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Pediatric and Congenital Heart Disease: Compassionate Allowances

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Geoffrey L. Rosenthal, MD, PhD November 9, 2010 * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * Financial conflicts of interest: None Identify most severe ... – PowerPoint PPT presentation

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Title: Pediatric and Congenital Heart Disease: Compassionate Allowances


1
Pediatric and Congenital Heart Disease Compassion
ate Allowances
Geoffrey L. Rosenthal, MD, PhD
November 9, 2010
2
Disclosures
  • Financial conflicts of interest None

3
Compassionate Allowances Goals of Testimony
  • Identify most severe forms of cardiovascular
    disease in children
  • Discuss diagnosis and treatment of these
    disorders
  • Provide insight into the pervasiveness of
    medicine in the lives of families
  • Discuss functional limitations/expectations for
    children with these diagnoses
  • Address emerging science and research

4
Compassionate Allowances Program Goals
  • Reduce backlog of cases by fast tracking
    applications
  • Reduce administrative costs of processing claims
  • Develop List of diagnoses which better
    discriminate children meeting cardiovascular
    disability criteria
  • Maintain a low rate of denying benefits when they
    should be allowed, and of allowing benefits when
    they should be denied

5
Compassionate Allowances Program Goals
  • Identify diseases that are certain or near
    certain to cause disability or death within 12
    months
  • Identify diseases that, by definition, cause
    disability

6
Compassionate Allowances SSA Suggested Dx for
Discussion
  • Single ventricle
  • Hypoplastic Left Heart Syndrome (HLHS)
  • Aortic valve atresia
  • Tricuspid valve atresia
  • Pulmonary atresia with intact ventricular septum
    (PAIS or PAIVS)
  • Long QTc Syndrome with aborted sudden death
  • Childhood myocardial infarction
  • Heart transplantation

7
Compassionate Allowances Finding a Framework for
Severe
  • Palliated Cardiovascular Malformations, and
    Repaired Malformations with lifelong sequelae
  • Single ventricle and complex two ventricle
    lesions
  • Channelopathies
  • Cardiomyopathies
  • Congenital Heart Disease with comorbidities
  • Congenital Heart Disease requiring specific
    treatments/events

8
Palliated and Repaired CHD, lt12 Month Old, Single
Ventricle
  • Hypoplastic left heart syndrome (HLHS)
  • Aortic atresia, Mitral atresia, AA/MA
  • Pulmonary atresia with intact ventricular septum
  • Tricuspid atresia (all subtypes)
  • Unbalanced atrioventricular canal (all subtypes)
  • Double inlet left ventricle
  • Some forms of double outlet right ventricle
  • Single ventricle with indeterminate morphology

9
All Forms of Single Ventricle
  • Diagnostic determination of single ventricle can
    be made with a very high degree of certainty
    using echocardiography
  • Resting cyanosis (paO2 lt 60 Torr) pre-op and
    post-op while infants
  • Clinically significant risk of death in the first
    year of life (approximately 10-50)
  • Usually require 3 or more hospitalizations in
    first year of life for diagnosis, stabilization,
    surgery, and/or cardiac catheterization

10
Palliated and Repaired CHD, lt12 Month Old,
Complex Two Ventricles
  • Tetralogy of Fallot
  • With pulm. atresia, absent pulm. valve syndrome,
    discontinuous or hypoplastic pulm. arteries
  • Transposition of the great arteries (d-TGA and
    l-TGA, with or without other cardiac lesions)
  • Pulmonary atresia with ventricular septal defect
    and multiple aortopulmonary collaterals
  • Remaining forms of double outlet right ventricle
  • Critical aortic valve stenosis
  • Shones complex
  • Critical pulmonary valve stenosis

11
Palliated and Repaired CHD, lt12 Month Old,
Complex Two Ventricles
  • Total anomalous pulmonary venous return (all
    types)
  • Interrupted aortic arch (all types)
  • Truncus arteriosus (all types)
  • Ebsteins anomaly diagnosed in infancy (with or
    without associated lesions)
  • Heterotaxy syndrome (all types)
  • Pulmonary vein stenosis/sclerosis involving 2 or
    more pulmonary veins

12
Complex Two Ventricles, Functional Sequelae
Common
  • Diagnosis of complex two ventricle lesions can be
    made with a very high degree of certainty using
    echocardiography
  • Precise anatomical diagnosis may require cMRI or
    cardiac catheterization
  • Resting cyanosis (paO2 lt 60 Torr) pre-op for
    most, low cardiac output for remaining

13
Complex Two Ventricles, Functional Sequelae
Common
  • Clinically significant risk of death in the first
    year of life (approximately 5-50)
  • Usually require 2 or more hospitalizations in
    first year of life for diagnosis, stabilization,
    surgery, and/or cardiac catheterization
  • Hospitalizations are often prolonged (LOS gt 2
    weeks)
  • Sequelae, residual lesions, need for nutritional
    support, need for home care is common

14
Cardiovascular Genetics
15
Inherited Channelopathies
  • Long QT syndromes
  • Brugada syndrome variants
  • Atrial arrhythmia syndromes
  • Short QT syndrome
  • Catecholaminergic ventricular tachycardia

16
Inherited Channelopathies
  • Diagnosis very reliable when based upon clinical
    features, family history, ECG, heart rhythm
    assessment, and genetic testing
  • Clinically significant risk of arrhythmia,
    syncope, and death
  • Most require medications and lifestyle
    modifications
  • Many have associated developmental/functional
    disabilities
  • Most require electrophysiology procedures,
    pacemaker, and/or internal cardioverter-defibrilla
    tor

17
Inherited Channelopathies
  • Devices require lifestyle modification to prevent
    lead fracture and device malfunction
  • Devices may be associated with psychological
    symptoms (body image, anxiety, depression)
  • Often limit age-appropriate abilities at home, in
    school, and in the community
  • Hospitalizations may be prolonged (LOS gt 2 weeks)

18
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19
Cardiomyopathies
  • Autosomal, Gonosomal, or Mitochondrial
  • Acquired (infectious, post-infarction,
    post-bypass, toxic, nutritional)
  • Dilated
  • Hypertrophic
  • Restrictive
  • Arrhythmogenic right ventricular dysplasia
  • Many occur within defined systemic syndromes

20
Childhood Myocardial Infarction
  • Anomalous left coronary arising from the
    pulmonary artery (ALCAPA)
  • Kawasaki Disease with coronary artery aneurysms
  • Anomalous left coronary arising from the right
    cusp and passing between the aorta and the
    pulmonary artery
  • The causes of myocardial infarction in children
    are different than in adults, but the outcomes
    are similar

21
Cardiomyopathies
  • Diagnosis very reliable when based upon clinical
    features, physiological assessment, family
    history, echocardiography, and genetic testing
  • Significant risk of arrhythmia, syncope, and
    sudden death
  • Most require medications, lifestyle modifications
  • Most have significant functional limitations
  • Many have associated developmental disabilities
  • Some require palliative surgery, internal
    cardioverter-defibrillator

22
Cardiomyopathies - Transplantation
  • Pediatric Heart Transplantation Medical Urgency
    Status Codes
  • Status 1A and 1B patients meet criteria for
    compassionate allowance prior to transplant

23
Cardiomyopathies - Transplantation
  • Status 1A - Registrant less than 18 yrs of age
    and meets at least one of the following criteria
    (a) requires assistance with a ventilator (b)
    requires assistance with a mechanical assist
    device (c) requires assistance with a balloon
    pump (d) is less than 6 months old with
    congenital or acquired heart disease exhibiting
    reactive pulmonary hypertension at greater than
    50 of systemic level (e) requires infusion of
    high dose or multiple inotropes or (f) meets
    none of the criteria specified above but has a
    life expectancy without a heart transplant of
    less than 14 days

24
Extracorporeal Membrane Oxygenator (ECMO) - Bypass
25
Cardiomyopathies - Transplantation
  • Status 1B - Registrant who (a) requires infusion
    of low dose single inotropes, (b) is less than 6
    months old and does not meet the criteria for
    Status 1A, or (c) exhibits growth failure (see
    OPTN policies for definition).

26
Congenital Heart Disease with Co-Morbidities
  • Prematurity lt37 weeks gestation
  • Neuroradiographic signs of injury
  • Microcephaly
  • Post-operative seizures
  • Developmental delay identified before 1 year of
    age
  • Multiple congenital anomalies
  • Syndromes associated with developmental delay and
    functional impairment (Down, Williams, DiGeorge,
    CHARGE, Noonan, Jacobsen)

27
Syndromes and Cardiovascular Anomalies
28
Congenital Heart Disease requiring Specific
Treatments/Events
  • Length of stay in ICU gt 2 weeks
  • Need for Cardiopulmonary resuscitation
  • Need for mechanical circulatory support (ECMO,
    VAD)
  • Need for tracheostomy
  • Need for continuous infusion of pulmonary
    vasodilators or inotropes
  • Prior fetal intervention

29
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30
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