Total anomalous pulmonary venous connection, also known as total anomalous pulmonary venous drainage and total anomalous pulmonary venous return (TAPVR), is a rare cyanotic congenital heart defect of the pulmonary veins where all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation.
For the infant to live, an atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body.
How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.
Pathophysiology: In normal circulation, blood is sent from the right ventricle to pick up oxygen in the lungs. It then returns through the pulmonary (lung) veins to the left side of the heart, which sends blood out through the aorta and around the body.
In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.
In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.
How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.
- right ventricular heave
- Loud S1
- fixed split S2
- S3 gallop
- systolic ejection murmur at left upper sternal border
- cardiomegaly
- right axis deviation on ECG
- Snowman sign or `figure of 8 configuration` on chest radiograph (see picture above)
- right ventricular hypertrophy
- cyanosis, tachypnea, dyspnea since the overloaded pulmonary circuit can cause pulmonary edema
Treatment: Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.
Prognosis: If this condition is not treated with surgery to repair the problem, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart. Infants who have obstructed veins have worsened survival.
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