Not Just for Kids Anymore Jane E Crosson MD Associate Professor Pediatric Cardiology Director Adult Congenital Heart Disease program Johns Hopkins Hospital Goals of Talk Define congenital heart disease ID: 139787
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Congenital Heart Disease: Not Just for Kids Anymore
Jane E. Crosson, MD
Associate Professor, Pediatric Cardiology
Director, Adult Congenital Heart Disease program
Johns Hopkins HospitalSlide2
Goals of TalkDefine congenital heart diseaseDiscuss how improved treatment has ballooned the number of adults living with these conditions
Review barriers to effective treatment of adult congenital heart disease patientsSlide3
What is congenital heart disease?
aka “CHD”
Defects in the heart structure that occur during fetal heart development
Almost 1% live births (most common birth defect
)
Range from simple ‘holes’ in the heart, or narrowed heart valves to hearts with only ½ of the needed structures
Normal heartSlide4
What is congenital h
eart disease?
aka “CHD”
“Tricuspid atresia” is an example of a heart with only ½ of the needed structures
Severely underdeveloped right side of the heart:
no heart valve between upper and lower chambers
Left (only) pumping chamber
Blue blood has to go to the other side of the heartSlide5
The Journey to SurvivalPrior to the 1940s, children with severe CHD had an >80% chance of dying before they reached adulthood
Now they have a 90% of
surviving
well into adulthood
How did we get there?Slide6
Collaboration:
cardiologists, surgeons, researchers
Dr. Helen
Taussig
, pediatric cardiologist
Dr. Alfred Blalock, surgeon
Vivian Thomas, lab technician extraordinaire
Johns Hopkins Hospital circa 1940Slide7
Johns Hopkins 1944:
“
Something the Lord Made
”:
The Blalock-
Taussig
Shunt1st “blue baby” surgery
Goal: provide reliable blood
flow to the lungs
Blalock
Partners of the Heart: Vivien Thomas and His Work with Alfred Blalock, by Vivien T. Thomas, University of Pennsylvania Press, 1985. ISBN 0-8122-1634-2Slide8
The modern era: increasing survival for patients with CHD
1952:
Heart-lung bypass machine 1
st
used successfully for
CHD repair
1963: Mustard: First successful operation for babies with fatal CHD condition called “transposition of the great arteries”1971: First operation to separate the blue and the red blood in patients with only one pumping chamber like tricuspid atresia (“Fontan” procedure)1981: First surgery for “HLHS”, previously uniformly fatal…..And on and onSlide9
Dr. Mustard in the Parlor1
st
“Mustard”
patient at her 16
th birthday partySlide10
Arterial Switch Operation: 1980s-present
Simple! move hair thin structures in a heart the size of a walnut
AO
PA
Detach/Re-implant
coronary arteries
)
<½ inch!Slide11
Fast-forward 70 years:Continued improved survival in CHD due to:
Congenital Heart Disease
Expanding population of adults with CHD
Fetal diagnosis
PGE1, Advances in NICU care
Improved surgical techniques
Early complete repair
Lower perioperative mortality
Improved re-operation, treatment for arrhythmiasSlide12
Great problem to have:Survival into Adulthood with CHD
~90%Slide13
The changing face of Congenital Heart Disease
More adults now than children with CHD
>1,000,000 adult CHD
pts
in US
Increasing by 5% per yearSlide14
Meeting the needs of adults with congenital heart disease
Most patients with CHD are not ‘cured’ of their disease
They get sick more often and have a higher risk of death than general population
Majority are self-supporting, but others have physical and cognitive limitations
Life-long follow-up is needed,
but
30-50% of patients are lost to follow-up in adulthoodSlide15
Where do we go from here….
Now that all of the children are growing up?
Need well-trained specialists to take care of all these adults with very specialized problems
However
, adult cardiologists traditionally
have
not been trained to care for these patients, since they didn’t live long enough to reach adulthoodSlide16
Barriers to effective care for adult CHD patients
Lack of enough dedicated
a
dult CHD facilities
Fragmented care among primary care doctors, adult cardiologists, & pediatric cardiologists
Paucity of adult cardiologists with CHD experience
Lack of coordinated management of contraceptive and pregnancy needs Slide17
Historically, patients had limited health insurance options:Work for a large companyGo on disability (not always qualifying)Changes in SSI regulations have expedited disability processing for the most severely affected (2012)
ACA has enabled most others to obtain health insurance without the burden of paying for their pre-existing condition
Congenital Heart Futures
Act
authorized NIH funding for CHD research & created National
Congenital Heart Surveillance
System Improved insurabilitySlide18
Canadian Model for Adult CHD Care
15 Regional Adult CHD Centers
The Good:
Well-coordinated, top-of-the-line care
Database support for outcomes research
The Bad:
Big country, lot of traveling to get to these regional centers for many patientsSlide19
How well does the Canadian model work?
Survey of 360 young adult CHD pts, ages 19-22 in Canada
Less than ½
successfully transferred
care
to specialized adult CHD clinics
27% had no cardiac follow-up since turning 18Successful transfer associated withMore heart surgeriesOlder age at last follow-up in pediatric centerDocumentation of advice to follow-up in ACHD center
Attending clinic without parents ** strongest predictor hands-down**
Reid, et al. Pediatrics 2004Slide20
32nd ACC/AHA Bethesda Conference
Convened in 2000 by American College of Cardiology to study the needs of a
dult CHD
patients
Major Recommendations:
Increase education of adult CHD providersNeed 30-50 US regional ACHD care centersAdults with moderate or complex CHD to receive regular care at these centersPhysicians without specific expertise in CHD should see patients in collaboration with adult CHD centerMajor interventions
should be performed in regional centers Slide21
Problems with 32nd Bethesda
Conference
Patients unwilling/unable to travel to regional centers
Not enough physicians
trained
New board certification now available may actually
inhibit increase in personpower (another $2800 plus study time, etc.) Pediatric and adult cardiologists unwilling to “give up” patients: emotional, economic factors, etc.Most recommendations have not translated into actionSlide22
Different models evolving
Programs based in children’s hospitals with adult hospital affiliations, or vice versa
Johns Hopkins: Pediatric cardiologists consult on management of inpatients with CHD, run outpatient service
Baltimore-Washington area
Separate programs in major academic centers
Most link pediatric and adult cardiologists; surgical colleagues cover pediatric and adult operating suites
Regional consortium that meets quarterly to discuss cases, exchange ideas, work on research projectsSlide23
Conclusions
Successful care of congenital heart disease has resulted in steady increase in number of adults living with well-palliated disease
Most adult CHD patients can live long and productive lives
Goals:
build on the work done by the pioneers like
Taussig
and Blalockimprove health care delivery to these patientsBetter data collection and outcomes research needed