Total Anomalous Pulmonary Venous Return to the Coronary Sinus (TAPVR to CS)

Overview and natural history

Total anomalous pulmonary venous return (TAPVR) is a rare congenital malformation in which all four pulmonary veins do not connect normally to the left atrium. Instead, the four pulmonary veins drain abnormally to the right atrium by way of an anomalous venous connection. There are four main types: supracardiac, intracardiac, infracardiac or mixed venous drainage.

Total anomalous pulmonary venous return to the coronary sinus (TAPVR to CS) is a form of intracardiac total anomalous pulmonary venous return. In TAPVR to CS, there is an anomalous connection of the pulmonary veins with abnormal pulmonary venous return with all four of the pulmonary veins returning to a pulmonary venous confluence, which in turn connects to the coronary sinus which subsequently drains into the right atrium. 







Goals of echocardiography exam

  • Determine size and location of each individual pulmonary vein
  • Determine location of pulmonary confluence
  • Determine course of the pulmonary venous channel and it’s site of termination
  • Determine level(s) and severity of obstruction
  • Evaluate interatrial communication
  • Evaluate of other anatomic and function abnormalities
  • Assess for right heart dilation
Lai W, Mertens L, Cohen M, Geva T. Echocardiography in pediatric and congential heart disease from fetus to adult. 2009