A child is born in the US every 7.6 seconds…

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

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

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

4. These hearts are unique

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

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. Mitral Atresia • d-TGA • CCTGA • DORV • Heterotaxy • Single V • Conduits • Truncus • Cyanotic • Eisenmenger • 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 • TOF • SV Defects • APV Drainage • AVC • Primum ASD • Sub PS • AoCo • Ebstein • VPS • PR • Complex PDA or VSD 60%: prior operations 50%: will have reops 3:1 interventions are CATH CHF, PAH, Arrhythmia “One Million Strong” US: 30-170 ACHD Centers to Fulfill Medical Needs and Care for ACHD Survivors ACHD Population complex 15% 47% 38% moderate simple Red font indicates some association with higher risk for development of PAH 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.

11. “One Million Strong” “One Million Frail” The ACHD Phenotype 1 – – 0.9 – – 0.8 – – 0.7 – TOF COA SCD-Free Survival (proportion) d-TGA AS n = 3589 0 10 15 20 25 30 35 5 Postoperative Interval (years) Silka et al. J Am Coll Cardiol . 1998; 32: 245-251.

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

13. ACHD Cardiovascular “Phenotype” : MVO2 Mean ± SD Aortic coarction 28.7 ± 10.4 Tetralogy of Fallot 25.5 ± 9.1 VSD 23.4 ± 8.9 Mustard-operation 23.3 ± 7.4 Valvular disease 22.7 ± 7.6 Ebsteins anomaly 20.8 ± 4.2 Pulmonary atresia 20.1 ± 6.5 Fontan-operation 19.8 ± 5.8 ASD (late closure) 19.2 ± 6.2 ccTGA 18.6 ± 6.9 Complex anatomy 14.6 ± 4.7 Eisenmenger 11.5 ± 3.6 ANOVA P<0.0001 5 10 15 20 25 30 35 40 Peak VO2 (ml s/b mL) MVO2 indicates Myocardial Oxygen Consumption; CCTGA, Congenitally Corrected Transposition of the Great Arteries; SD, Septal Defect; ANOVA, Analysis of the Variance; VO2, Volume of Oxygen Diller GP, et al. Circulation2005, 828-835. 12

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

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

17. n=643 (100%) n=643 (100%) 650 – 520 – 390 – 260 – 130 – 0 – Attrition 177 (28%) n=466 (72%) 53 (8%) Attrition 123 (19%) n=343 (53%) Diagnosed by cardiologist 643 (100%) CHD Patients Attrition 94 (15%) 51 (8%) Seen by cardiologist 413 (64%) Seen by cardiologist 292 (45%) Seen by cardiologist 249 (39%) < 6 6-12 13-17 18-22 Age Group Understanding Loss of CHD Follow-Up Theblue 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). 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 • 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. • Patients and parents perceive such as severely lacking.  • 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