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WE should look after
Adult Congenital Heart Disease Patients
Peta Alexander
Staff Physician, Department of Cardiology
Medical Lead, Cardiac ECMO
Boston Children’s Hospital and Harvard Medical School
DISCLOSURES
Site PI for Novartis serelaxin study
Site PI for Tenax Therapeutics, Inc levosimendan expanded access study
Member of Event Adjudication Committee for Entresto
pediatric randomized control trial (Novartis)
2
3
Survival from birth to 18 years
1970-1974
1975-1979
1980-1984
P<0.0001
1985-1989
1990-1992
Age
1.00
0.98
0.96
0.94
0.92
0.90
0.88
0.86
0.84
0.82
0.80
0 42 6 8 10 12 14 16 18
RATIO OF ADULTS TO PAEDIATRIC SURVIVORS
1965 1985 2010
Pediatric patients
Adult patients
Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707; Marelli, AJ, et al. CirculationAHA. June 2014.
33% 66%
Loss of follow-up from age 6 to 22 years among the entire study cohort
Mackie A S et al. Circulation 2009;120:302-309
0
130
260
390
520
650
<6 6 to 12 13 to 17 18 to 22
Diagnosed
by
Cardiologist
643 (100 %)
Seen by
Cardiologist
413 (64%)
Seen by
Cardiologist
292 (49%) Seen by
Cardiologist
249 (39%)
Attrition
177 (28%)
Attrition
123 (19%)
Attrition
94 (19%)
53 (8%)
51 (8%)
N = 643
(100%)
N = 643
(100%)
N = 466
(72%)
N = 343
(53 %)
LOSSTOFOLLOWUP
LOSS TO FOLLOW UP
7
Mackie A S et al. Circulation 2009;120:302-309
0
10
20
30
40
50
60
70
80
90
100
0 2 4 6 8 10 12 14 16 18
Outpatient visits to cardiologist
Outpatient visit to primary care
Any encounter with health care system
Mylotte D et al. Circulation. 2014;129:1804-1812
100
80
60
40
20
0
20
18
16
14
12
10
8
6
4
2
0
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005
Year
Deaths(per1,000ACHDpopulationperyear)
PatientsreferredtoACHDcenters
(per1,000ACHDpopulationperyear)
Guideline
presentation
Guideline
publication
Change-pointvisits
Change-pointdeaths
Interaction
p<0.0001
Interaction
p=0.04
Referral to specialized ACHD centre and mortality
WHAT IS REQUIRED OF AN ACHD CENTRE?
1965 1985 2010
Pediatric patients
Adult patients
Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707; Marelli, AJ, et al. CirculationAHA. June 2014.
33% 66%
WHAT IS REQUIRED OF AN ACHD CENTRE?
EXPERTISE
What is required of an ACHD centre?
EXPERTISE with relevant VOLUME
0%
1%
2%
3%
4%
5%
6%
7%
8%
9%
10%
0
200
400
600
800
1000
1200
1400
%Mortality
TotalAdmissions
Total # Patients Percentage
CICU ADMISSIONS
1992-2015
What is required of an ACHD centre?
EXPERTISE with relevant VOLUME
CARDIAC SURGICAL
VOLUME
1998-2015
0
200
400
600
800
1000
1200
1400
1600
1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015
706 691 688 722 677
756
685 698 721
666
766
827 820 848 841 871 880 897
280 306 338
335 395 257 379 387
444
456
496
550
434 381 408
573 583 533
Pump Cases NonPump Cases
What is required of an ACHD centre?
EXPERTISE with relevant VOLUME
0
200
400
600
800
1000
1200
1400
1600
706 691 688 722 677 756 685 698 721 666
766 827 820 848 841 871 880 897
280 306 338 335 395 257 379 387
444
456
496
550
434 381 408
573 583 533
Pump Cases
0%
1%
2%
3%
4%
5%
6%
7%
8%
9%
10%
0
200
400
600
800
1000
1200
1400
Total # Patients Percentage
Neonate
15%
1-12 months
26%
1-5 years
27%
5-10 years
10%
10-18 years
13%
18+ years
9%
What is required of an ACHD centre?
SUBSPECIALTY COLLABORATIVE CARE
What is required of an ACHD centre?
ACADEMIC PRODUCTIVITY
What is required of an ACHD centre?
EDUCATION AND COLLABORATION
WhatisrequiredofACHDcentre?
RESOURCESFORTEAMTRAINING
What is required of an ACHD centre?
BUILDING for the FUTURE
BACH
BOSTON CHILDREN’S HOSPITAL
AND BRIGHAM AND WOMEN’S HOSPITAL
21
22

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Debate: Who should care for GUCH?

  • 1. WE should look after Adult Congenital Heart Disease Patients Peta Alexander Staff Physician, Department of Cardiology Medical Lead, Cardiac ECMO Boston Children’s Hospital and Harvard Medical School
  • 2. DISCLOSURES Site PI for Novartis serelaxin study Site PI for Tenax Therapeutics, Inc levosimendan expanded access study Member of Event Adjudication Committee for Entresto pediatric randomized control trial (Novartis) 2
  • 3. 3
  • 4. Survival from birth to 18 years 1970-1974 1975-1979 1980-1984 P<0.0001 1985-1989 1990-1992 Age 1.00 0.98 0.96 0.94 0.92 0.90 0.88 0.86 0.84 0.82 0.80 0 42 6 8 10 12 14 16 18
  • 5. RATIO OF ADULTS TO PAEDIATRIC SURVIVORS 1965 1985 2010 Pediatric patients Adult patients Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707; Marelli, AJ, et al. CirculationAHA. June 2014. 33% 66%
  • 6. Loss of follow-up from age 6 to 22 years among the entire study cohort Mackie A S et al. Circulation 2009;120:302-309 0 130 260 390 520 650 <6 6 to 12 13 to 17 18 to 22 Diagnosed by Cardiologist 643 (100 %) Seen by Cardiologist 413 (64%) Seen by Cardiologist 292 (49%) Seen by Cardiologist 249 (39%) Attrition 177 (28%) Attrition 123 (19%) Attrition 94 (19%) 53 (8%) 51 (8%) N = 643 (100%) N = 643 (100%) N = 466 (72%) N = 343 (53 %) LOSSTOFOLLOWUP
  • 7. LOSS TO FOLLOW UP 7 Mackie A S et al. Circulation 2009;120:302-309 0 10 20 30 40 50 60 70 80 90 100 0 2 4 6 8 10 12 14 16 18 Outpatient visits to cardiologist Outpatient visit to primary care Any encounter with health care system
  • 8. Mylotte D et al. Circulation. 2014;129:1804-1812 100 80 60 40 20 0 20 18 16 14 12 10 8 6 4 2 0 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 Year Deaths(per1,000ACHDpopulationperyear) PatientsreferredtoACHDcenters (per1,000ACHDpopulationperyear) Guideline presentation Guideline publication Change-pointvisits Change-pointdeaths Interaction p<0.0001 Interaction p=0.04 Referral to specialized ACHD centre and mortality
  • 9. WHAT IS REQUIRED OF AN ACHD CENTRE? 1965 1985 2010 Pediatric patients Adult patients Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707; Marelli, AJ, et al. CirculationAHA. June 2014. 33% 66%
  • 10. WHAT IS REQUIRED OF AN ACHD CENTRE? EXPERTISE
  • 11. What is required of an ACHD centre? EXPERTISE with relevant VOLUME 0% 1% 2% 3% 4% 5% 6% 7% 8% 9% 10% 0 200 400 600 800 1000 1200 1400 %Mortality TotalAdmissions Total # Patients Percentage CICU ADMISSIONS 1992-2015
  • 12. What is required of an ACHD centre? EXPERTISE with relevant VOLUME CARDIAC SURGICAL VOLUME 1998-2015 0 200 400 600 800 1000 1200 1400 1600 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 706 691 688 722 677 756 685 698 721 666 766 827 820 848 841 871 880 897 280 306 338 335 395 257 379 387 444 456 496 550 434 381 408 573 583 533 Pump Cases NonPump Cases
  • 13. What is required of an ACHD centre? EXPERTISE with relevant VOLUME 0 200 400 600 800 1000 1200 1400 1600 706 691 688 722 677 756 685 698 721 666 766 827 820 848 841 871 880 897 280 306 338 335 395 257 379 387 444 456 496 550 434 381 408 573 583 533 Pump Cases 0% 1% 2% 3% 4% 5% 6% 7% 8% 9% 10% 0 200 400 600 800 1000 1200 1400 Total # Patients Percentage Neonate 15% 1-12 months 26% 1-5 years 27% 5-10 years 10% 10-18 years 13% 18+ years 9%
  • 14. What is required of an ACHD centre? SUBSPECIALTY COLLABORATIVE CARE
  • 15. What is required of an ACHD centre? ACADEMIC PRODUCTIVITY
  • 16.
  • 17. What is required of an ACHD centre? EDUCATION AND COLLABORATION
  • 19. What is required of an ACHD centre? BUILDING for the FUTURE
  • 20. BACH BOSTON CHILDREN’S HOSPITAL AND BRIGHAM AND WOMEN’S HOSPITAL
  • 21. 21
  • 22. 22