August 9, 20151 THE CARDIOVASCULAR SYSTEM IN CHILDHOOD EVALUATION AND TREATMENT SSA Conference,...

April 19, 2023 1

THE CARDIOVASCULAR SYSTEMIN CHILDHOOD

EVALUATION AND TREATMENT

SSA Conference, September 24, 2008

Joel Brenner, MDDirector, Pediatric CardiologyHelen B. Taussig Children’s Congenital Heart Center

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.

• Chronic CHF or ventricular dysfunction.• Pain due to myocardial ischemia• Syncope from any cardiac cause• Central cyanosis

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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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• 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

• Signs– Increased respiratory

rate (PVC)– Hepatomegaly,

peripheral edema (SVC)

– Failure to thrive– Decreased O2 sat– Elevated Hct

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Well repaired minor or moderate CHD will have no/minor medical issues and require regular but infrequent surveillance.

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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• 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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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

• 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 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 CHD

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Evaluation and Treatment of the Cardiovascular System in Childhood: 50 years of progress

• 1958• 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

• 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: repaired

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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

RV

AO

Diagnosis and Treatment of TGA:2008

• Primary care physician recognizes cyanosis

• Echo confirms diagnosis of TGA

• Surgical repair:ASO• Post op echo

RV

AO

LV

PA

MRI in Patient following Arterial Switch Procedure for TGA

RV

PA

RV

PA

AO

AO

Advances in Cardiac Imaging

Myocardial enhancement Abnormal coronary origin


• 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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• 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

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Complete Heart Block


• 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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• Association between syncope and heart rhythm abnormality must be established and documented:– Holter monitoring– Tilt table testing– Event recorder

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• 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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VTach

VFib


• 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 SAnatomy

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.

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Heart Transplantation In Children

ISHLT J Heart Lung Trans 2007:26, 796

0

50

100

150

200

250

300

350

400

45011-17 Years

1-10 Years

<1 Year

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Heart Transplantation In ChildrenJanuary 1996 - June 2006

ISHLT data, 2007

0

100

200

300

400

500

600

700

800

900

1000

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17

Recipient Age (Years)

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Heart Transplantation In ChildrenJanuary 1997 – June 2006

121

17

105

16 1490

10

20

30

40

50

60

70

80

90

100

110

120

1-4/yr 5-9/yr 10-19/yr

Average number of heart transplants per year

Nu

mb

er

of

cen

ters

. 1997-2001

2002-6/2006

ISHLT data, 2007

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ISHLT data, 2007

30

40

50

60

70

80

90

100

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20

Years

<1 Year (N = 1,148) 1-10 Years (N = 1,845)11-17 Years (N = 1,968) Overall (N = 4,961)

0-<1 vs. 1-10: p = 0.0029; 0-<1 vs. 11-17: p=<.0001;1-10 vs. 11-17: p=<.0001

Half-life: <1: n.c.; 1-10: 17.5 Years; 11-17: 15.2 Years

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ISHLT data, 2007

0

20

40

60

80

100

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20

Years

1982-1989 (N = 860) 1990-1994 (N=1,842)

1995-1999 (N=1,877) 2000-6/2005 (N=2,096)

All p-values significant at p< 0.0001 except comparison of 1995-1999 vs. 2000-6/2005

Half-life 1982-1989: 10.8 years; 1990-1994: 12.0 years; 1995-1999: n.c.; 2000-6/2005: n.c.

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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• 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 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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August 9, 20151 THE CARDIOVASCULAR SYSTEM IN CHILDHOOD EVALUATION AND TREATMENT SSA Conference,...

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