1. Unsettled and Unanswered Questions Christopher Caldarone AATS symposium April 26 th, 2015

2. Case report HLHS, 5d, 3.1 kg AS/MS –AoV 3.5mm –MiV 4mm –Mildly restrictive ASD ‘Serpiginous’ isthmus Good candidate –Bilateral PAB, ductal stent –No reverse BT shunt Judgment Error #1

3. Post-op 5 hours postop –Rt radial arterial line ‘not working’ –Suspect retrograde arch malperfusion To cath lab for intervention –Planned stent across isthmus Delay in Diagnosis

4. Cath lab Hypotensive: –Open chest, ECMO standby Attempted retrograde isthmus cannulation !!! No flow >> Right radial pressure: 20 (CPR) >>>ECMO (RA - MPA) Rt radial pressure: mid 30’s Diminished function Good judgment What now?

5. Restore arch perfusion Transfer arterial flow to right carotid Return of function! Rescued!

6. Stability Now what? –ECMO as bridge to transplant? –Norwood after recovery of function? Tough problem

7. Idea Advance arterial cannula to aortic arch Reverse BT shunt as ‘rescue’ treatment

8. Idea Wean off ECMO D/C from ICU POD 9 Success!

9. Success >> Late failure 3 months postop Balloon atrial septostomy >> ischemia Poor ventricular function with troponins (+) >> reverse BT shunt is rendered inadequate after septostomy Arch is malperfused Failure (again)!

10. Idea #2 Try ECMO (via left carotid) to perfuse arch Prompt return of function Dx: Hibernating myocardium Norwood (4.0 mm BT) Success!

11. Subsequent course –Small rt parietal stroke - seizures BCPS at age 10m –Osteoporosis leading to multiple fractures –Delayed milestones –Persistent ventricular dysfunction –Borderline high PVR –Resolved with medical therapy and time Fontan at 6 years

12. Technical Conclusions Augmentation of arch perfusion with ECMO can reverse myocardial hibernation Reverse BT shunt can be used as a ‘rescue’ 3.5 mm reverse BT shunt can keep you alive, but may not be adequate for long term

13. Big picture Better to avoid trouble Successful rescue can have a big price Success or Failure?

14. 25th July 2008 to 24th January 2015 ICU – 56 days Hospital LOS – 105 days Interventions – 9 Cost - $357,692 ER visits – 9 Admissions – 17 Total LOS – 222 Total Interventions – 15 Total Clinic Visits – 89

15. Bigger picture Rescues are important to preserve life ‘Success or Failure?’ is the wrong question ‘Quality of Rescue’ –Life –Morbidity –Cost

17. First Admission Length of Stay in Cardiac ICU – 49.3 days Length of Stay in Cardiac Unit – 55.7 days Resource Intensity Weight – 68.778 Total Length of Stay for the visit – 105 days Number of Surgeons and Physician involved in the care - 10 Total Cost for Visit - $357,652 Number of Interventions - >9

18. Owen is Going Home! Age - 6 years oldLength of Stay in Cardiac ICU – 2 days Admission Date – 12/01/2015Length of Stay in Cardiac Unit – 10.2 days Discharge Date – 24/01/2015Number of Surgeons and Physician involved in the care - 5 Total Length of Stay for the visit – 12.2 days Number of Interventions - 3

19. Owen’s SickKids Journey 25 th July 2008 to 24 th January 2015