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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Tetralogy of Fallot
Macroscopic appearance
  • complex heart defect:
    • infundibular pulmonic stenosis
    • ventricular septal defect
    • dextroposition of the aorta overriding the septal defect
    • right ventricle hypertrophy
  • 75% of affected children have a combination of infundibular and pulmonary valve stenosis
  • pulmonary valve is often abnormal: in most cases bicuspid, with thickened valves
  • pulmonary arteries often show localized stenosis or diffuse hypoplasia
  • the defect of the ventricular septum is often large
Clinical signs
  • the degree of pulmonary obstruction at the infundibulum or secondarily at the pulmonary valve or peripheral arteries is quite variable ranging from mild stenosis to complete atresia and account for the variability of clinical symptoms
  • those with severe pulmonic obstrucion present in the first days of life as the ductus closes with extreme cyanosis and hypoxemia due to right to left shunting
  • in milder stenosis the infants are initially acyanotic and typically have progressive cyanosis in the first 6 months of life
  • the stenotic infundibulum is prone to spasms which acute episodes of extreme cyanosis, dyspnea, convulsions. They often appear during feeding, crying or in the morning.
Classification

Pulmonary atresia with ventricular septal defect is an extreme form of tetralogy of Fallot. There is no flow through the pulmonic valve. The mediastinal portions of the pulmonary arteries may be hypoplastic or atretic as well. As the ductus closes in the first days of life the infant is dependent on blood flow through the aortopulmonary collateral arteries. These usually arise from the thoracic aorta. If the collateral arteries are small the infant is markedly cyanotic.

Syndrom of absence of the pulmonary valve is a rare anomaly associated with tetralogy of Fallot. There is a narrow annulus, no valve and marked poststenotic dilatation of the pulmonary arteries. The dilated arteries compress the bronchi, the affected children present with major respiratory problems. Absent pulmonary valve is associated with Di George syndrome.

Pictures

Pulmonary atresia with ventricular septal defect (extreme variant of the tetralogy of Fallot): Atresia of the pulmonary artery, Macro, autopsy (73169) Atresia of the pulmonary artery, Macro, autopsy (73170) Atresia of the pulmonary artery, Macro, autopsy (73171)

Another case of pulmonary atresia with ventricular septal defect: Atresia of the pulmonary artery, Macro, autopsy (73172) Atresia of the pulmonary artery, Macro, autopsy (73173) Atresia of the pulmonary artery, Macro, autopsy (73174) Atresia of the pulmonary artery, Macro, autopsy (73175)

Tetralogy of Fallot with absent pulmonary valve: Tetralogy of Fallot, Macro, autopsy (73219) Tetralogy of Fallot, Macro, autopsy (73220) Tetralogy of Fallot, Macro, autopsy (73221) Tetralogy of Fallot, Macro, autopsy (73222) Tetralogy of Fallot, Macro, autopsy (73223) Tetralogy of Fallot, Macro, autopsy (73224) Tetralogy of Fallot, Macro, autopsy (73225)

Tetralogy of Fallot, surgery: Tetralogy of Fallot, surgery, Macro, autopsy (73649) Tetralogy of Fallot, surgery, Macro, autopsy (73650)

Tetralogy of Fallot: Tetralogy of Fallot, Macro, autopsy (73651) Tetralogy of Fallot, Macro, autopsy (73652)

Tetralogy of Fallot: Tetralogy of Fallot, Macro, autopsy (73653)

Tetralogy of Fallot, immature newborn: Tetralogy of Fallot, Macro, autopsy (73979) Tetralogy of Fallot, Macro, autopsy (73980) Tetralogy of Fallot, Macro, autopsy (73981) Tetralogy of Fallot, Macro, autopsy (73982)