a child is born in the us every 7.6 seconds…

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