Congenital Heart Disease:
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
• Discuss how improved treatment has ballooned the
number of adults living with these conditions
• Review barriers to effective treatment of adult
congenital heart disease patients
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 heart
What is congenital heart disease?
aka “CHD”
“Tricuspid atresia” is an example
of a heart with only ½ of the
needed structures
Blue blood has
to go to the
other side of
the heart
Severely underdeveloped right side of the heart:
no heart valve between upper and lower
chambers
Left (only)
pumping
chamber
The Journey to Survival
• Prior 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?
Collaboration:
cardiologists, surgeons, researchers
Johns Hopkins Hospital circa 1940
Dr. Alfred Blalock, surgeon
Dr. Helen Taussig,
pediatric cardiologist
Vivian Thomas,
lab technician
extraordinaire
Johns Hopkins 1944:
“Something the Lord Made”: The Blalock-Taussig Shunt
1st “blue baby”
surgery
Goal: provide
reliable blood
flow to the lungs
•Partners of the Heart: Vivien Thomas and His Work
with Alfred Blalock, by Vivien T. Thomas, University of
Pennsylvania Press, 1985. ISBN 0-8122-1634-2
The modern era:
increasing survival for patients with CHD
• 1952: Heart-lung bypass machine 1st 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 on
Dr. Mustard in the Parlor
1st “Mustard” patient at her 16th birthday party
Arterial Switch Operation: 1980s-present
Simple! move hair thin structures in a heart the size of a walnut
<½ inch!
Detach/Re-implant
coronary arteries)
PA
AO
Fast-forward 70 years:
Continued improved survival in CHD due to:
Expanding population of adults with CHD
Improved re-operation,
treatment for arrhythmias
Lower perioperative
mortality
Improved surgical
techniques
Fetal diagnosis
Congenital Heart Disease
Early complete repair
PGE1, Advances
in NICU care
Great problem to have:
Survival into Adulthood with CHD
~90%
1980s
1970s
1960s
1940s
0%
20%
40%
60%
80%
100%
The changing face
of Congenital Heart Disease
2010
Adults
Children
1985
1965
0%
20%
40%
60%
80%
100%
• More adults now
than children with
CHD
• >1,000,000 adult
CHD pts in US
• Increasing by 5%
per year
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 adulthood
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 adulthood
Barriers to effective care for adult CHD
patients
• Lack of enough dedicated adult 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
Improved insurability
• Historically, patients had limited health
options:
insurance
– Work for a large company
– Go 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
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
patients
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 18
– Successful transfer associated with
• More heart surgeries
• Older age at last follow-up in pediatric center
• Documentation of advice to follow-up in ACHD center
Attending clinic without parents ** strongest predictor
hands-down**
Reid, et al. Pediatrics 2004
32nd ACC/AHA Bethesda Conference
• Convened in 2000 by American College of Cardiology to
study the needs of adult CHD patients
• Major Recommendations:
– Increase education of adult CHD providers
– Need 30-50 US regional ACHD care centers
– Adults with moderate or complex CHD to receive regular care at
these centers
– Physicians without specific expertise in CHD should see patients in
collaboration with adult CHD center
– Major interventions should be performed in regional centers
Problems with
nd
32
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 action
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 projects
Conclusions
• Successful care of congenital heart disease has resulted
in steady increase in number of adults living with wellpalliated disease
• Most adult CHD patients can live long and productive lives
• Goals:
– build on the work done by the pioneers like Taussig and Blalock
– improve health care delivery to these patients
• Better data collection and outcomes research needed