Anomalous pulmonary vein connection, total (TAPVC) or partial (PAPVC)

Definition und Indication

Total and partial anomalous pulmonary vein connections are distinguished. In both, the connection of the pulmonary veins is abormal, either to a systemic vein or directly into the right atrium. In partial anomalous pulmonary vein connection only some of the pulmonary veins show anomalous connection; the rest are normal. The occurrence of symptoms depends on the extent of the left-right shunt. In the case of right ventricular dilatation the indication for an operation is given. With total anomalous connection (TAPVC) symptoms occur early due to the considerable left-right shunt. If the atrial septal defect is too small (restrictive) or the outflow is obstructed (often in the infracardiac type), an emergency corrective operation may be necessary directly after the birth.

Diagnosis and types

Echocardiographic visualization of the anomalously connected pulmonary veins

  • supracardiac (connection above in heart, in approx.. 40%): connection to the anonymous vein (left), superior caval vein or azygos vein (right)
  • cardiac (connection to the atrium, in approx. 25%): connection to the coronary sinus, right atrium
  • infracardiac (connection below the heart, in approx.. 25%): connection to the lower caval vein after passage through the diaphragm into the portal vein or hepatic veins (second capillary area, therefore often obstructed). Special case: Scimitar syndrome: connectio nof the right-sided pulmonary veins to the inferior caval vein; then often associated with hypoplasia of the right lung.
  • mixed (in approx. 10%): heart catheterization. Obsolete for obstructive/total APVC. Indication for heart catheterization only in mixed type and when echocardiographic findings are unclear.