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
1/200 of us is born with
congenital heart disease…
A child is born in the US every
7.6 seconds…
1/10 extended families…
1 million ACHD survivors
A child with CHD is born in the US every
26 minutes…
1789
Liberty Leading the People
Eugene Delacroix 1830 (L’Hopital des Enfants-Malades 1802 )
These hearts are unique
“Something the Lord Made”
Alfred Blalock, Helen Taussig, and Vivien Thomas: 29 Nov 1944
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
“One Million Strong”
US: 30-170 ACHD Centers to Fulfill Medical Needs
and Care for ACHD Survivors
Atresia
d-TGA
ACHD Population
complex ••• Mitral
CCTGA
• 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
15%
47%
38%
simple
moderate
Red font indicates some association with higher risk for development of PAH
•
•
•
•
•
•
•
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
ACHD, adult congenital heart disease; ASD, atrial septal defect; PDA, patent ductus arteriosus; VPS, vascular positioning system; d-TGA, dextrotransposition 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”
“One Million Frail”
The ACHD Phenotype
1–
–
0.9 –
SCD-Free
Survival
(proportion)
TOF
COA
–
d-TGA
0.8 –
AS
n = 3589
–
0.7 –
0
5
10
15
20
25
30
Postoperative Interval (years)
Silka et al. J Am Coll Cardiol . 1998; 32: 245-251.
35
ACHD “Medical Phenotype”
Billett J et al. Heart. 2008, 1194-1199.
BACH Original Cohort.
11
ACHD Cardiovascular “Phenotype” : MVO2
Mean ± SD
Aortic coarction
Tetralogy of Fallot
VSD
Mustard-operation
Valvular disease
Ebsteins anomaly
Pulmonary atresia
Fontan-operation
ASD (late closure)
ccTGA
Complex anatomy
Eisenmenger
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
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. Circulation 2005, 828-835.
12
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
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!
15
ACHD Patients in USA vs
Those in ACHD Clinics
787,000
Number
Of Patients
800,000 –
700,000 –
600,000 –
500,000 –
400,000 –
300,000 –
200,000 –
100,000 –
0–
38,000 in ACHD
Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707.
ACHA Clinic Directory Working Group 2007
16
Understanding Loss of CHD Follow-Up
650 –
n=643 (100%)
Attrition
177 (28%)
520 –
CHD Patients
n=643 (100%)
53 (8%)
Attrition
123 (19%)
390 –
260 –
Diagnosed
by cardiologist
643 (100%)
130 –
n=466 (72%)
51 (8%)
Seen by
cardiologist
413 (64%)
Seen by
cardiologist
292 (45%)
6-12
13-17
n=343 (53%)
Attrition
94 (15%)
Seen by
cardiologist
249 (39%)
0–
<6
18-22
Age Group
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).
Adapted from: Mackie A, et al. Circulation. 2009;120:302-309.
Finding the “Lost”
Percent of Outpatients > 18yrs
35
30
25
20
15
10
5
0
2000 2001
2002 2003 2004 2005
2006 2007
35.00
30.00
25.00
20.00
15.00
10.00
5.00
<1y
>18y
2005
2003
2001
% Echos by Age
1999
1997
1995
1993
1991
1989
1987
1985
1983
0.00
<1y
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 EducationResources
• 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 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.
Acknowledgments
Boston Adult Congenital Heart (BACH) Program