122 – DORV, TGA, TAPVR, heterotaxy

(See Normal Heart Image for comparison)
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supracardiac Total Anomalous Pulmonary Vein Return (TAPVR) [supra=above, cardiac=of the heart, anomalous=not normal, pulmonary=having to do with the lungs, vein=blood vessel, return=come back] a structural defect where the pulmonary veins (which normally carry oxygen-rich blood from the lungs back to the left atrium of the heart) do not connect to the left atrium at all. Instead, the pulmonary veins connect to the innominate Vein (which brings oxygen-depleted blood from the head back to the superior vena cava and then into the right atrium of the heart).  A VSD is the only source of blood for the left side of the heart (which normally supplies oxygen-rich blood to the body).  This causes oxygen-rich and oxygen-depleted blood to mix before being pumped out to the body.

Transposition of the Great Arteries (TGA) [transposition=switch the order, great arteries=the aorta & the pulmonary artery] the positions of the great vessels are switched so that the aorta (which carries oxygen-rich blood to the body) is closer to the oxygen-poor blood AND the pulmonary artery (which carries oxygen-poor blood to the lungs) is closer to the oxygen-rich blood.  It is usually accompanied by a hole in the wall between the ventricles (ventricular septal defect or VSD).

Pulmonary Atresia (pa) [pulmonary=having to do with the lungs, atresia=without openings] a complete blockage of the pulmonary artery (which carries blood from the heart to the lungs) caused by a missing or fused-shut pulmonary valve.

small Tricuspid Valve  (t) [stenosis=narrowing of a passage ]– The valve between the right atrium and the right ventricle is smaller than normal.

Double Outlet Right Ventricle (DORV) [outlet=passage for exit, ventricles=lower chambers of the heart] – both vessels (aorta & pulmonary artery) carrying blood away from the heart come out of the right ventricle. (Normally the aorta carries oxygen-rich blood from the left ventricle and the pulmonary artery carries oxygen-poor blood from the right ventricle).,

Hypoplastic Right Ventricle (HRV) [hypo=under, plasia=formation or development, ventricles=lower chambers of the heart] The right ventricle is under developed. It is too small. This means the heart will have to work harder and won’t last as long. Sometimes the valves entering or leaving the right ventricle are also too small.

Ventricular Septal Defect (VSD) [septum=wall between the chambers of the heart, ventricles=lower chambers of the heart] – holes in the inner walls of the heart allowing extra blood flow between the two lower chambers of the heart (ventricles). This causes the oxygen-rich and oxygen-poor blood to mix before leaving the heart.


The owner of this heart also has:

  • Heterotaxy [hetero=different, taxy=forming]:  abnormal position of the organs inside the body. Look here for more information: http://www.chop.edu/service/cardiac-center/heart-conditions/heterotaxy-syndrome.html
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