic time of PPM; Improper syncope septum. No acute MI device selection) 3 77 with sick sinus syndrome, advanced AV block, CAD s/p MI, CVA, Alzheimer's dz DC PPM 4 60 with CHB, AF DC PPM No MI; acute bronchopneumonia Rectal adenocarcinoma, cardiomegaly, 30% LAD stenosis, and severe myocardial fibrosis. No MI or acute COD No VT/VF. Rapid rise in RV lead impedance (650 >1620 ohms) 1wk prior to death concerning for lead fracture. See efigure 4. Increase in RV lead impedance 2 days prior to death. PMVT/VF at the time of death. See efigure 5. PEA PMVT/VF Pneumonia Arrhythmic Yes (RV lead; Possible hardware failure) Yes (RV lead; Hardware failure) 5 26 with 2nd deg. HB, L Normal PPM function w/o recorded events at time of death. See efigure Massive pulmonary DC PPM Massive pulmonary hemorrhage PEA TGA s/p mechanical TVR 1. hemorrhage No 6 87 with 2nd deg. HB, Intracranial DC PPM Intracranial hemorrhage Acute increase in RV lead impedance (380 >1340 ohms) post mortem. AF w/ RVR >Asystole AF, CAD s/p MI hemorrhage No 7 98 with CHB, AF DC PPM Cardiac (heart Pulmonary edema and scar from Normal PPM function w/o recorded events at the time of death. PEA (AS VP) failure), nonarrhythmic remote MI but no acute MI No 8 70 with sinus node Scar from remote MI. No acute MI DC PPM dysfunction or other COD Normal PPM function with VF at the time of death. VF Arrhythmic No 9 84 with CHB, AF DC PPM No acute cause of death No electrograms recorded (device was ERI), but intervals suggest VF. VF Arrhythmic No with CHB, CHF DC PPM Dilated CM w/o MI or other acute COD Normal PPM function. Device at ERI, full interrogation not possible. Unknown Arrhythmic No with AF and Blunt force injuries of head/neck DC PPM bradycardia w/ CAD Normal PPM function. AF with RVR at time of death. AF with RVR Occult trauma No with CHB, AF, CAD s/p MI, CHF 87 with tachy brady syndrome 77 with CHB, CAD, idiopathic CM DC PPM DC PPM with Dilated CM DC ICD with Ischemic CM, AF, CAD 70 with Ischemic CM, CHF 74 with CAD s/p MI, ischemic CM, AF, VT No MI. Acute lung inflammation due to pneumonia No MI or other acute COD. 50% LAD stenosis Normal PPM function. NSVT > Idioventricular rhythm Pneumonia Normal PPM function with VF at time of death. VF Arrhythmic No CRT P N/A No device interrogation. VF recorded by paramedics. VF Arrhythmic No DC ICD CRT D DC ICD Massive subarachnoid hemorrhage, cardiomegaly (heart 760g) Scar from remote MI and CM but no acute MI or other COD CM without acute MI or other COD Scar from remote MI and 99% LAD stenosis but no acute MI VF documented at time of death (likely secondary to subarachnoid hemorrhage). See efigure 6. Episodes of VF during EMS rescue were not recorded by device and required external shocks for rescue. Delay to shock due to ATP programming in the VF zone. See Figure 2. VF undersensed with device interpreting return to sinus rhythm (no shock delivered). See Figure 3. VF with undersensing. 40 VF and 2 VT episodes identified and 17/42 shocks aborted due to undersensing. The second event showed significant undersensing with a significant delay in therapy. See efigure 7. VF VF VF Subarachnoid hemorrhage Arrhythmic Arrhythmic No No Yes (VF episodes missed; Programming and Device algorithm issue) Yes (VF undersensing; Device algorithm issue) VF Arrhythmic Yes (VF undersensing)

3 with dilated CM, ESRD on HD 71 with AF, CAD, ischemic CM 80 with ischemic CM, CAD, CHF 80 with Ischemic CM, AF DC ICD SC ICD CRT D DC ICD N/A Acute RCA thrombus. Cardiomegaly, cirrhosis, COPD N/A Hypertensive heart disease, COPD VT that was undersensed. This was treated with ATP but wavering VT cycle length resulted in a delayed shock. VT degraded into fine VF that was undersensed. Patient left in VF/VT and device stopped recording and was unable to detect after that point. See efigure 8. Extended VF with undersensing leading to a delay in interval counts that delayed therapy. See efigure 9. VF storm with 4 shocks and multiple rounds of ATP. Final event shows VT but device did not rescue due to programming of tachycardia zone. See efigure 10. VF with successful defibrillation x 3 followed by RV lead noise and increase in lead impedance with 4th shock suggesting lead fracture. See Figure 4. VF Arrhythmic Yes (VT undersensing) VF VT VF Arrhythmic Arrhythmic Arrhythmic Yes (VF undersensing, Programming and Device algorithm issue) Yes (VT slower than VT zone; Programming) Yes (Lead fracture; Hardware failure) AF atrial fibrillation, CAD coronary artery disease, CHB complete heart block, CHF congestive heart failure, CM cardiomyopathy, COD cause of death, CRT P cardiac resynchronization therapy pacemaker, CRT D cardiac resynchronization therapy defibrillator, DC dual chamber, MI myocardial infarction, PEA pulseless electrical activity, PPM permanent pacemaker, RV right ventricle, RVR rapid ventricular response, SC single chamber, VF ventricular fibrillation, VT ventricular tachycardia

4 efigure 1 (Case 5). Left: Post mortem device interrogation showing no ventricular high rate episodes. Right: H&E section of lung (12.5X) demonstrating dilated bronchial veins (arrows) and hemosiderin laden macrophages (arrowheads). Reprinted with permission from Hayward RM, Ursell PC, Foster E, Tseng ZH. Sudden death due to nonarrhythmic cause in a patient with L TGA. Ann Noninvasive Electrocardiol. 2014;19(3):

5 efigure 2 (Case 1). Top: PPM interrogation 5 weeks prior to death shows estimated battery longevity of 6 months and > 95% ventricular pacing. The patient s PVC burden was at least 3%, which accounted for most of the non-paced ventricular beats and underlying rhythm at device check was sinus bradycardia with complete heart block and a slow junctional escape. The patient s ventricular pacing threshold at this this time was 2.5V with a pulse width of 0.4 ms. Bottom: Pacemaker interrogation within 1 day after death demonstrates excessive voltage decrease of 0.46 V consistent with rapid battery depletion and no ventricular high rate episodes.

6 efigure 3 (Case 2). Top: Atrial marker channel. Middle: Ventricular marker channel. Bottom: Intervals. VF at the time of death. ICD is a IIa recommendation in 2008 ACC/AHA/HRS Guidelines for HCM patients with unexplained syncope and septal thickness > 30 mm. This patient died of VF with a PPM in place.

7 efigure 4 (Case 3). Postmortem PPM interrogation demonstrating a rapid substantial increase in atrial and ventricular lead impedances that was first detected 2 days prior to death. The patient died suddenly on January 13, The intrinsic R-wave was measured at 5.5 mv on the day prior to death, suggesting intrinsic ventricular activity. However, lead fracture can be intermittent and the sensing could have been due to junctional or ventricular escape beats. Atrial lead impedance also increased, raising the possibility of changes due to acidosis or electrolyte disturbances, but atrial lead impedance normalized on the day of death. In this case, although pneumonia was found on autopsy and occult pneumonia was adjudicated as the COD, right ventricular lead fracture or a global pacemaker problem resulting in profound bradycardia or asystole could not be excluded as a contributor to sudden death.

8 efigure 5 (Case 4). Top: Increase in ventricular lead impedance 2 days prior to death (the patient died suddenly on 11/3/13). Bottom: Ventricular electrogram shows PMVT/VF at the time of death. No acute COD was found on autopsy. The lead malfunction may have led directly to VT/VF via pause dependent mechanism caused by inconsistent ventricular capture or R on T phenomenon due to undersensing.

9 efigure 6 (Case 15). 78M with dilated CM and VF documented at the time of death which was recognized and treated by the ICD (Left). VF was due to hemorrhage in the right insular cortex (Right) leading to arrhythmia (neurocardiogenic VF).

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11 efigure 7 (Case 18). The patient had 40 VF and 2 VT episodes identified and 17 of 42 shocks aborted due to undersensing. Top: atrial electrogram. Middle: ventricular electrogram. Bottom: device markers. Strips are continuous. This event shows a return to sinus (*) after the previous shock due to undersensing of VF (arrowheads). This resulted in a significant delay in therapy.

12 efigure 8 (Case 19). Top: atrial electrogram. Middle: ventricular electrogram. Bottom: Shock electrogram. After 3 unsuccessful ATP attempts, the patient was in VT/VF. Undersensing resulted in a wavering VT cycle length and diversion of charging (*). The device redetected VF and delivered a shock, but undersensing resulted in a delay to therapy of 12.2 seconds (A). Ultimately, the patient was left in VF that the device was unable to detect and stopped recording (B). The patient s final recorded rhythm, which showed undersensing of VF. Intervals show varying cycle lengths wavering in and out of VT zone (C).

13 efigure 9 (Case 20). Top and Middle Strips (continuous): Extended VF event with undersensing (arrowheads) that delays therapy. In addition, ATP delays shock therapy. Less than 1 minute later, the patient had multiple additional episodes of VT/VF requiring defibrillation. Bottom Strip: 4 minutes later, the final rhythm recorded by the device was VF with no further shocks delivered. Autopsy showed acute RCA thrombus. Cumulative delays to shock may have resulted in refractoriness of VF and further opportunity for undersensing.

14 efigure 10 (Case 21). Top: atrial electrogram. Middle: ventricular electrogram. Bottom: device markers. After 4 shocks and multiple rounds of ATP, the patient is left in VT (mean cycle length 447 msec) but the device did not rescue due to the programming of the tachycardia zone (VT detection zone >166 bpm or <360 msec).