8th March 2024

Total Anomalous Pulmonary Venous Drainage

Total anomalous pulmonary venous drainage (TAPVD) is a cyanotic heart disease in which all four pulmonary veins fail to drain into the left atrium and instead drain abnormally into the systemic venous circulation via various other pathways. The incidence of TAPVD is between 0.6 to 1.2 per 10,000 live births. Without definitive surgical correction, the mortality rate is approximately 80% by one year of age. 

Signs include right ventricular heave, loud S1 and systolic ejection murmur at the left upper sternal border. The ECG will show right axis deviation and the chest radiograph may reveal a Snowman/figure of 8/cottage loaf sign.

Partial anomalous pulmonary venous drainage (PAPVD) occurs when blood from one or more pulmonary veins return abnormally into the right atrium. PAPVD occurs in approximately 0.7% of the population and is commonly associated with ASD and Turner syndrome. Patients can remain asymptomatic with small defects, whereas symptomatic cyanotic patients would require definitive surgical intervention where any associated heart defects such as ASD, VSD, PFO and PDA are surgically closed. 

The 5 T’s

One way to remember the five topics presented above is using the method presented below:

1. Truncus arteriosus (1 vessel)
2. Transposition of great vessels (2 vessels)
3. Tricuspid atresia (Tri=3)
4. Tetralogy of Fallot (Tetra=4)
5. Total anomalous pulmonary venous drainage (5 letters in the name)