CASE 44

Clinical Presentation

A newborn infant developed severe respiratory distress and cyanosis immediately after birth.

Figure 44A

Radiologic Findings

A frontal chest radiograph (Fig. 44A) obtained immediately after birth demonstrates a markedly enlarged heart. The right atrial border extends to the upper mediastinum and bulges far to the right. The pulmonary vascularity is slightly prominent.

Figure 44B

Figure 44C Frontal chest radiograph of a newborn with pulmonary atresia and intact ventricular septum shows a markedly enlarged heart with an egg-on-side appearance. The right atrial contour is prominent, and the pulmonary vascularity is reduced.

Figure 44D Frontal chest radiograph of a newborn with heart block shows severe cardiomegaly and severe pulmonary edema.

Diagnosis

Ebstein’s malformation of the tricuspid valve (Fig. 44B) with patent ductus arteriosus

Differential Diagnosis

Severe cardiomegaly in the newborn:

• Ebstein’s malformation*
  
• Unguarded tricuspid valve*
  
• Uhl’s anomaly: absence of the myocardium of the right ventricle*
  
• Pulmonary atresia with intact ventricular septum* (Fig. 44C)
  
• Congenital heart block** (Fig. 44D)
  
• Congenital aortic insufficiency; aorto-left ventricular tunnel**
  
• Cardiac tumor
  
• Massive pericardial effusion

Discussion

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