Total anomalous pulmonary venous connection (TAPVC)

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Total anomalous pulmonary venous connection (TAPVC), is a rare cyanotic congenital heart defect (CHD) in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation.
Types :

1. SupraCardiac 

• Most Common 
• Pulm. Veins join Superior Vena Cava

2. Cardiac  

• Pulm. Veins join Coronary sinus

3. InfraCardiac 

• Pulm. Veins join Portal Vein 
• Most severe - because of Obstructive pulmonary venous hypertension.
• Newborn with cyanosis.

All patients have ASD - Very important.
Without ASD there will be pulmonary circuit with no blood for systemic circuit.

MCQ point : O2 Saturation in Pulmonary artery is more than Aorta.

X-Ray features: 
1. SupraCardiac - Snowman Shaped Heart
2. InfraCardiac - Ground Glass appearance with Kerly B lines.

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