Congenital Heart Disease Causes

Aka: Congenital Heart Disease Causes, Cyanotic Congenital Heart Disease, Acyanotic Congenital Heart Disease, Ductal Dependent Lesion

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II. Causes: Most Common

  1. Cyanotic Congenital Heart Disease (5 T's, with 1-5 mnemonic)
    1. Truncus Arteriosus (1 vessel)
    2. Transposition of the Great Vessels (2 vessels switched)
    3. Tricuspid valve atresia (3 valve cusps)
    4. Tetralogy of Fallot (4 components)
    5. Total Anomalous Pulmonary Venous Return (5 words)
  2. Acyanotic Congenital Heart Disease: Left to right shunt
    1. Ventricular Septal Defect (most common CHD cause)
    2. Atrial Septal Defect
    3. Patent Ductus Arteriosus
    4. Bicuspid Aortic Valve (most common CHD cause affecting adults)

III. Causes: Pressure Overload - Ductal Dependent Lesions (Obstructive, presenting early within first month of life)

  1. Right-sided obstruction (Blue babies with Cyanosis, ductus required for pulmonary Blood Flow)
    1. Tricuspid atresia
    2. Pulmonary atresia (with intact ventricular septum)
    3. Tetralogy of Fallot (if associated with critical pulmonary stenosis)
    4. Pulmonary stenosis
    5. Ebstein's Anomaly
    6. Transposition of the Great Vessels
  2. Left-sided obstruction (Gray babies in shock, ductus required for systemic Blood Flow)
    1. Hypoplastic Left Heart
    2. Critical Aortic Stenosis
    3. Aortic Coarctation
    4. Aortic atresia

IV. Causes: Volume Overload - Congestive Heart Failure

  1. Large Ventricular Septal Defect (VSD)
  2. Large Patent Ductus Arteriosus (PDA)
  3. Endocardial Cushion Defect
  4. Partial Anomalous Pulmonary Venous Return (PAPVR)
  5. Total Anomalous Pulmonary Venous Return (TAPVR)
  6. Truncus Arteriosus
  7. Tetralogy of Fallot (with critical pulmonary stenosis)
  8. Ebstein Anomaly

V. Causes: Presentation in Week 1

  1. Transient Myocardial Ischemia
  2. Dysrhythmias
  3. Arteriovenous Fistula
    1. Increased Pulses
  4. Critical Aortic Coarctation (Interrupted aortic arch)
    1. Asymmetric Pulses
  5. Aortic Stenosis
    1. Decreased pulses
    2. Left Ventricular Hypertrophy
  6. Hypoplastic Left Heart Syndrome
    1. Most common CHF cause in first week
    2. Decreased pulses
    3. Right Ventricular Hypertrophy
    4. Hyperactive precordium
  7. Myocarditis
    1. Decreased pulses
    2. Right Ventricular Hypertrophy
    3. Decreased precordium

VI. Causes: Presentation at Weeks 2 to 4 (or later)

  1. General
    1. Onset with drop in pulmonary vascular resistance
    2. Pressure drops enough to allow left to right shunt
  2. Acyanotic (PaO2 >150 on 100% O2)
    1. Aortic Coarctation
      1. Most common CHF cause in first 2-4 weeks
    2. Aortic Stenosis
    3. Myocarditis
    4. Endocardial fibroelastosis
    5. Patent Ductus Arteriosus
    6. Aortopulmonary Window
    7. Arteriovenous Fistula
    8. Ventricular Septal Defect
    9. Atrioventricular Canal Defects
  3. Cyanotic (PaO2 <150 on 100% O2)
    1. Hypoplastic Left Heart Syndrome
    2. Tetralogy of Fallot
    3. Total Anomalous Pulmonary Venous Return
    4. Truncus Arteriosus
    5. Transposition of the Great Vessels and Ventricular Septal Defect
    6. Tricuspid atresia and Ventricular Septal Defect
    7. Single Ventricle

VII. References

  1. Civitarese and Crane (2016) Crit Dec Emerg Med 30(1): 14-23
  2. Joseph and Webb (2015) Crit Dec Emerg Med 29(1): 10-8
  3. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]
  4. Cyran (1998) PREP review lecture, October, Phoenix

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