Anthony's Heart Defects
Although you wouldn’t know it by looking at him (thanks to the amazing care he receives at the Herma Heart Center), inside the body of our Little A beats a very serious and complicated problem. The set of surgeries Anthony needs to allow his heart to function in such a way that his body can grow and thrive are quite new and it simply isn’t known if a heart with Fontan (Anthony’s next surgery) anatomy can last a lifetime.
Anthony’s heart is, in layman’s terms, quite a mess. There has not been a moment in Anthony’s life where his heart has functioned the way a normal heart does, and there is a strong chance it never will. Despite this, Anthony has grown and thrived and become a living testament to the amazing work done at the Herma Heart Center. I am going to try and explain, as I understand them, each of Anthony’s individual heart defects and the surgeries he has had & needs in the future, in the hopes of providing an idea of the depth what the Herma Heart Center has done for Anthony and why we are so passionate about supporting their work.
Anthony’s Main Heart Defects (see Anthony's heart catheterization report):
Transposition of the Great Arteries (TGA)
Anthony’s Aorta and Pulmonary Artery are reversed and connected to the wrong chambers of his heart. This means his blood circulation is reversed as well. Oxygen poor blood circulates through the right side of his heart and is pumped to the aorta instead of the pulmonary artery. This oxygen poor blood is circulated to his body, while the oxygen rich blood circulates through the left side of his heart, bypasses the rest of his body and goes back into his lungs, putting them at risk for failure.
Double Outlet Right Ventricle (DORV)
Anthony’s Aorta and Pulmonary Artery both arise out of his right ventricle. In a normal heart the pulmonary artery arises from the right ventricle and the aorta arises out of the left ventricle. This causes low blood oxygen levels and makes his heart work harder to maintain circulation, putting him at risk for heart failure.
Ventricular Septal Defects (VSD) and Atrial Septal Defects (ASD)
VSD’s and ASD’s are, simply put, holes in the heart. Anthony has many and in his case they are actually somewhat helpful because they allow for the oxygen poor blood and oxygen rich blood to mix in his body, where it wouldn’t be able to otherwise because of the TGA and DORV. The problem is that Anthony has so many holes that it alters the pressure within his heart. His heart wouldn’t be able to withstand the patching of all of the holes because it would so drastically change the pressure his heart functions with. Having all these holes makes his heart work harder, and so puts him at risk for heart failure.
Straddling Mitral Valve
Anthony’s mitral valve functions fairly normally but is connected to his heart in all the wrong places. What this means for Anthony is that repairing Anthony’s TGA or DORV would mean destroying his mitral valve, which is the most crucial valve in the heart.
Anthony’s Heart Surgeries
TGA and DORV are defects that can usually be surgically corrected. The child would still need lifelong follow up, but would be left with a heart that functions normally. Unfortunately, because of the holes in his heart and the Straddling Mistral Valve, Anthony is not a candidate for a complete repair of his defects.
At 5 days old, Anthony underwent his 1st heart surgery, the Pulmonary Artery Banding. This surgery was a “buying time” surgery, restricting the blood flow to his lungs to protect them and allow him to grow big enough to withstand the next set of surgeries.
Anthony requires a series of surgeries that is changing the anatomy of his heart to function despite the defects. These surgeries are the Glenn & Damsel Kaye Stansel, which Anthony had in 8/05, and the Fontan Pathway, which he will have sometime in next 1-2 years. These procedures will leave Anthony with Single Ventricle Anatomy, meaning only one of his two ventricles will be pumping blood that circulates in his body. The good news is this blood will be oxygenated, giving him closer to normal oxygen saturation levels, which should mean higher quality of life. The less good news is that these surgeries leave him very vulnerable to rhythm problems, acquired heart disease, and as mentioned earlier, it is not clear whether a Fontan heart can last a lifetime. However, treatment for Congenital Heart Defects is a rapidly advancing field and we have not stopped believing that a “fixed” heart for Anthony is possible.
Your support of the Herma Heart Center is quite literally helping make our dream of a healed heart for our son a reality!
Sarah Gomez
