BACH and Transitioning: Preparing Adolescents with CHD for Self-Care in
Adulthood
Susan M. Fernandes, MPH / Michael J. Landzberg, MDBoston Adult Congenital Heart Service
Children’s Hospital and BWH, Boston, MA
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
Liberty Leading the People Eugene Delacroix 1830 (L’Hopital des Enfants-Malades 1802 )
1789
These hearts are unique
Alfred Blalock, Helen Taussig, and Vivien Thomas: 29 Nov 1944
“Something the Lord Made”
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 AdulthoodSurviving to / through Adulthood
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 operations50%: 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%
moderatesimple
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”
0
1 –
–
0.9 –
–
0.8 –
–
0.7 –
SCD-FreeSurvival
(proportion)
Postoperative Interval (years)Silka et al. J Am Coll Cardiol . 1998; 32: 245-251.
5 10 15 20 25 30 35
TOF
d-TGA
COA
AS
n = 3589
“One Million Strong”“One Million Frail”
The ACHD Phenotype
Billett J et al. Heart. 2008, 1194-1199. BACH Original Cohort. 11
ACHD “Medical Phenotype”
405 10 15 20 25 30 35
Aortic coarctionTetralogy of Fallot
VSDMustard-operation
Valvular diseaseEbsteins anomalyPulmonary atresiaFontan-operation
ASD (late closure)ccTGA
Complex anatomyEisenmenger ANOVA P<0.0001
Mean ± SD
28.7 ± 10.425.5 ± 9.123.4 ± 8.923.3 ± 7.422.7 ± 7.620.8 ± 4.220.1 ± 6.519.8 ± 5.819.2 ± 6.218.6 ± 6.914.6 ± 4.711.5 ± 3.6
Peak VO2 (ml s/b mL)
ACHD Cardiovascular “Phenotype” : MVO2
Diller GP, et al. Circulation 2005, 828-835.
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
12
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
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
NumberOf 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
650 –
520 –
390 –
260 –
130 –
0 –
< 6
Age Group
CH
D P
atie
nts
6-12 13-17 18-22
Diagnosedby cardiologist
643 (100%)
n=643 (100%) n=643 (100%)
Seen bycardiologist413 (64%)
Seen bycardiologist292 (45%)
Seen bycardiologist249 (39%)
n=466 (72%)
n=343 (53%)
Attrition177 (28%)
53 (8%) Attrition123 (19%)
51 (8%) Attrition94 (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.
Finding the “Lost”
Percent of Outpatients > 18yrs
0
5
10
15
20
25
30
35
2000 2001 2002 2003 2004 2005 2006 2007
1983
1985
1987
1989
1991
1993
1995
1997
1999
2001
2003
2005
<1y
0.00
5.00
10.00
15.00
20.00
25.00
30.00
35.00
<1y >18y
% Echos by Age
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.
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
Table 1. Clinician Characteristics
(Fernandes et al. 2011 Int Journal of Adol and Child Health)
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)
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
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
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
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.
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
Key Findings① 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.
AcknowledgmentsAcknowledgmentsBoston Adult Congenital Heart (BACH) ProgramBoston Adult Congenital Heart (BACH) Program