17 Pulmonary Atresia with Intact Ventricular Septum

• Extreme form of pulmonary valve stenosis with imperforate valve resulting in complete obstruction to right ventricular outflow
  
• Not a simple isolated lesion but considered part of the more complex hypoplastic right heart anomalies
  
• Tricuspid valve apparatus is almost always abnormal and often hypoplastic.
  
• Classification relates to a spectrum of right ventricular dimensions (Fig. 17.1):
  
  
  
  • • Classic hypertrophic form: small hypertrophied right ventricle with small, not severely incompetent tricuspid valve, often associated with fistulous communications between the right ventricle and the coronary arterial systems
    
  • • Dilated form: large right ventricle with large and incompetent tricuspid valve, typically with Ebstein malformation or dysplasia of the leaflets

Fig. 17.1 Classification of pulmonary atresia. PA, pulmonary artery; RA, right atrium; RV, right ventricle.

Pathophysiology

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