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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. The Cardiovascular System in Childhood: Disability Evaluation under Social Security.

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The cardiovascular system in childhood

THE CARDIOVASCULAR SYSTEMIN CHILDHOOD

EVALUATION AND TREATMENT

SSA Conference, September 24, 2008

Joel Brenner, MD

Director, Pediatric Cardiology

Helen B. Taussig Children’s Congenital Heart Center


The cardiovascular system in childhood disability evaluation under social security
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
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


The cardiovascular system in childhood disability evaluation under social security1
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


Evaluation of the cardiovascular system in childhood congenital heart disease
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


Evaluation of the cardiovascular system in childhood1
Evaluation of the Cardiovascular System in Childhood Congenital Heart Disease

  • Symptoms and signs usually observed over time—3 months

  • Laboratory findings– appropriate, medically acceptable imaging

  • Response to prescribed therapy

  • Functional limitation


Evaluation of chf cyanosis
Evaluation of CHF & Cyanosis Congenital Heart Disease

  • 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


Evaluation of the cardiovascular system in childhood2
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.

Well palliated complex CHD will have minor to serious cardiac issues requiring regular, frequent surveillance through adult years.


Evaluation of the cardiovascular system in childhood imaging tests
Evaluation of the Cardiovascular System in Childhood: Imaging Tests

  • Chest X Ray

  • Echocardiography

  • Radionuclide angiography

  • Cardiac catheterization

  • Computerized tomography (CT)

  • Magnetic resonance imaging (MRI)


Imaging studies chest x ray
Imaging Studies: Chest X Ray Imaging Tests

  • 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.


Imaging studies echo
Imaging Studies: ECHO Imaging Tests

  • 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


Imaging studies echo1
Imaging Studies: ECHO Imaging Tests

  • 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 echo2
Imaging Studies: ECHO Imaging Tests


New imaging modalities that need to be added to evaluation scheme
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


New imaging modalities that need to be added to the evaluation scheme
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


Evaluation and treatment of the cardiovascular system in childhood 50 years of progress
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


Diagnosis and treatment of tga 1978
Diagnosis and Treatment of TGA: 1978 Childhood: 50 years of progress

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
Diagnosis and Treatment of TGA:2008 Childhood: 50 years of progress

Primary care physician recognizes cyanosis

Echo confirms diagnosis of TGA

Surgical repair:ASO

Post op echo

AO

PA

LV

RV


Mri in patient following arterial switch procedure for tga
MRI in Patient following Arterial Childhood: 50 years of progressSwitch Procedure for TGA

AO

PA

AO

PA

RV

RV


Advances in cardiac imaging
Advances in Cardiac Imaging Childhood: 50 years of progress

Abnormal coronary origin

Myocardial enhancement


Evaluation of the cardiovascular system in childhood3
Evaluation of the Cardiovascular System in Childhood Childhood: 50 years of progress

  • 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


Evaluation of the cardiovascular system in childhood4
Evaluation of the Cardiovascular System in Childhood Childhood: 50 years of progress

  • 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
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 abnormalities1
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


Evaluation of the cardiovascular system in childhood heart rhythm abnormalities2
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


Evaluation of the cardiovascular system in childhood heart rhythm abnormalities3
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 abnormalities4
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 abnormalities5
Evaluation of the Cardiovascular System in Childhood: Heart Rhythm Abnormalities

  • Syncope must occur 3 or more times within 12 monthsdespite 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
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
Kawasaki Disease Inflammatory Heart Disease

Angiogram

S

Anatomy


Heart transplantation in childhood
Heart Transplantation in Childhood Inflammatory Heart Disease

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
Heart Transplantation In Children Inflammatory Heart Disease

ISHLT

J Heart Lung Trans 2007:26, 796

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Heart transplantation in children january 1996 june 2006
Heart Transplantation In Children Inflammatory Heart Disease January 1996 - June 2006

ISHLT data, 2007

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Heart transplantation in children january 1997 june 2006
Heart Transplantation In Children Inflammatory Heart Disease January 1997 – June 2006

ISHLT data, 2007

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Heart transplantation in children1
Heart Transplantation In Children Inflammatory Heart Disease

ISHLT data, 2007

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Heart transplantation in children2
Heart Transplantation In Children Inflammatory Heart Disease

ISHLT data, 2007

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Evaluation and treatment of the cardiovascular system in childhood 2008
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.


Evaluation of the cardiovascular system in childhood5
Evaluation of the Cardiovascular System in Childhood Childhood: 2008

  • 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.


Evaluation of the cardiovascular system in childhood6
Evaluation of the Cardiovascular System in Childhood Childhood: 2008

  • 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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