Rosen & Barkin's 5-Minute Emergency Medicine Consult (168 page)

• Oxygen saturation (pre- and postductal)
  
• ABG, CBC, basic chemistries, and glucose
  
• Sepsis evaluation
  
• CXR to assess pulmonary blood flow
  
• EKG (axis, hypertrophy, conduction delays)
  
• 4-extremity BPs
  
• Cardiology consult with ECG
  

• CXR:
  
  • L→R shunting lesions all show cardiomegaly (specific chambers) and ↑ pulmonary markings
    
    • ASD (RA, RV), VSD (RV, LA), PDA (LA, LV)
      
    • AV canal (globular; all chambers enlarged)
      
• Obstructive lesions: Normal to cardiomegaly
  

EKG:

• ASD: Right axis deviation:
  
  • RVH or right bundle branch block (RBBB)
    
• VSD–LAH, LVH (if large, also RVH):
  
  • Notched or peaked P-waves (large VSD)
    
• PDA: Biventricular hypertrophy (large PDA)
  
• AV canal: Superior axis, LVH, RVH:
  
  • RBBB and prolonged PR interval
    
• AS: Normal to LVH (severe cases)
  
• PS: Normal to RVH, RAE (severe cases):
  
  • RBBB
    
• Coarctation of aorta: RVH or RBBB
  
• HLHS: RAE, RVH, peaked P-waves
  

• CHF
  
• Hypertrophic cardiomyopathy
  
• Cardiogenic shock
  
• Aortic dissection
  
• Myocarditis
  
• Bronchopulmonary dysplasia
  
• Pulmonary HTN
  
• Pneumonia/bronchiolitis
  
• Hypoglycemia
  
• Adrenal insufficiency, CAH
  
• Glycogen storage diseases
  
• Sepsis
  
• Shock
  

TREATMENT

• Maintain warmth and oxygenation.
  
• Treat hypoglycemia and acidosis.
  
• 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.
    
• Evaluate and treat alternate causes:
  
  • Septic workup and empiric antibiotics
    
  • Maintain normoglycemia
    
• Circulatory collapse from CHD:
  
  • Fluid resuscitation (increments of 10 mL/kg)
    
  • Inotropes
    
  • Aggressive treatment of acidosis
    
• CHF:
  
  • Digoxin and diuretics
    

• Ampicillin 50 mg/kg IV
  
• Digoxin dosing requires extreme caution:
  
  • Range 25–40 μg/kg IV
    
• Dobutamine: 5–20 μg/kg/min IV
  
• Dopamine: 2–20 μg/kg/min IV
  
• Epinephrine: 0.1–2 μg/kg/min IV
  
• Furosemide: 1 mg/kg IV
  
• Gentamicin: 4 mg/kg/d IV or 2.5 mg/kg/dose
  
• Milrinone 0.25–1 μg/kg/min
  
• 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 pneumonia or bronchiolitis
  

Determine in consult with cardiologist

Primary care physician to coordinate care with cardiologist and cardiothoracic surgery

Plan for follow-up should be made in consult with the pediatric cardiologist.

• Acyanotic lesions presenting at 2–12 wk:
  
  • Coarctation as DA closes
    
  • Septal defects as pulmonary vascular resistance drops
    
• Classic ECG in AV canal: Superior QRS axis
  
• Classic CXR in coarctation: Rib notching (late)
  

• Bonow RO, Mann DL, Zipes DP, et al., eds. Congenital heart disease.
  Braunwald’s Heart Disease
  . 98th ed. Philadelphia, PA: Saunders Elsevier; 2012:1411–1467.
  
• Dolbec K, Mick N. Congenital heart disease.
  Emerg Med Clin North Am.
  2011;29:811–827.
  
• Yee L. Cardiac emergencies in the first year of life.
  Emerg Med Clin North Am
  . 2007;25:981–1008.
  

See Also (Topic, Algorithm, Electronic Media Element)

• Congestive Heart Failure
  
• Failure to Thrive
  
• Neonatal Sepsis
  

CODES

• 745.4 Ventricular septal defect
  
• 745.5 Ostium secundum type atrial septal defect
  
• 746.89 Other specified congenital anomalies of heart
  

BASICS

• Aberrant embryonic development results in mixing of deoxygenated and oxygenated blood returning to systemic circulation by 2 mechanisms:
  
  • Right-to-left intracardiac shunt
    
  • Anatomic defects of the aortic root
    
• Subtypes: 5 T’s, 2 E’s, single ventricle:
  
  • Tetralogy of Fallot (TOF):
    
    • Ventricular septal defect (VSD)
      
    • Right ventricular (RV) outflow obstruction
      
    • Overriding aorta
      
    • RV hypertrophy (RVH)
      
  • Transposition of the great arteries (TGA):
    
    • Aorta arises from RV and pulmonary artery from left ventricle (LV)
      
  • Tricuspid atresia:
    
    • No outlet from right atrium to RV
      
    • Obligatory atrial level connection
      
  • Truncus arteriosus:
    
    • Single arterial trunk for systemic, pulmonic, and carotid circulations
      
  • Total anomalous pulmonary venous return (TAPVR):
    
    • Pulmonary veins drain into systemic venous circulation
      
    • Supracardiac, cardiac, infracardiac, or mixed
      
  • Ebstein anomaly of tricuspid valve:
    
    • Abnormal and displaced tricuspid valve divides RV resulting in poor RV function
      
  • Eisenmenger syndrome:
    
    • Complication in longstanding acyanotic heart disease with L→R shunts
      
    • Pulmonary vascular resistance reaches suprasystemic levels; R→L shunt
      
  • Single ventricle physiology:
    
    • Total mixing of systemic and venous return
      

For most forms, cause is unknown

DIAGNOSIS

• Most common initial ED presentations of cyanotic congenital heart disease (CHD):
  
  • Cyanosis
    
  • CHF
    
  • Circulatory collapse
    
• Physiologic stress triggers cyanosis in older patients with CHD:
  
  • Cardiac shunt obstruction
    
  • Pulmonary disease
    
  • Decreased systemic vascular resistance
    
  • Fever
    
  • Dehydration