Presentation
Newborn with cyanosis.
Patient Data
Venous system:
All 4 pulmonary veins connect to a common vein then connect subdiaphragmatically to
the portal vein. There is narrowing at the end of the connection and dilated portal and hepatic venous system. The veins subsequently connect most likely via ductus venosus, then IVC.
Arteries:
Small PDA noted. Left arch, mild narrowing at the arch with normal branching
Ventricles:
Mildly dilated RV, Normal LV, No VSD noted
All described features are confirmed as total anomalous pulmonary venous return (TAPVR) infradiaphragmatic type 3
Case Discussion
Total anomalous pulmonary venous return (TAPVR) occurs when all pulmonary veins fail to drain into the left atrium. Instead, all pulmonary veins drain anomalously into the systemic venous tributaries or into the right atrium. An interatrial communication (right-to-left shunt), either an atrial septal defect (ASD) or patent foramen ovale (PFO), is necessary for survival.
Depending on the drainage site of the pulmonary veins, the anomaly may be divided into the four types. The current case represents type III (infracardiac or infradiaphragmatic), which accounts for 13% of TAPVR cases. The common pulmonary venous sinus drains to the portal vein, ductus venosus, hepatic vein, or inferior vena cava (IVC). The common pulmonary vein penetrates the diaphragm through the esophageal hiatus. This type is virtually always accompanied by some degree of obstructed venous return.
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