Hypoplastic Right Heart Syndrome is the general diagnosis that Sunley has. It's an umbrella term, which can include several different combinations of heart defects, which means that kids with the "same" conditions can have very different cardiac anatomies, with very different surgery plans, and very different outcomes. Sunley was born with a few specific heart defects:

Transposed Greater Arteries - The aorta and pulmonary arteries usually cross. In a healthy heart, the PA rises out of the right ventricle and carries blood to the lungs, while the Aorta rises from the left ventricle, carrying blood to the rest of the body (arms, legs, etc). Sunley's Aorta and Pulmonary Arteries do not cross. While the heart is forming, which is around 5 weeks gestation (usually before mom even knows she's pregnant!), one of the first things that happens is the heart "folds" on itself. This is when the arteries cross, or in our case, don't cross. A few doctors suspect that if Sunley's arteries had fully crossed, the rest of her heart may have developed perfectly. And they almost did; they overlap, but just don't fully cross. I will always wonder if there was something environmental that caused this. Because heart defects happen so early on, there is VERY little data pointing to the underlying causes.

Sunley also has hypoplasia (underdevelopment) of the right ventricle -- Actually, she has NO right ventricle at all. Both arteries feed out of and into the left ventricle, which is extremely rare. This is called Double Outlet Left Ventricle and Double Inlet Left Ventricle. There was some concern fetally that she had some issues with her mitral valve, which connects to her only ventricle, but that has remained mild enough to be left alone.

All of this is "umbrella'ed" under the term Hypoplastic Right Heart Syndrome. When you Google that, the drawings look nothing like Sunley's heart because you can obviously have so many different combinations of defects that are still categorized as HRHS.

We were very blessed that Sunley's arteries developed well -- there was no stenosis or atresia, which meant that she did not need the Norwood procedure at birth. Instead, Sunley's open heart surgeries included a PA Banding surgery (MUCH less invasive than the Norwood) and the Bi-Directional Glenn with a DKS procedure.

Part 2