Pediatric Cardiac Emergencies

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Pediatric Cardiac Emergencies

• Gavin Greenfield
• Peggy Thomsen

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• 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

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• 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

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Infant Cardiac Disease Leading to ER Presentation

• Congenital
• Acquired
• Cardiomyopathy
• Myocarditis (usually with CHF)
• Dysrhythmias

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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

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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

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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

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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

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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

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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

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Etiology

• infectious
• viral
• bacterial
• TB
• fungal
• parasitic
• Connective tissue
• RA
• Rheumatic fever
• SLE

• Metabolic / Endocrine
• uremia
• hypothyroid
• Hematology / Oncology
• bleeding diathesis
• malignancy
• Trauma
• Iatrogenic

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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

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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

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

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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

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Cardiac Syncope

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

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Sudden Cardiac Death

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

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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

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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

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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

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Other dysrhythmias

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

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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

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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

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• 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

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• 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

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• 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

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Congenital Heart Disease

• Fetal to Neonatal Circulation

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Classification
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Congenital Heart Disease Classification

• pink (in failure)
• blue (no distress)
• gray (in shock)

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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

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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

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CHD Classified as Cyanotic vs. Acyanotic

• Acyanotic (L to R shunts, stenotic lesions)
• ASD
• VSD
• PDA
• aortic valve stenosis
• pulmonic valve stenosis
• aortic coarctation

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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

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Congenital Heart Disease

• History
• feeding difficulties
• tachypnea
• diaphoresis
• syncope
• cyanotic episodes
• failure to thrive

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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

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Cyanotic Congenital Heart Disease

• R to L shunts
• mixing lesions

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Tetralogy of Fallot the classic cyanotic lesion

• RV outflow obstruction
• RVH
• VSD
• overriding aorta
• CXR reveals boot shaped heart with decreased
  pulmonary blood flow

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• 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

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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

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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

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Acyanotic Congenital Heart Disease

• L to R shunts
• stenotic lesions

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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

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• 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

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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

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LV Outflow Obstruction
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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

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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

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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

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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

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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

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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

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Bradyarrhythmias

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

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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

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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

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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

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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

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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

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• 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

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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

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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

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Erythema Marginatum
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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

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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)