1. BACH and Transitioning: Preparing Adolescents with CHD for Self-Care in Adulthood Susan M. Fernandes, MPH / Michael J. Landzberg, MD Boston Adult Congenital Heart Service Children’s Hospital and BWH, Boston, MA

2. A child is born in the US every 7.6 seconds… A child with CHD is born in the US every 26 minutes… 1/200 of us is born with congenital heart disease… 1/10 extended families… 1 million ACHD survivors

3. Liberty Leading the People Eugene Delacroix 1830 (L’Hopital des Enfants-Malades 1802 ) 1789

4. These hearts are unique

5. Alfred Blalock, Helen Taussig, and Vivien Thomas: 29 Nov 1944 “Something the Lord Made”

6. CHD – Paradigm Shift 40,000 infants born with CHD/ year What is successful outcome? –Surviving initial surgical repair –Surviving to 1 year of age –Normal childhood –Normal adolescence Surviving to / through Adulthood

10. US: 30-170 ACHD Centers to Fulfill Medical Needs and Care for ACHD Survivors complex Mitral Atresia d-TGA CCTGA DORV Heterotaxy Single V Conduits Truncus Cyanotic Eisenmenger TOF SV Defects APV Drainage AVC Primum ASD Sub PS AoCo Ebstein VPS PR Complex PDA or VSD Size makes a difference (ASD > 2 cm, VSD greater than 1 cm, PDA > 0.6-0.8 cm) Simple ASD Simple Aortic Disease Simple Mitral Disease Simple PDA Mild VPS 60%: prior operations 50%: will have reops 3:1 interventions are CATH CHF, PAH, Arrhythmia ACHD Population Red font indicates some association with higher risk for development of PAH 15% 47% 38% moderate simple ACHD, adult congenital heart disease; ASD, atrial septal defect; PDA, patent ductus arteriosus; VPS, vascular positioning system; d-TGA, dextro- transposition of the great arteries; CCTGA, congenitally corrected transposition of the great arteries; DORV, double outlet right ventricle; TOF, tetralogy of Fallot; SV, stroke volume; APV, absent pulmonary valve; AVC, atrioventricular canal; PS, pulmonary stenosis; AoCo, aortic coarctation; PR, pulmonary valve regurgitation; VSD, ventricular septal defect; CATH, catheterizations. Marelli A et al. Am Heart J. 2009;157:1-8. Warnes C et al. J Am Coll Cardiol.. 2001;37:1170-1175. “One Million Strong”

11. 0 1 – – 0.9 – – 0.8 – – 0.7 – SCD-Free Survival (proportion) Postoperative Interval (years) Silka et al. J Am Coll Cardiol. 1998; 32: 245-251. 5 101520253035 TOF d-TGA COA AS n = 3589 “One Million Strong” “One Million Frail” The ACHD Phenotype

12. Billett J et al. Heart. 2008, 1194-1199. BACH Original Cohort. 11 ACHD “Medical Phenotype”

13. 405101520253035 Aortic coarction Tetralogy of Fallot VSD Mustard-operation Valvular disease Ebsteins anomaly Pulmonary atresia Fontan-operation ASD (late closure) ccTGA Complex anatomy Eisenmenger ANOVA P<0.0001 Mean ± SD 28.7 ± 10.4 25.5 ± 9.1 23.4 ± 8.9 23.3 ± 7.4 22.7 ± 7.6 20.8 ± 4.2 20.1 ± 6.5 19.8 ± 5.8 19.2 ± 6.2 18.6 ± 6.9 14.6 ± 4.7 11.5 ± 3.6 Peak VO 2 (ml s/b mL) ACHD Cardiovascular “Phenotype” : MVO 2 Diller GP, et al. Circulation 2005, 828-835. MVO 2 indicates Myocardial Oxygen Consumption; CCTGA, Congenitally Corrected Transposition of the Great Arteries; SD, Septal Defect; ANOVA, Analysis of the Variance; VO 2, Volume of Oxygen 12

14. Bouchardy J, et al. Circulation 2009 20 year risk of AA: 15% (> 3x higher) AA in ACHD: > 50%  mortal risk Atrial Arrhythmias (AA) in ACHD ACHD Arrhythmic “Phenotype”

15. 15 Who is providing the care? Many being seen by Pediatric Cardiologists NOT trained in ACHD Many being seen by Adult Cardiologists NOT trained in ACHD Few being seen in ACHD clinics (<5%) In Reality: We Do Not Know!

16. 16 ACHD Patients in USA vs Those in ACHD Clinics Number Of Patients 800,000 – - 700,000 – - 600,000 – - 500,000 – - 400,000 – - 300,000 – - 200,000 – - 100,000 – - 0 – 787,000 Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707. ACHA Clinic Directory Working Group 2007 38,000 in ACHD

17. 650 – 520 – 390 – 260 – 130 – 0 – < 6 Age Group CHD Patients 6-1213-1718-22 Diagnosed by cardiologist 643 (100%) n=643 (100%) Seen by cardiologist 413 (64%) Seen by cardiologist 292 (45%) Seen by cardiologist 249 (39%) n=466 (72%) n=343 (53%) Attrition 177 (28%) 53 (8%) Attrition 123 (19%) 51 (8%) Attrition 94 (15%) The blue bars indicate patients who were not seen by a cardiologist within the indicated age range but were seen again by a cardiologist in an older age group (ie, transiently lost to follow-up). Understanding Loss of CHD Follow-Up Adapted from: Mackie A, et al. Circulation. 2009;120:302-309.

18. Finding the “Lost”

23. CHB Transitioning Working Group 30 clinicians throughout CHB representing all outpatient clinics Numerous focus groups to identify key transitioning education areas that are common to a wide spectrum of pediatric diseases Established Goals –1). Assess the current state of preparing patients at CHB for self-care in adulthood.

24. Current Practices for the Transition and Transfer of Patients with a Wide Spectrum of Pediatric-Onset Chronic Diseases (Fernandes et al. 2011 Int Journal of Adol and Child Health) Single Center- CHB Random sample of 479 outpatient clinicians Overall response rate: 76.8% (368/479), 329/368 meet inclusion criteria –143 Physicians –157 Nurses/nurse practitioners –75 Social workers –26 Physician assistants

25. Table 1. Clinician Characteristics (Fernandes et al. 2011 Int Journal of Adol and Child Health)

26. Transitioning Education 73% of clinicians stated their patients receive transitioning education –71% Physicians –75% Nurses/Nurse Practitioners –80% Physician Assistants –60% Social Workers 92% provide transitioning education informally 61% begin transitioning education before age 16 years (Fernandes et al. 2011 Int Journal of Adol and Child Health)

27. Perceptions of Transitioning and Transfer: Results of a Survey of CHB Cardiology Clinicians 31 Clinicians (16.9±9.7 years in practice) –16 MD –15 Nurses, NP’s, PA’s All cardiology clinicians stated that their patients are provided with this education/assessment –74.2% informally –22.6% checklist –13.0% unknown

28. Patient and Parent Perceptions of Transitioning and Transfer @ CHB Recruitment of 16-25 year old patients with pediatric onset disease likely to require life long medical care and their parents 166 Patients 104 Parents –93 Matched Patient/Parent

32. Patient and Parent Perceptions of Transitioning and Transfer in Cardiology @ CHB 30 Patients CHB Cardiology –Mean age 19.5±3.0 years –14 Complex – 6 Moderate – 1 simple – 2 HCM –3 CVG clinic –4 cardiac transplant

36. Transitioning Education- Resources 90% of clinician’s support resources for the development of transitioning education programs 73% of parents identified the need for such programs.

37. Parental Knowledge of LLCCC Multi-center study of 500 Parents –S/p arterial switch operation (n=92) –S/p tetralogy of Fallot repair (n=134) –S/p Fontan procedure (n=140) –S/p aortic coarctation repair (n=126) 9 U.S. Centers Pediatrics, 2011 Nov 28 (Epub) –118 from CHB

40. Key Findings ① The overwhelming majority of clinicians believe they are providing their patients with the necessary skills to become independent adults capable of self care, although informally. ① Patients and parents perceive such as severely lacking. ① Clinicians, patients and parents overwhelmingly support the need for resources to improve the delivery of this type of education.

41. Acknowledgments Boston Adult Congenital Heart (BACH) Program