Ventricular Septal Defect

Aka: Ventricular Septal Defect, VSD

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II. Epidemiology

  1. Incidence: 15-25% of Congenital Heart Disease
    1. Most common Congenital Heart Disease cause
    2. Most common CHD found in chromosomal abnormalities
  2. Gender
    1. More common in males

III. Pathophysiology

  1. Defect in interventricular septal wall
    1. Most often located in membranous ventricular septum
  2. Physiology of VSD changes with time after birth
    1. Newborn initially has a right to left shunt due to increased pulmonary pressures
    2. By 6 months of age, pulmonary pressures decrease, and shunt is left to right
    3. Longterm large VSD leads to Eisenmenger Syndrome with right to left shunt
      1. Results from chronic pulmonary overload with Pulmonary Hypertension

IV. Signs and symptoms

  1. Severity of symptoms related to:
    1. Defect size
    2. Pulmonary vascular resistance
    3. Associated cardiac lesions
  2. Small to moderate VSD
    1. Normal P2 component of the Second Heart Sound
    2. Pansystolic harsh murmur
      1. Grade II-VI of VI
      2. Located at lower left sternal border
  3. Large VSD with significant shunt
    1. Includes moderate VSD findings
    2. Mid-diastolic flow rumble at apex
    3. Congestive Heart Failure signs and symptoms in a pink child (not cyanotic)
  4. Marked Pulmonary Hypertension
    1. Right Ventricular lift
    2. Loud P2 component of the Second Heart Sound
    3. Short systolic ejection murmur at left sternal border

V. Imaging (large defect findings)

  1. Chest XRay
    1. Cardiomegaly
    2. Increased pulmonary vasculature
    3. Lungs appear dark in Pulmonary Hypertension (contrast with white of Pulmonary Edema)
  2. Echocardiogram
    1. Defines position and size of defect

VI. Management: Medical

  1. Small Ventricular Septal Defect
    1. No surgical repair indicated
    2. SBE Prophylaxis
  2. Congestive Heart Failure
    1. See Pediatric Congestive Heart Failure
    2. Supplemental Oxygen
      1. May worsen Pulmonary Hypertension (increasing left to right shunt)
    3. Positive Pressure Ventilation (BiPAP)
    4. Diuretics (e.g. Furosemide 1 mg/kg IV)
    5. Consider Nitroglycerin in flash Pulmonary Edema (Cardiogenic Shock)
      1. However, may worsen left to right shunt
    6. Consider inotropes (Dobutamine or Milrinone)
      1. Indicated in Cardiogenic Shock with Pulmonary Edema (cold and wet)
      2. Consider Epinephrine if hypotensive
    7. Consider Digoxin

VII. Management: Surgical repair indications

  1. Growth failure refractory to medical therapy
  2. Pulmonary Hypertension
  3. Pulmonary to systemic flow ratio > 2:1

VIII. Course

  1. Spontaneous closure age <6 months: 30-40% related to:
    1. Membranous and muscular defects
    2. Smaller defects

IX. Complications: Without repair

  1. Congestive Heart Failure
  2. Eisenmenger's Complex

X. Complications: With repair

  1. Conduction defect: transient Right Bundle Branch Block

XI. References

  1. Claudius and Strobel (2024) EM:Rap, 9/9/2024
  2. Cyran (1998) PREP review lecture, October, Phoenix
  3. Merenstein (1994) Pediatrics, Lange
  4. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]

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