1. Education War Era Cardiac Surgery Health-Related Quality of Life (HRQOL) in Adult Survivors after the Fontan Operations James K. Kirklin, MD Manisha Kukreja, MBBS, MPH David C. Cleveland, MD. Robert J Dabal, MD. Ayesha S. Bryant, MSPH, MD

2. Education War Era Cardiac Surgery Presenter Disclosure James Kirklin James K. Kirklin, MD – None Manisha Kukreja, MBBS, MPH – None David C. Cleveland, MD., Robert J Dabal, MD., Ayesha S. Bryant, MSPH, MD – None

3. Education War Era Cardiac Surgery Ascertain the long-term HRQOL of adult patients who underwent a childhood Fontan operation for functional univentricular cardiac anomalies HRQOL After Fontan Primary Objective

4. Education War Era Cardiac Surgery Secondary Objectives HRQOL After Fontan Compare long-term QOL of Fontan group to that of heart transplant recipients and to a healthy general population

5. Hazard Functions Percent survival Years% survivalN at risk 195%190 591%116 1088%64 2077%14 Years after Fontan operation Fontan operation (UAB); 1988 – 2011 using internal or external PTFE tubes (N=207) Hazard (Deaths/Year) Early + Constant phase model Hazard=1.3%/year

6. Hazard Functions Percent survival Years% survivalN at risk 195%190 591%116 1088%64 2077%14 Years after Fontan operation Fontan operation (UAB); 1988 – 2011 using internal or external PTFE tubes (N=207) Hazard (Deaths/Year) Early + Constant phase model Hazard=1.3%/year

7. Education War Era Cardiac Surgery HRQOL After Fontan Study Population Fontan Inclusion: 49 Surviving adult patients (82% response rate) 19 years and older, who underwent a pediatric Fontan operation at UAB between 1988 – 2012, and who responded to the QOL survey Heart Transplant Inclusion: 13 Surviving adult heart transplant recipients (93% response rate) 19 years and older, transplanted at pediatric age, no H/O previous Fontan, who responded to the QOL survey.

8. Education War Era Cardiac Surgery 36 questions measuring 8 domains of health : PHYSICAL COMPONENT - Physical functioning - Role limitations due to physical health - Bodily pain - General health MENTAL HEALTH COMPONENT - Vitaliy - Social functioning - Role limitations by emotional h ealth - Mental health HRQOL Fontan Study SF – 36 Health Survey

9. Education War Era Cardiac Surgery The HRQOL scores of the Fontan patients were compared to H Tx patients scores and normalized against the general US population. For each domain, scores ranged from 0 to 100, a higher score indicating a better health state. National normalized mean value is set at 50 ( validated by Quality Metric Inc, Lincoln, RI) with SD of 10 HRQOL Fontan Study SF – 36 Health Survey

10. Education War Era Cardiac Surgery HRQOL After Fontan Methods Study Design: A cross-sectional survey design using the Standard Short Form 36 (SF-36), self administered by patients Survey completion date 7/15/2014 Average duration of follow up for Fontan group was 18 years ( range 3-24), with 90% >10 yrs Average duration of f/u for C Tx group was 13 yrs (range 3-23 yrs).

11. Overall mental component standardized scores by different age groups Age Gr 1 8-24 25-34 35-44 45-54 55-64 65-74 75+ Fontan 49.94 49 56.28 0 50.98 0 0 US pop 49 48.48 48.28 49.2 51.36 53.71 53.49 # in Sample 26 19 3 0 1 0 0

12. Overall mental component standardized scores by different age groups Age Gr 1 8-24 25-34 35-44 45-54 55-64 65-74 75+ Fontan 49.94 49 56.28 0 50.98 0 0 US pop 49 48.48 48.28 49.2 51.36 53.71 53.49 # in Sample 26 19 3 0 1 0 0

13. Overall mental component standardized scores by different age groups Age Gr 1 8-24 25-34 35-44 45-54 55-64 65-74 75+ Fontan 49.94 49 56.28 0 50.98 0 0 US pop 49 48.48 48.28 49.2 51.36 53.71 53.49 # in Sample 26 19 3 0 1 0 0

14. Mean Standardized HRQOL scores by cohort and of the general population (age controlled to 25 to 34) years using the SF-36v2 survey

17. Mean Standardized HRQOL scores by cohort and of the general population (age controlled to 25 to 34 years) using the SF-36v2 survey

18. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized physical component scores (PCS) (age 25-34 yrs) Percent ≤ Stated Overall Physical Component Scores Median, (range): 52 (28-70) 55.4351.3 US median 75 th percentile 50 th percentile 25 th percentile

19. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized physical component scores (PCS) (age 25-34 yrs) Percent ≤ Stated Overall Physical Component Scores Median, (range): 52 (28-70) 55.4351.3 US median 75 th percentile 50 th percentile 25 th percentile

20. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized physical component scores (PCS) (age 25-34 yrs) Percent ≤ Stated Overall Physical Component Scores Median, (range): 52 (28-70) 55.4351.3 US median 75 th percentile 50 th percentile 25 th percentile

21. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized physical component scores (PCS) (age 25-34 yrs) Percent ≤ Stated Overall Physical Component Scores Median, (range): 52 (28-70) 55.4351.3 US median 75 th percentile 50 th percentile 25 th percentile

22. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized mental component scores (MCS) (age 25-34 yrs) Percent ≤ Stated Overall Mental Component Scores Median, (range): 55 (18-66) 25 th percentile 50 th percentile 75 th percentile US median 51.97 45.17

23. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized mental component scores (MCS) (age 25-34 yrs) Percent ≤ Stated Overall Mental Component Scores Median, (range): 55 (18-66) 25 th percentile 50 th percentile 75 th percentile US median 51.97 45.17

24. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized mental component scores (MCS) (age 25-34 yrs) Percent ≤ Stated Overall Mental Component Scores Median, (range): 55 (18-66) 25 th percentile 50 th percentile 75 th percentile US median 51.97 45.17

25. QOL after Fontan Operations; UAB 1988-2011; Group=Fontan; N=49 Overall standardized mental component scores (MCS) (age 25-34 yrs) Percent ≤ Stated Overall Mental Component Scores Median, (range): 55 (18-66) 25 th percentile 50 th percentile 75 th percentile US median 51.97 45.17

26. Education War Era Cardiac Surgery Study Limitations This was a cross sectional study which depended on patient self-reporting and therefore could not explore the temporal trends in health perception. We cannot exclude the possibility that non- responders may have a different perception of their HRQOL.

27. Education War Era Cardiac Surgery Inferences Despite the long-term imponderables of the Fontan physiology, the majority of patients surviving an average of nearly 2 decades enjoy a good health-related QOL. Although their perception of the physical components of health lags behind an age- controlled normal population, it’s encouraging that their mental health and emotional scores were equal to those of the normal population.

28. Education War Era Cardiac Surgery Inferences When comparing surgical strategies (such as complex 2-ventricle repairs, Fontan procedure, transplantation, etc.) for complex conditions such as univentricular hearts and variants, HRQOL will increasingly become an important component of the overall calculus of “outcome” in the late phase and an ingredient of truly informed patient consent.