THE CARDIOVASCULAR SYSTEM
IN CHILDHOOD
EVALUATION AND TREATMENT
SSA Conference, September 24, 2008
Joel Brenner, MD
Director, Pediatric Cardiology
Helen B. Taussig Children’s Congenital Heart Center
March 20, 2016
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The Cardiovascular System in Childhood:
Disability Evaluation under Social
Security
• There may be some cynicism about
governmental (and NGO) processes when it
comes to health care decision making.
• The role of health care professionals,
administrators, patient advocates, and
patients is to come together to learn from
each other and make the system work for the
betterment of patient care.
Evaluation of the Cardiovascular
System in Childhood
• Definition of cardiovascular impairment:
– Any disorder that affects the proper
function of the heart or circulatory system,
whether congenital or acquired.
•
•
•
•
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Chronic CHF or ventricular dysfunction.
Pain due to myocardial ischemia
Syncope from any cardiac cause
Central cyanosis
4
The Cardiovascular System in Childhood:
Disability Evaluation Under Social
Security
Category of Impairments
Chronic heart failure
Recurrent arrhythmia
Congenital heart disease
Heart transplant
Rheumatic heart disease
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Evaluation of the Cardiovascular System in
Childhood: Congenital Heart Disease
• Definition of CHD: any abnormality of
the heart or major blood vessels present
at birth
– Abnormalities of septation: VSD, AV canal
– Cyanotic heart disease: TOF, TGA
– Obstruction to ventricular outflow: PS, AS
– Major abnormalities of ventricular
development: HRH, HLH
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Evaluation of the Cardiovascular
System in Childhood
• Symptoms and signs usually observed
over time—3 months
• Laboratory findings– appropriate,
medically acceptable imaging
• Response to prescribed therapy
• Functional limitation
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Evaluation of CHF & Cyanosis
• Symptoms
–
–
–
–
–
Tachypnea
Poor feeding
Poor weight gain
Cyanotic spells
Exercise intolerance
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• Signs
– Increased respiratory
rate (PVC)
– Hepatomegaly,
peripheral edema
(SVC)
– Failure to thrive
– Decreased O2 sat
– Elevated Hct
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Evaluation of the Cardiovascular
System in Childhood
Well repaired minor or
moderate CHD will
have no/minor
medical issues and
require regular but
infrequent
surveillance.
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Well palliated complex
CHD will have minor
to serious cardiac
issues requiring
regular, frequent
surveillance through
adult years.
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Evaluation of the Cardiovascular System in
Childhood: Imaging Tests
•
•
•
•
•
•
Chest X Ray
Echocardiography
Radionuclide angiography
Cardiac catheterization
Computerized tomography (CT)
Magnetic resonance imaging (MRI)
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Imaging Studies: Chest X Ray
• Cardiomegaly is
present if:
– CT ratio > 60% in an
infant or > 55% in a
toddler or child on a 6
foot PA chest XRay
– But 6 foot PA film
rarely done in infants
and toddlers.
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Imaging Studies: ECHO
• Cardiomegaly and heart dysfunction:
– LVDD or LVSD > 2 standard deviations above
mean for BSA
– LV mass > 2 SD
– SF(% of blood pumped with each beat) > 2 SD
below mean for BSA
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Imaging Studies: ECHO
• Cardiomegaly and
heart dysfunction:
– LVDD or LVSD > 2
standard deviations
above mean for BSA
– LV mass > 2 SD
– SF(% of blood
pumped with each
beat) > 2 SD below
mean for BSA
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Imaging Studies: ECHO
New Imaging Modalities that need
to be added to Evaluation Scheme
• MRI/cine
– Ideal for functional
evaluation,
especially with
complex CHD, or for
patients with poor
echo windows
– No XRay exposure
– Long acquisition time
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• CT angiography
– Better definition of
vascular anatomy
– Quick acquisition
time
– Substantial XRay
exposure
– Ubiquitous
availability
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New Imaging Modalities that need to be
added to the Evaluation Scheme
• MRI
– Must be used in
conjunction with on
going clinical
evaluation and care
– Requires 3D
reconstruction for
functional and
anatomic evaluation
– Requires specific
knowledge of cardiac
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anatomy
• CT
--Must be used in
conjunction with on
going clinical evaluation
and care
--Should be used
sparingly, given
radiation exposure
--Should be used in
centers with specific
expertise in complex
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CHD
Evaluation and Treatment of the Cardiovascular
System in Childhood: 50 years of progress
• 1958
• 2008
•
•
•
•
• HLH:Norwood/Fontan
palliation or transplant
• Critical AS: excellent
palliation with balloon
• Critical PS: excellent
relief with balloon cath
• VSD and AVC:most
often repaired
• TOF: repaired
• TGA: repaired17
HLH: uniformly fatal
Critical AS: often fatal
Critical PS:high risk op
VSD and AV canal:
palliation with
pulmonary artery band
• TOF: palliation with
Blalock-Taussig shunt
• TGA-uniformly fatal
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Diagnosis and Treatment of TGA: 1978
• Primary care physician
recognizes cyanosis,
confirmed by ABG
• Diagnostic cardiac cath
with balloon septostomy
• Pre-op cardiac cath
• Surgical repair: Mustard
procedure
• Post op cardiac cath
• Frequent Holter
monitoring for SVT/SSS
• Eventual RV failure
AO
RV
Diagnosis and Treatment of TGA:2008
• Primary care
physician
recognizes cyanosis
• Echo confirms
diagnosis of TGA
• Surgical repair:ASO
• Post op echo
AO
PA
RV
LV
MRI in Patient following Arterial
Switch Procedure for TGA
AO
AO
PA
PA
RV
RV
Advances in Cardiac Imaging
Myocardial enhancement
Abnormal coronary origin
Evaluation of the Cardiovascular System in
Childhood
• Patients with moderate heart disease,
e.g., Tetralogy of Fallot, will often have
no restrictions placed upon them during
childhood, yet may develop long term
problems requiring medical/surgical rx:
– RV dilatation and dysfunction from chronic
pulmonary valve regurgitation
– Rhythm disturbance
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Evaluation of the Cardiovascular System in
Childhood
• Patients with successfully palliated severe
CHD, e.g., HLH, HRH, single ventricle, will
face restrictions and limitations during
childhood and likely additional difficulties
during adulthood:
– Progressive ventricular dysfunction
– Recurrent hypoxemia
– Rhythm disturbance
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Evaluation of the Cardiovascular System in
Childhood: Heart Rhythm Abnormalities
Complete Heart Block
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Evaluation of the Cardiovascular System in
Childhood: Heart Rhythm Abnormalities
• Arrhythmia: a change in the regular beat
of the heart
– Irregular heart beat
– Tachycardia: SVT, VT
– Bradycardia
• Syncope: loss of consciousness
• Near syncope: altered consciousness
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Evaluation of the Cardiovascular System in
Childhood: Heart Rhythm Abnormalities
• Association between syncope and heart
rhythm abnormality must be established
and documented:
– Holter monitoring
– Tilt table testing
– Event recorder
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Evaluation of the Cardiovascular System in
Childhood: Heart Rhythm Abnormalities
• Syncope must occur 3 or more times
within 12 months despite appropriate
medical therapy
– Tachycardia: chaotic, rapid rhythm
– Bradycardia: profound slowing
– Superimposed congestive heart failure
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Evaluation of the Cardiovascular System in
Childhood: Heart Rhythm Abnormalities
VTach
VFib
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Evaluation of the Cardiovascular System in
Childhood: Heart Rhythm Abnormalities
• Syncope must occur 3 or more times
within 12 months despite appropriate
medical therapy
– Syncope due to heart rhythm abnormality
is a marker for sudden death
– Symptoms occurring 3 or more times within
12 months requires more aggressive
therapy
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Evaluation of the Cardiovascular System in
Childhood: Inflammatory Heart Disease
Rheumatic Fever
• Persistence of LV dilatation and
dysfunction, valvular regurgitation
uncommon, but readily evaluated.
Kawasaki Disease
• Small percentage of children with residual
abnormality in childhood: coronary artery
aneurysms, thrombosis, obstruction.
• Unknown implications for ischemic heart
disease in adulthood.
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Kawasaki Disease
Angiogram
S
Anatomy
Heart Transplantation in Childhood
• The majority of transplants are done in
children with cardiomyopathy /
myocarditis.
• Palliation for complex congenital heart
disease as a primary strategy or failed
conventional surgical therapy is the 2nd
leading cause for transplantation.
Heart Transplantation In Children
450
400
350
300
11-17 Years
1-10 Years
<1 Year
250
200
150
100
50
0
82 983 984 985 986 987 988 989 990 991 992 993 994 995 996 997 998 999 000 001 002 003 004 005
9
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
2
2
2
2
2
2
ISHLT
J Heart Lung Trans 2007:26, 796
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Heart Transplantation In Children
January 1996 - June 2006
1000
900
800
700
600
500
400
300
200
100
0
0
1
2
3
4
5
6
7
8
9
10 11 12 13 14 15 16 17
Recipient Age (Years)
ISHLT data, 2007
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Number of centers
.
Heart Transplantation In Children
January 1997 – June 2006
120
110
100
90
80
70
60
50
40
30
20
10
0
1997-2001
2002-6/2006
121
105
17
1-4/yr
16
5-9/yr
9
14
10-19/yr
Average number of heart transplants per year
ISHLT data, 2007
March 20, 2016
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Heart Transplantation In Children
100
Half-life: <1: n.c.; 1-10: 17.5 Years; 11-17: 15.2 Years
90
80
70
0-<1 vs. 1-10: p = 0.0029;
0-<1 vs. 11-17: p=<.0001;
1-10 vs. 11-17: p=<.0001
60
50
<1 Year
(N = 1,148)
11-17 Years (N = 1,968)
40
1-10 Years (N = 1,845)
Overall
(N = 4,961)
30
0
1
2
3
4
5
ISHLT data, 2007
March 20, 2016
6
7
8
9
10 11 12 13 14 15 16 17 18 19 20
Years
36
Heart Transplantation In Children
100
1982-1989 (N = 860)
1995-1999 (N=1,877)
80
1990-1994 (N=1,842)
2000-6/2005 (N=2,096)
Half-life 1982-1989: 10.8 years; 1990-1994: 12.0 years;
1995-1999: n.c.; 2000-6/2005: n.c.
60
40
20
All p-values significant at p< 0.0001 except
comparison of 1995-1999 vs. 2000-6/2005
0
0
1
2
3
4
5
ISHLT data, 2007
March 20, 2016
6
7
8
9
10 11 12 13 14 15 16 17 18 19 20
Years
37
Evaluation and Treatment of the Cardiovascular
System in Childhood: 2008
• Successfully repaired, the majority of patients
with minor or moderate cardiac abnormalities
will have few if any medical issues/cardiac
disability after the 3 month recovery period.
• Successfully palliated, the vast majority of
patient with moderate or complex CHD will
continue to require close medical surveillance
and likely have cardiac symptoms and
limitations.
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Evaluation of the Cardiovascular System in
Childhood
• Many patients with complex lesions,
corrected or well palliated in childhood,
are now surviving into adulthood.
• The long-term “natural history” of
operated patients with CHD, particularly
those with complex lesions, is still being
written.
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Evaluation of the Cardiovascular System in
Childhood
• Evaluation will need to encompass
more than imaging for evaluation of
cardiac size and muscle mechanics.
– Functional assessment of heart rhythm,
exercise capacity, myocardial oxygen
consumption will eventually need to be
incorporated into guidelines.
• Confounding factors will influence results:
– Psycho-social issues with child, adolescent and
family
– Exogenous obesity
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