About Olivia's Procedures

This is a post for the info-minded. And it's not short.

Many have asked what kind of surgery Olivia had, what makes it different from the next surgery and how it all works. While my understanding is still limited, I'll try to point you in the right direction.

First, here's a normal heart, followed by a Tetralogy of Fallot heart, which is what Olivia has been dealing with.



Olivia has a heart defect called Tetralogy of Fallot (rhymes with shallow, with the emphasis on the last syllable). Also see here and here and here for great info on TOF. “Tetra” means four, as in the popular game Tetris in which four squares are arranged in shapes that must be fit together in rows to gain points. Tetralogy, then, is the study of a series of four heart defects categorized by a frenchman named Etienne Fallot around 1888.

As some of you have noticed with Olivia, infants and children with tetralogy of Fallot usually have blue-tinged skin (cyanosis), lips, nail beds and any other part of the body where blood flow is close to the surface of the skin. It is more pronounced especially during times of exertion. As you can see from the picture, Olivia had a ventricular septal defect (VSD), which is a hole in the wall that separates the left and right ventricles. This hole can be small or large, but Olivia's was quite large; more than three-quarters of the entire span of her heart, which made it impossible to repair the hole. [Click here for some key points about TOF.]

As seen in the last link, a week after Olivia was born, she had a Blalock-Taussig shunt (BT shunt) put in. It's an artificial Gortex™ artery intended to put more oxygen-rich blood into her heart to be pumped out through her body. The problem is, with that VSD, once the oxygen-rich blood was pumped into her heart, it mixed with the blood coming back from the body that had already delivered its oxygen payload ... oxygen-rich blood + oxygen-depleted blood = oxygen-diluted blood. She wasn't getting enough oxygenated blood pumping throughout her body, which brings us to the current surgery. The BT shunt was just buying some time until her body could handle some major open-heart surgery.


The surgery she just had is called a Glenn and the next one will be a complete Fontan repair, which is the final step in this two-step process.

From the Mayo Clinic site:
“Often, children with [TOF] require more than one surgery. The goal of these surgeries is to create sufficient blood flow into and out of the heart and lungs, allowing your baby's body to receive the proper amount of oxygen-rich blood.”

“The first step, sometimes called a bi-directional Glenn or hemi-Fontan, is typically performed around four to six months of age. It reduces the work of the right ventricle by allowing it to pump blood mainly to the aorta and allowing most of the blood returning from the body to flow directly into the lungs. After this operation, all the blood returning from the upper body is sent to the lungs, so blood with more oxygen is pumped to the aorta to supply organs and tissues throughout the body.” And another paragraph: “The Glenn procedure: When babies have outgrown the [BT] shunt, they often require a surgery that sets the stage for the Fontan procedure. Doctors usually perform the Glenn procedure when a child is about 6 months old. It connects one of the large veins that return blood to the heart (superior vena cava) to the pulmonary artery. This allows oxygen-poor blood to flow directly to the lungs. The procedure reduces the workload on the left ventricle, decreasing the risk of damage to it.”

“The second step, called the Fontan procedure, is done between 18 months and 3 years of age, [but they are planning on doing this between ages 2-1/2 and 3-1/2 in Olivia]. It allows the rest of the blood coming back from the body to go to the lungs. After this procedure, there's no mixing of oxygen-rich and oxygen-poor blood in the heart, so [Olivia's] skin will no longer look blue.” And another paragraph: “A surgery called the Fontan procedure is the best option for treatment ... During a Fontan surgery, the surgeon creates a path for the oxygen-poor blood returning to the heart to flow directly into the pulmonary arteries, which then pump the blood into the lungs.”

Summaries are supposed to be shorter, I know, but hopefully this better explains what she's going through.

Thanks for all the prayers and calls. The surgical team was outstanding and Olivia is doing well.