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Pediatric Cardiac Emergencies

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Title: Pediatric Cardiac Emergencies


1
Pediatric Cardiac Emergencies
  • Gavin Greenfield
  • Peggy Thomsen

2
  • 4 year old female presents with fast breathing,
    grunting, cough x 5 days
  • seen 2 days earlier and started on steroids and
    bronchodilators
  • initial vitals HR 150, BP 100/85, RR 36, T 37.5

3
  • 1 month old with irritability, poor feeding
    (fatigues), failure to thrive, fast breathing
  • no fever or runny nose
  • physical exam HR 160, RR 60 with minimal
    respiratory distress, gallop rhythm, rales

4
Infant Cardiac Disease Leading to ER Presentation
  • Congenital
  • Acquired
  • Cardiomyopathy
  • Myocarditis (usually with CHF)
  • Dysrhythmias

5
Congestive Heart Failure
  • the physiologic state in which cardiac output is
    unable to meet tissue metabolic demands (Rosen)
  • CO HR x SV
  • SV dependent upon preload, afterload,
    contractility

6
CHF - Presentation
  • infants irritable, poor feeding (early fatigue),
    failure to thrive, respiratory symptoms
  • always consider in patients with respiratory
    symptoms
  • often misdiagnosed as respiratory illness /
    infection

7
CHF - Etiology
  • Increased Preload
  • L to R shunts (VSD, PDA, AV fistula)
  • severe anemia
  • Increased Afterload
  • HTN
  • Congenital (aortic stenosis, coarctation of
    aorta)
  • Decreased Contractility
  • myocarditis, pericarditis with tamponade
  • cardiomyopathy (dilated or hypertrophic)
  • Kawasaki syndrome (early phase)
  • metabolic electrolyte, hypothyroid
  • myocardial contusion
  • toxins dig, calcium channel blockers, beta
    blockers
  • Dysrhythmia

8
CHF - Etiology
  • presents immediately at birth
  • anemia, acidosis, hypoxia, hypoglycemia,
    hypocalcemia, sepsis
  • presents at 1 day (congenital)
  • PDA in premature infants
  • presents in first month (congenital)
  • HPLV, aortic stenosis, coarctation, VSD presents
    later
  • presents later (acquired)
  • myocarditis, cardiomyopathy (dilated or
    hypertrophic), SVT, severe anemia, rheumatic fever

9
Myocarditis
  • leading cause of dilated cardiomyopathy and one
    of the most common causes of CHF in children
  • etiology idiopathic, viral, bacterial, parasitic
  • hallmark is CHF
  • failure to respond to bronchodilators in wheezing
    child
  • treatment includes inotropes, afterload
    reduction, diuretics, antibiotics, antivirals

10
Pericarditis
  • sharp stabbing precordial pain
  • worse with supine and better leaning forward
  • no sensory innervation of the pericardium
  • pain referred from diaphragmatic and pleural
    irritation

11
Etiology
  • infectious
  • viral
  • bacterial
  • TB
  • fungal
  • parasitic
  • Connective tissue
  • RA
  • Rheumatic fever
  • SLE
  • Metabolic / Endocrine
  • uremia
  • hypothyroid
  • Hematology / Oncology
  • bleeding diathesis
  • malignancy
  • Trauma
  • Iatrogenic

12
Pericarditis
  • usually a benign course
  • virulent bacteria (H. flu, E. coli) can cause
    constrictive pericarditis and subsequent
    tamponade may need urgent pericardiocentesis
  • uncomplicated pericarditis usually responds to
    rest and anti-inflammatories

13
Chest Pain
  • 4 of children will have a cardiac origin
  • remainder MSK, pulmonic (asthma, bronchitis,
    pneumonia), GI
  • Cardiac causes myocarditis, pericarditis,
    structural abnormalities such as congenital heart
    disease or hypertrophic cardiomyopathy

14
  • 14 year old male collapses at school while in
    class
  • non-responsive for one minute
  • feels fine in the department
  • Approach?

15
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16
Syncope
  • 20-50 of adolescents experience at least one
    episode of syncope
  • most cases benign
  • Pathophysiology
  • vascular
  • orthostatic, hypovolemia
  • neurally mediated
  • hypoxia PE, CNS depression from OD, CO
  • cardiac

17
Cardiac Syncope
  • Dysrhythmias
  • tachy
  • brady
  • Outflow obstruction
  • Myocardial Dysfunction
  • cardiac syncope often precedes future sudden
    cardiac death

18
Sudden Cardiac Death
  • includes those causes that directly relate to
    cardiovascular dysfunction
  • one third of all sudden deaths

19
Sudden Cardiac Death
  • Etiology
  • myocarditis
  • cardiomyopathy (hypertrophic)
  • cyanotic and noncyanotic congenital heart disease
  • valvular heart disease
  • congenital complete heart block
  • WPW
  • long QT syndrome
  • Marfan syndrome
  • coronary artery disease
  • anomalous coronary arteries

20
Risk Factors for Serious Cause of Syncope
  • history of cardiac disease in patient
  • FH of sudden death, cardiac disease, or deafness
  • recurrent episodes
  • recumbent episode
  • exertional
  • prolonged loss of consciousness
  • associated chest pain or palpitations
  • medications that can alter cardiac conduction

21
What to look for in the Department EKG
  • Long QT syndrome
  • congenital or acquired
  • get paroxysmal v tach with torsades de pointes
  • congenital long QT associated with hypertrophic
    cardiomyopathy
  • long QT defined as corrected QT longer than 0.44
    s
  • T wave alternans sometimes present
  • can have normal ECG in the department
  • two clinical syndromes not associated with
    structural heart disease Romano-Ward and
    Jervell-Lange-Nielsen

22
Other dysrhythmias
  • WPW and other SVTs
  • AV block
  • usually acquired, rarely congenital
  • Sick sinus syndrome

23
Idiopathic Hypertrophic Cardiomyopathy
  • aka IHSS
  • both a fixed and dynamic subvalvular obstruction
  • characterized by ventricular hypertrophy with
    principle involvement of the ventricular septum
  • associated with long QT
  • autosomal dominant
  • often presents with exertional syncope
  • 10 year mortality is 50 for children diagnosed
    by age 14

24
Other structural cardiac diseases
  • dilated cardiomyopathy
  • usually secondary to myocarditis
  • syncope and death secondary to ventricular
    dysrhythmias or severe myocardial dysfunction
  • arrhythmogenic RV dysplasia
  • congenital cyanotic and non-cyanotic heart
    disease
  • valvular diseases
  • aortic stenosis
  • coronary artery anomalies
  • exertional syncope or sudden death
  • aberrant artery passes between aorta and
    pulmonary artery

25
  • 2 week old infant brought in by parents with
    difficulty breathing
  • HR 180, BP 50/P, RR 80, T 37.5
  • history and physical
  • investigations
  • repeat vitals HR 30, no BP, RR 12
  • definitive treatment

26
  • 4 year old male presents with 2 weeks history of
    cough, fast breathing, fatigue, decreased
    exercise tolerance, puffy eyes
  • On exam tachypneic, moderate respiratory
    distress, O2 sats 92, bilateral crackles

27
  • 6 month male presents with failure to thrive,
    fast breathing, blue lips
  • On exam tachypnea but no respiratory distress,
    lips and extremities blue, oxygen saturations 70

28
Congenital Heart Disease
  • Fetal to Neonatal Circulation

29
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30
Classification
31
Congenital Heart Disease Classification
  • pink (in failure)
  • blue (no distress)
  • gray (in shock)

32
Anatomic Classification 4 groups
  • Right to Left Shunt
  • Tetralogy of Fallot
  • Transposition of the Great Arteries
  • Tricuspid Atresia
  • Left to Right Shunt
  • ASD
  • VSD
  • PDA
  • Stenotic
  • Aortic valve stenosis
  • Pulmonic valve stenosis
  • Aortic coarctation  
  • Mixing
  • Truncus
  • Total Anomalous Pulmonary Venous Return
  • Hypoplastic left heart syndrome

33
CHD Classified as Cyanotic vs. Acyanotic
  • Cyanotic (R to L shunt and mixing lesions)
  • tetralogy of Fallot
  • transposition of great vessels
  • tricuspid atresia
  • total anomalous pulmonary venous return
  • truncus arteriosus
  • hypoplastic left heart syndrome

34
CHD Classified as Cyanotic vs. Acyanotic
  • Acyanotic (L to R shunts, stenotic lesions)
  • ASD
  • VSD
  • PDA
  • aortic valve stenosis
  • pulmonic valve stenosis
  • aortic coarctation

35
Cyanosis
  • Classified as central or peripheral
  • Central cyanosis (always abnormal)
  • mucous membranes, trunk, extremities
  • classified as cardiac (R to L shunt) or pulmonary
  • Peripheral cyanosis (acrocyanosis)
  • no involvement of mucous membranes
  • involves hands, feet, circumoral area
  • common in neonates from vasomotor instability
  • CHF, PVD, shock, cold extremities

36
Congenital Heart Disease
  • History
  • feeding difficulties
  • tachypnea
  • diaphoresis
  • syncope
  • cyanotic episodes
  • failure to thrive

37
Congenital Heart Disease
  • Physical Examination
  • colour pink, blue, gray
  • vitals tachypnea, tachycardia, BP
  • symptoms suggestive of infection
  • palpation and auscultation of precordium
  • chest auscultation
  • survey for organomegaly
  • pulses in all extremities

38
Cyanotic Congenital Heart Disease
  • R to L shunts
  • mixing lesions

39
Tetralogy of Fallot the classic cyanotic lesion
  • RV outflow obstruction
  • RVH
  • VSD
  • overriding aorta
  • CXR reveals boot shaped heart with decreased
    pulmonary blood flow

40
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41
  • 2 month old female with known tetralogy of Fallot
    brought in with 24 hour history of vomiting and
    diarrhea
  • On exam moderate dehydration
  • during IV attempts patient becomes irritable and
    cyanotic

42
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43
Treatment of Tet Spell
  • quiet, calm environment
  • knee-chest or squatting position
  • increases afterload thus decreasing R to L
    shunting
  • Oxygen
  • Morphine
  • to treat hyperpnea and decrease systemic
    catecholamines
  • Phenylephrine
  • increases afterload thereby decreasing R to L
    shunt
  • Manual external aortic compression below level of
    renal arteries
  • Propranolol
  • to block beta receptors in infundibulum therefore
    lessening RV outflow obstruction

44
Consider
  • consider small volume challenge (5-10 cc/kg) to
    increase preload and reduce dynamic outflow
    obstruction
  • ?NaHCO3 for correction of acidosis
  • may need general anesthesia if severe and/or
    prolonged spell
  • interim prophylactic treatment with propranolol
    while awaiting surgery

45
Acyanotic Congenital Heart Disease
  • L to R shunts
  • stenotic lesions

46
VSD
  • Most common congenital lesion
  • Large VSDs may be silent and become symptomatic
    in first few weeks as pulmonary resistance ?
  • SOB and diaphoresis w feeds
  • Poor weight gain
  • Systolic murmur
  • CXR demonstrates CHF

47
  • 2 week old infant brought in by parents with
    difficulty breathing
  • HR 180, BP 50/P, RR 80, T 37.5
  • history and physical
  • investigations
  • repeat vitals HR 30, no BP, RR 12
  • definitive treatment

48
Differential Dx of Infant Shock
  • infection (septic shock/ meningitis)
  • bacterial GBS, E. coli, S. aureus
  • virus enteroviruses, H. simplex
  • metabolic amino/organic acidopathies, urea cycle
    defect
  • hypoxic shock eg. RSV, C.N.S. depression
  • heart disease congenital or acquired

49
LV Outflow Obstruction
50
LV Outflow Obstruction
  • Aortic coarctation
  • Hypoplastic left heart syndrome
  • Aortic stenosis (presents later)
  • Rosen any neonate in shock that does not
    respond to fluids or pressors has LV outflow
    obstruction until proven otherwise
  • complete obstruction incompatible with life
    unless there is shunting

51
Coarctation of the aorta
  • Most often distal to L subclavian
  • Can be diagnosed anytime
  • Neonates present as acutely ill, gray shocky
    (from DA closure)
  • Systolic murmur at the back
  • Hepatomegaly
  • Diminished femoral pulses
  • BP difference b/t arms and legs
  • CXR demonstrates CHF
  • Treatment of CHF
  • Prostaglandin E1

52
Duct Dependant Lesions
  • Duct needed to perfuse lungs or periphery
  • Lungs
  • Tetralogy of Fallot, transposition of great
    arteries, tricuspid or pulmonary atresia
  • a patent ductus arteriosus results in preserved
    pulmonary blood flow
  • Periphery
  • Aortic coarctation (severe) and Hypoplastic left
    heart

53
Treatment of acute decline in patients with
ductal dependant lesions
  • Open the closed duct
  • Prostaglandin E1 0.1 ug/kg/min infusion
  • reduce dosage as perfusion and colour return
  • Rosen any infant in the first week of life with
    decreased perfusion, hypotension, or acidosis
    should be considered a candidate for PGE1
    administration

54
What do you need to know about PGE ?
  • it functions by dilating vascular smooth muscle,
    both systemically and in the pulmonary vascular
    bed
  • its use in CHD pts is to maintain patency of
    the PDA, whether to maintain PBF or to maintain
    systemic blood flow past a

55
Classification Review
  • pink child in respiratory distress suggests
    acyanotic chd (L to R shunt, coarct, aortic
    stenosis)
  • blue cyanotic child in little respiratory
    distress suggests R to L shunt or mixing lesions
  • gray, shocky baby suggests outflow tract
    obstruction

56
Bradyarrhythmias
  • Etiology
  • hypoxia, acidosis, hypoglycemia
  • excess vagal stimulation (ex. intubation)
  •  
  • Treatment
  • Epinephrine
  • Atropine if known vagally mediated or heart block

57
Congenital Bradyarrhythmias
  • complete AV block
  • autoimmune injury to fetal conduction system
    secondary to maternal autoimmune disease
  • atropine, isoproteronol, epinephrine may be tried
    temporarily prior to pacing

58
Tachyarrhythmias
  • Supraventricular Tachycardia
  • re-entrant with accessory pathway (AV nodal or
    WPW)
  • re-entrant without accessory pathway (re-entry
    occurs within sinus node or within atrium)
  • ectopic
  • nonspecific presentations in infants

59
Murmurs
  • Areas
  • aortic R 2nd intercostal space
  • pulmonic L 2nd intercostal space
  • mitral apex
  • tricuspid and VSD L lower sternal border
  • Pathologic
  • diastolic, holosystolic, late systolic, continuous

60
Innocent Heart Murmurs
  • History
  • normal growth and development, normal exercise
    tolerance
  • no history of cyanosis
  • Physical Examination
  • Grade II or less, localized
  • varies with position (decreased with upright
    posture)
  • normal precordium
  • normal pulses
  • Lab
  • normal EKG, normal CXR

61
3 innocent murmurs
  • Stills
  • short ejection systolic murmur
  • musical or vibratory quality
  • heard best between apex and left sternal border
  • physiologic pulmonary flow murmur
  • harsh, located at pulmonic area
  • peripheral arterial stenosis
  • low-intensity systolic ejection murmur best heard
    in axilla and back

62
  • 8 year old male presents with fever, arthralgias
  • mother mentions that he had a sore throat 3 weeks
    ago for a few days with spontaneous resolution
  • a throat swab was done and positive for GAS but
    patient better so did not take the prescribed
    antibiotics

63
Acute Rheumatic Fever
  • school aged children
  • associated with certain strains of Group A
    beta-hemolytic streptococcal infections
  • the streptococcal organism stimulated antibody
    production to host tissues
  • CT of heart, joints, CNS, subcutaneous tissues,
    skin
  • carditis is an endomyocarditis with valvulitis
    involving mitral and aortic valves
  • 2 to 6 weeks post streptococcal pharyngitis

64
Jones Criteria
  • Major
  • carditis
  • new or changing murmur
  • cardiomegaly, CHF
  • pericarditis
  • migratory polyarthritis
  • chorea
  • erythema marginatum
  • subcutaneous nodules
  • Minor
  • fever
  • arthralgia
  • history of previous ARF
  • elevated ESR, CRP
  • prolonged PR on EKG
  • Rising titer of antistreptococcal antibodies

65
Erythema Marginatum
66
ER Treatment
  • management of complicating features of carditis
    (CHF)
  • significant carditis or CHF managed with
    glucocorticoids
  • high-dose ASA 75-100 mg/kg/day
  • pencillin
  • long term management of rheumatic heart disease

67
Pediatric EKGs General Principles
  • RV Dominance at birth gradually changes to LV
    dominance
  • axis up to 180 in normal newborn
  • T waves negative in right precordial leads until
    adolescence (except they are upright in first
    week of life)
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