CASE 45

Clinical Presentation

A term newborn developed severe hypoxemia, cyanosis, and tachypnea immediately after birth. The obstetric history was unremarkable.

Figure 45A

Radiologic Findings

A frontal chest radiograph (Fig. 45A) shows moderately hyperinflated lungs with generalized increase in interstitial lung markings. The heart is normal in size and configuration.

Figure 45B Injection into the main pulmonary artery at cardiac catheterization shows that all pulmonary veins make a confluence at a vertical vein that connects to the portal vein in the liver.

Figure 45C Plain chest radiograph, of a patient with hypoplastic left heart syndrome and restrictive atrial communication, shows generalized increase in interstitial lung markings and air trapping as in Fig. 45A.

Diagnosis

Total anomalous pulmonary venous connection (TAVPC) to the portal vein (Fig. 45B)

Differential Diagnosis

• Obstructive type of TAVPC; most commonly seen with anomalous connection to the portal vein
  
• Stenosis of the individual pulmonary veins
  
• Hypoplastic left heart syndrome with restrictive atrial communication (Fig. 45C)
  
• Congenital lymphangiectasia
  
• Transient respiratory distress of the newborn
  
• Interstitial viral pneumonia
  
• Hyaline membrane disease in the term infant with surfactant deficiency

Discussion

Clinical Findings

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