1. Tetralogy of Fallot Neurodevelopmental Outcomes October 25, 2013 Tetralogy of Fallot “Spelling It Out” Gwen Alton RN, MN Complex Pediatric Therapies Follow-Up Program

2. 2 TOF population Complex Pediatric Therapies Follow-Up Program –1999-2008 registered the children with full repairs at < 6 weeks –2008- register children with shunts prior to full repair up to 6 months of age. –6 sites across western Canada

3. 2000-2013 n= 51 CPB (2000-2013) n=42(82%) Survival to 2y n=37(88%) no chrom Abn n=28 * Chrom Abn n=9 * Deaths to 2y n=5(12%) No Chrom Abn n=2 ** Chrom Abn n=3 shunt (2008-2013) n=9 (18%) Survival to 2y n= 7(78%) No Chrom Abn n=3 Chrom Abn n=4 Deaths to 2y n=2(22%) No Chrom Abn n=0 Chrom Abn n=2 Total survivors = 44 no chrom abn = 31 Chrom Abn =13 *ECMO

4. Acute Care Variables mean (sd); n(%) n= 51 VariablesCPB n= 42BT n=9 Age (d)39(36)31(28) Ventilator days19(38)9(8) Hospital days36(30)26(16) Sepsis2(5%)1(11%) Convulsions4(10%)0(0%) CPR anytime2(5%)1(11%) No significant difference between groups

5. Acute care variables mean(sd) n=51 VariablesCPB n= 42BT n=9 PO d1 inotrope score17(12)11(9) PO d1 lactate (highest)4(4)2(1) PO d2-5 inotrope score16(8)20(23) PO d2-5 lactate (highest)3(1)3(4) No significant difference between groups

6. Acute care variables mean(sd); n(%) n=51 Variables No Chr Abn n=33Chr Abn n=18 Age (d) at OR40(37)34(29) Ventilator days11(12)29(55) Hospital days30(22)43(36) Sepsis2(6%)1(6%) Convulsions1(3%)3(17%) CPR anytime2(6%)1(6%) CPB*30(91%)12(67%) * p=.052 – trend to significance

7. Acute care variables mean(sd); n=51 Variables No Chr Abn n=33Chr Abn n=18 CPB n=30114(49)139(59) X-clamp n=2956(26)64(23) PO d1 inotrope score16(11)16(13) PO d1 lactate (highest)4(4)3(2) PO d2-5 inotrope score16(9)19(16) PO d2-5 lactate (highest)2(2)3(3) No significance difference between groups

8. 8 Developmental Examination Complex Pediatric Therapies Follow-Up Program General health Vision Hearing Language – expressive Language comprehension Performance skills Gross motor Fine Motor Behavior Social interaction Intellect Environment Functional Behavior Quality of Life Nutrition Multisite assessments Multidisciplinary teams

9. 9 Over view Outcomes n=44 Survivors n= 44 4 year n=14 2 year n= 21 Not yet seen n=7 LOST n=2

10. 10 Disabilities + General Health– n=35 Motor: Cerebral Palsy n=2(6%) Sensory: Vision loss n=1 (with CP) Hearing loss n=1 (2.8%)(unilateral, not aided) G-tube at 2 year assessment n=9 (5 with Chrom Abn) At 4 year assessment n=3 (2 with Chrom Abn) Hospitalizations # 3-9 in first 2 years (non-cardiac)

11. 11 Growth at 2 years: n (%) n=31 AnthropometricsNo chrom n=23Chrom abn n=8 Length < 3%5(22%)2(25%) Weight < 3%5(22%)2(25%) HC below -2SD3(13%)3(37%)

12. 12 Outcomes at 2 and 4 years n=35 Outcome combined 4y + 2 y Mean(sd) No chrom abn n=25 Chrom abn n=10 Cognitive81.8(17.2)69.3(20) GAC (ABAS)78(17)68(17) No significant difference between groups No difference between CPB/BT group GAC-General Adaptive Composite; ABAS - Adaptive Behavioral Assessment System

13. 13 TOF – outcomes n=35 Outcome combined 4y + 2 y Mean(sd) No chrom abn/ Congenital n=18 Chrom abn/ Congenital * n=17 Cognitive84(17)72(19) GAC(ABAS)81(18)69(16) Quality of Life85(18)69(12) * Skeletal dysplasia, VACTERL, neuroblastoma, dysmorphic

14. 14 Cognitive outcome– n=35 %

15. 15 DEFINITION: Adaptive/Functional Level to which individuals meet standards of personal self-determination and social conscientiousness that is expected for their age, development and culture. Includes real life skills such as grooming, dressing, safety, cleaning, making friends, social skills Used in evaluation of children to determine strengths and weaknesses to help improve their success in school and life

16. 16 GAC – Functional outcome %

17. 17 COMPOSITE DOMAINS 10 SKILL AREAS Motor CONCEPTUAL Communication Functional Pre/Academics Self-Direction SOCIAL Leisure Social PRACTICAL Community Use Home Living Health and Safety Self-Care General Adaptive Composite Score (GAC) includes all 10 domains ABAS II Profile of Function

18. 18 Skill area differences at 2 yr, m(SD)

19. 19 Early Developmental Intervention 0-3 years - is family centered Target groups: 1.Established impairment – e.g. Down Syndrome, Autism Spectrum Disorder 2.Environmental risk – e.g. poverty 3.Biological risk – e.g. early or ongoing illness Availability: -parent requests - community -referral to tertiary level

20. 20 Early Developmental Intervention Benefit – influences child, parent, family and reduces future concerns Child Outcomes - social competence - emotional development - behavioral engagement - motivation Parent Outcomes - resources, advocacy skills

21. 21 What can parents do to provide early assistance to their child? –Read, sing, tell stories –Watch and listen to see how your child communicates –Encourage your child to explore –Use words to help your child understand their feelings –Give your child the chance to do things for themselves – encourage to keep trying Zero to Three – Early Learning

22. 22 Thank you All the families and children who have participated – Complex Pediatric Therapies Follow-Up Program Other sites across Western Canada NICU, PICU, surgical staff – Stollery Children’s Hospital 4C staff & therapists – Stollery Children’s Hospital Glenrose Rehabilitation Hospital – therapist and support staff Co-chairs – Dr. Charlene Robertson, Dr. Ari Joffe, Dr. Reg Sauvé

23. 23 QUESTIONS