Matters of the Heart: Surgery Is Not a Cure
There is no cure for a congenital heart defect (CHD.) Having a CHD is a lifelong condition. You can’t grow out of it. It doesn’t go away with medication or therapy. And it certainly isn’t cured with surgery. But there seems to be a common misconception that once surgery is over, the CHD is gone.
It happens a lot. People find out that Millie has a congenital heart defect for which she had open heart surgery (OHS) and they want to know if she’s okay now. If she’s, “all better.” These are hard questions to answer and make people truly understand.
Yes, Millie is doing very well. She’s thriving in almost every way imaginable. And for that we are so unbelievably grateful. But she is not cured.
The surgery she had at 18 days old repaired her heart defect and made it possible for her heart to function despite its structural defects, but it did not make her heart “normal.” Her mended little heart does not look or function like ours. Her heart had to learn how to work around its new structure to do its job and keep our little heart warrior alive.
Let me explain in more detail. Millie has a heart defect known as total anamolous pulmonary venous return (TAPVR). In Millie’s case, this meant that pulmonary veins, which are supposed to return oxygen-rich blood from the lungs to the left atrium of the heart for distribution to the rest of the body, were not connected correctly.
TAPVR is what is known as a critical congenital heart defect, meaning she would not live without surgery to repair the defect. Her left atrium (in fact, the entire left side of the heart) was underdeveloped so the pulmonary veins had connected to her right atrium, which meant that she wasn’t getting the oxygen she needed. The only thing that likely saved her from needing surgery immediately after birth was a hole between the left and right atriums (an atrial septal defect) that allowed some of the oxygen-rich blood to mix with the oxygen-poor blood to deliver a small amount of oxygen to her body.
There are two surgical methods typically used to correct this defect. The first involves disconnecting the pulmonary veins from the right atrium, moving them and connecting them to the left atrium, where they belong. Because Millie’s left atrium was so underdeveloped, this was not a viable option for her. Instead, her cardiac surgeon proposed removing the existing wall between the two atriums and creating a new wall that would essentially result in the pulmonary veins being connected to a new, larger right atrium.
As you can imagine, this would be a difficult surgery on an adult. Now imagine a surgeon (with fairly large hands, I might add) performing this surgery on a heart roughly the size of a walnut. And keep in mind that open heart surgery performed on an infant is no different than on an adult, except that everything about the person is tiny and more delicate. Millie was put under, an incision was made, her tiny chest was cracked open, her heart was stopped and she was connected to a bypass machine. For hours, this machine kept her alive while her surgeon worked on her tiny, delicate heart.
The slideshow below shows what a normal heart should look like, what Millie’s heart looked like prior to surgery, the proposed repair and what her heart essentially looks like after surgery.
The surgery to repair Millie’s heart defect was successful. But her heart is still not “normal.” The new wall between her right and left atrium is what’s known as a bovine baffle. (That’s right, Millie’s part cow now.) She has regular EKGs and echocardiograms to make sure that her heart function is strong and that her heart and the repair are growing with her.
For a while after surgery she needed medication to manage her blood pressure. Fortunately, her blood pressure is fine now and medication is no longer necessary. But it’s something we check regularly to be sure it stays that way.
Because of where the surgeon needed to work within the structure of Millie’s heart, there was a risk of a heart block. This occurs when something (like surgery or structural changes to the heart) interferes with the electrical impulses that serve as the heart’s natural pacemaker. During her last Holter Study (a 24-hour heart monitoring test) Millie’s heart was still pacing from a different location than a normal heart. As a result, her heart rate was lower than a normal infant’s, ranging from 54-126 beats per minute compared to 80-160 beats per minute. This may or may not correct itself over time and we don’t know the long-term effects. In the meantime, we just have to wait and see what happens.
Of course, the most immediate challenge post-OHS were the feeding issues that kept us in the hospital for almost another month. Millie suffered a paralyzed vocal cord resulting from intubation prior to, during and after surgery. This caused her to aspirate her feedings. We thickened her formula in an attempt to help her feed, but this was so difficult to get out of the bottle she would tire long before getting the nourishment she needed. Ultimately, this resulted in a second surgery to insert a g-tube to help with feeding so she could focus on growing stronger so we could go home.
Millie also has a chronic cough that continues to be concerning. It’s the kind of barky cough that causes perfect strangers to stop and take notice. But after numerous consultations and follow-ups, neither her pediatrician nor cardiologist nor pulmonologist nor ENT can find a reason for the cough. So, here again we just wait and see if anything changes for better or worse.
And let’s not forget that in addition to her CHD, Millie also has a condition known as heterotaxy syndrome (or situs ambiguous). We basically have no idea where most of her internal organs are. Obviously, we know where her heart and lungs are. We know her stomach is on the opposite side as ours. We know she has multiple spleens that are all connected (polysplenia.) We know her liver is midline. We know the surgeon who performed her g-tube surgery couldn’t find her appendix to remove it. But that’s about all we know. It appears they are all connected in a way that works for her, and for that we are grateful. But we have to be very cautious with any abdominal discomfort in the future and she likely won’t be allowed to play any sort of contact sports.
So when you ask me or another heart parent whether their child is fine now and we look a little exasperated, please know that it’s not you. It’s just that life is not that simple or black and white for families living with CHDs. We worry a lot because there is a lot to worry about. More than we ever thought possible.
We may turn you down for a get-together if you or someone in your family is sick – even with just a cold. We may even pull our other children out of school for a few days when there are too many viruses going around to avoid bringing something home to our #HeartWarrior. We can’t come out and play when the air quality is poor or if it’s too hot to breathe easily. We won’t visit your house or let our kids ride in your car if there’s a risk of exposure to secondhand or environmental smoke. Truly, it’s not you. It’s just that these things and so many others are now a matter of life and death for our family.
Two times as many children die from CHDs than all other childhood cancers combined. That’s a shocking, sobering fact. Too often, it’s not the heart itself that causes a hospitalization (or worse) for someone living with a CHD. It’s an infection or some other health issue that developed and put too much stress on the heart. Even after surgery (or surgeries) to repair a CHD, these mended little hearts are fragile. As heart parents, we will do whatever it takes to protect them.
At 20 months old, Millie is doing amazingly well. But she is not cured. We are very cautious when it comes to her health. We make sure she never misses a scheduled follow-up with any of her doctors. We request appointments in between checkups when ANYTHING concerns us, no matter how minor. And we don’t take unnecessary risks.
So let me say it again. Surgery is not a cure for a CHD. There is no cure for a CHD. It is a lifelong condition that requires yearly followup with a cardiologist and other specialists.