Total anomalous pulmonary venous return (tapvr)


  • In total anomalous pulmonary venous return, the pulmonary veins, blood vessels that normally carry oxygenated blood from the lungs to the left side of the heart, connect instead to the right side of the heart. This blood then flows to the left heart through a hole in the wall separating the left and right heart chambers.

    Children with a mild form of total anomalous pulmonary venous return may have few symptoms.
    Children with more severe total anomalous pulmonary venous return may have bluish discoloration of the skin (cyanosis), shortness of breath and fatigue.
    Echocardiography is needed for diagnosis
    Surgical repair is needed

    (See also Overview of Heart Defects.)
    Total anomalous pulmonary venous return (TAPVR) accounts for 1 to 2% of birth defects of the heart.
    Normally, the pulmonary veins return blood that has picked up oxygen in the lungs to the left atrium. This oxygenated blood then flows from the left atrium into the left ventricle, where it is pumped to the body. (see also Normal Fetal Circulation) In infants born with total anomalous pulmonary venous return, the pulmonary veins do not connect normally to the left atrium and connect instead to the right atrium. So the right atrium, which usually receives only deoxygenated blood from the body to be pumped to the lungs, receives a mix of oxygenated and deoxygenated blood. The additional blood causes the heart to have to work harder than normal. Even more importantly, flow from the pulmonary veins makes its way to the right atrium through various pathways, going up above the heart, down below the heart, or around the back wall of the heart. These pathways can be narrow or blocked, causing blood to back up in the lungs and pressure to build up in the lungs, preventing their normal function. Blood only flows to the left side of the heart through a hole between the right and left atria (a patent foramen ovale or atrial septal defect).


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