Rosen & Barkin's 5-Minute Emergency Medicine Consult (169 page)

• Central cyanosis:
  
  • Visible in lips, nail beds, mucosa
    
  • Increases with cry or agitation
    
  • Minimal change with 100% O
    ₂
    
• CHF:
  
  • Rales, gallop, hepatomegaly, scalp edema
    
• Hypercyanotic spells or “Tet spells”:
  
  • Restless and hyperpneic then increased cyanosis then syncope
    
  • Follows reductions in already compromised pulmonary blood flow:
    
    • Wakening, feeding, vigorous cry, exercise
      
• Older child may compensate by squatting.
  
• Temporary reduction or absence of systolic ejection murmur during spell
  

• Family history of CHD:
  
  • If parent or sibling: Increased risk of CHD
    
  • If 2 relatives: Risk of CHD triples
    
• Prenatal history:
  
  • Exposure to teratogens
    
  • Abnormal fetal ultrasound
    
• TOF:
  
  • Often asymptomatic at birth; symptoms develop as RV infundibulum hypertrophies
    
  • Severe RV outflow obstruction; neonatal cyanosis (duct-dependent lesion)
    
  • Cyanosis during crying or feeding (Tet spells) in toddlers
    
  • Older, uncorrected patients:
    
    • Dyspnea on exertion and growth delay
      
• Tricuspid atresia:
  
  • Usually cyanotic from birth
    
  • Feeding difficulties
    
  • Older patients show dyspnea on exertion and easy fatigability
    
• Ebstein anomaly:
  
  • Teens may present with dysrhythmias.
    
• TGA:
  
  • Presents in 1st hr to days of life
    
• TAPVR:
  
  • Neonatal presentation; severely ill:
    
    • Cyanosis
      
    • Does not improve with mechanical ventilation
      
  • Infantile presentation; heart failure:
    
    • Mild cyanosis
      
  • If no obstruction to pulmonary venous return:
    
    • Asymptomatic or mild cyanosis
      
    • Frequent pneumonias
      
    • Growth problems
      
• Truncus arteriosus:
  
  • Mild cyanosis in newborn
    
  • Heart failure in older infants
    

• TOF:
  
  • Loud systolic murmur left sternal border (LSB)
    
  • Systolic thrill in 50%
    
  • +/− Continuous murmur of PDA
    
  • Loud, single 2nd heart sound (S2)
    
  • RV prominence/bulge
    
  • Older, uncorrected patients:
    
    • Dusky, blue skin
      
    • Clubbing of digits
      
    • Retinal engorgement
      
• Tricuspid atresia:
  
  • Tachypnea
    
  • Regurgitant murmur from VSD at LSB
    
  • +/− continuous PDA murmur
    
  • Single S2
    
  • Prominent LV impulse
    
• Ebstein anomaly:
  
  • Holosystolic murmur of tricuspid regurgitation
    
  • Many have diastolic murmur
    
  • Gallop
    
• TGA:
  
  • Single, loud S2
    
  • Severe hypoxemia
    
• TAPVR:
  
  • Neonatal; severe tachypnea and cyanosis
    
  • Infantile; heart failure:
    
    • Tachycardia
      
    • Systolic ejection murmur at LSB
      
    • Mid-diastolic murmur at lower LSB
      
    • Gallop
      
    • Fixed, split S2
      
    • Hepatomegaly
      
• Truncus arteriosus:
  
  • Newborn:
    
    • Mild cyanosis
      
    • Regurgitant systolic murmur at LSB
      
    • Single S2
      
• Older infants; heart failure:
  
  • Hyperdynamic precordium
    
  • Bounding pulses
    
  • Wide pulse pressure
    
  • Loud, single S2
    
  • Systolic ejection murmur and thrill
    
  • Mid-diastolic murmur
    

• Oxygen saturation
  
• ABG
  
• CBC, glucose
  
• Sepsis evaluation
  
• CXR to assess pulmonary blood flow
  
• EKG to assess for hypertrophy and QRS axis
  
• Cardiology consult and ECG
  

• Decreased room air oxygen saturation
  
• Hyperoxia test:
  
  • ABG in room air and after several minutes 100% oxygen:
    
    • PaO
      ₂
      >150 in O
      ₂
      ; no intracardiac shunt
      
    • PaO
      ₂
      <100 in O
      ₂
      ; highly suspicious of cyanotic CHD
      
• CBC: Erythrocytosis with chronic cyanosis
  

• CXR:
  
  • Decreased pulmonary blood flow:
    
    • TOF (enlarged RV)
      
    • Tricuspid atresia (enlarged LV)
      
    • Single ventricle physiology
      
  • Increased pulmonary flow:
    
    • Transposition of the great vessels (large RV)
      
    • TAPVR (large RV)
      
    • Truncus arteriosus (large LV and RV)
      
  • Classic CXR descriptions:
    
    • Boot-shaped heart: TOF; large and upturned RV looks like toe of boot
      
    • Egg on a string: TGA; narrow mediastinum, great vessels anterior/posterior position
      
    • Snowman sign: Supracardiac TAPVR; upper portion formed by pulmonary veins
      

EKG:

• TOF:
  
  • Right axis deviation (RAD)
    
  • RVH
    
• TAPVR:
  
  • RAD
    
  • RVH and right atrial enlargement (RAE)
    
• Transposition of the great vessels:
  
  • RAD
    
  • RVH
    
• Tricuspid atresia:
  
  • Superior axis
    
  • LVH, RAE, LAE
    
• Truncus arteriosus:
  
  • RVH, LVH
    
• Ebstein anomaly:
  
  • Right bundle branch block
    
  • Often Wolff–Parkinson–White
    

• Pulmonary:
  
  • Pneumothorax/hemothorax
    
  • Bronchopulmonary dysplasia
    
  • Congenital lung hypoplasia/dysplasia
    
  • Pulmonary hemorrhage
    
  • Pulmonary embolus
    
  • Pulmonary HTN
    
  • Diaphragmatic hernia
    
  • Foreign body/anatomic obstruction
    
• Cardiac:
  
  • Cyanotic CHD
    
  • CHF
    
  • Cardiogenic shock
    
• Infectious:
  
  • Pneumonia, bronchiolitis
    
  • Sepsis
    
• Neurologic:
  
  • Seizure
    
  • Neuromuscular disease
    
  • Drug-induced respiratory depression
    
• Other:
  
  • Polycythemia
    
  • Methemoglobinemia
    
  • Dehydration
    
  • Hypoglycemia
    

TREATMENT

• Maintain warmth (cold ↑ O
  ₂
  consumption).
  
• Treat hypoglycemia and acidosis.
  
• Maintain oxygenation.
  
• Establish IV access.
  
• Prepare for endotracheal intubation.
  

• Administer prostaglandin E
  ₁
  (PGE
  ₁
  ) to dilate or reopen the ductus arteriosus:
  
  • Continuous IV infusion 0.05–0.1 μg/kg/min
    
  • Complications include apnea, bradycardia, hypotension, and seizures:
    
    • Generally intubate prior to transport
      
  • Not effective for obstructed TAPVR:
    
    • May require ECMO awaiting surgery
      
  • Overall benefits far outweigh potential risks
    
• Evaluate and treat alternate causes of cyanosis:
  
  • Septic workup and empiric antibiotics
    
  • Fluid resuscitate (increments of 10 mL/kg)
    
  • Maintain normoglycemia
    
• Patients with Tet spells:
  
  • Provide a calming environment.
    
  • Place child in knee-chest position.
    
  • Supplemental O
    ₂
    if not agitating to patient
    
  • IV or IM morphine
    
  • For severe cases not responding to above:
    
    • IV bicarbonate to treat severe acidosis
      
    • IV phenylephrine to ↑systemic vascular resistance and reduce R→L shunt
      
    • IV propranolol for β-adrenergic blockade
      
• Cyanosis in the older patient with known CHD:
  
  • 10–20 mL/kg NS IV if dehydration likely
    
  • Supplemental O
    ₂
    if suspicious for pulmonary diseases
    
  • Antipyretics for fever
    
  • Antibiotics for pneumonia/infectious process
    
• Circulatory collapse from CHD:
  
  • Fluid resuscitation
    
  • Inotropes: Dobutamine, dopamine, milrinone
    
  • Aggressive treatment of acidosis
    

• Acetaminophen: 15 mg/kg PO or PR
  
• Ampicillin: 50 mg/kg IV
  
• Dobutamine: 5–20 μg/kg/min IV
  
• Dopamine: 5–20 μg/kg/min IV
  
• Gentamicin: 4 mg/kg/d IV or 2.5 mg/kg/dose
  
• Ibuprofen: 10 mg/kg PO (>6 mo)
  
• Milrinone: 0.25–1 μg/kg/min
  
• Morphine sulfate: 0.1 mg/kg SC, IM, or IV
  
• Phenylephrine: 0.5–5 μg/kg/min IV
  
• Propranolol: 0.1 mg/kg IV
  
• PGE
  ₁
  : 0.05–0.1 μg/kg/min
  
• Sodium bicarbonate: 1–2 mEq/kg IV
  

FOLLOW-UP

• All newborns with suspected CHD:
  
  • Admit to pediatric ICU.
    
• CHD with acute worsening of cyanosis or CHF
  
• CHD with symptomatic pneumonia or respiratory syncytial virus
  

• Determine in consult with cardiologist
  
• Patients who respond to minimal intervention (i.e., TOF patients treated noninvasively)
  
• Ensure close follow-up.