D-Day

Official diagnosis: Double Outlet Right Ventricle with remote Ventricular Septal Defect, D-Malposed Great Vessels with Pulmonary Atresia, Bilateral SVC, and a Right Aortic Arch.

This diagram is the closest I could find to our baby's diagnosis. Heart defects are the most common of birth defects, nearly 8 of every 1,000 births. However, our specific combination of defects is extremely rare. The Pediatric Cardiologist didn't have numbers for us and did not have an accurate diagram either. She actually had to draw on the closest one she had to accurately depict his diagnosis. This diagram is missing the Bilateral SVC, the ventricular defect being remote (meaning the aorta is further from the left ventricle than shown), and instead of pulmonary stenosis (narrowing of the aorta), our baby's heart has pulmonary atresia (complete blockage or underdevelopment of the aorta).

To summarize, baby P has 3 major heart defects and 2 minor abnormalities.

The 3 defects are:

1) DORV with remote VSD
2) D-malposed Great Vessels
3) Pulmonary Atresia. 

The 2 minor abnormalities, the right aortic arch and bilateral SVC, were also noted but were stated as more of an anatomical anomaly than an issue and don't necessarily pose a risk of any kind. Hallelujah!

For the curious here is a detailed breakdown:

(d-TGA) Dexter-Transposition of the Great Arteries [transposition=switch the order, great arteries=the aorta & the pulmonary artery, dexter=on the right side] – 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).

(DORV) Double Outlet Right Ventricle [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).

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.

(VSD) Ventricular Septal Defect [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.

Bilateral Superior Vena Cava (b-SVC) [bilateral=both sides, superior=upper, vena cava=large vein] There is an extra upper vena cava on the left side of the body that drains into the right ventricle through an enlarged coronary artery (one of the blood vessels that normally supply the heart muscle with oxygen-rich blood).

(RAA) Right Aortic Arch [aorta=main vessel carrying oxygen-rich blood from the heart to the body] the part of the aorta that leaves the heart, arches to the owner’s right-side , and then turns downward.  (In a normal heart, the aorta arches to the left.)

Thank you to HeartBabyHome.com  for the images and information.