Derek here. Normally, my role on Andrea's blog posts is that of editor and occasional target of snark. However, she's a bit indisposed at the moment, and there are a few people interested in getting the latest information on the situation, so here I am. 

On Saturday night, I was asking her what she expected of me in the way of posts. Most importantly, she didn't want me stealing her thunder. "Just make sure you leave something for me to tell." There were a few Facebook posts yesterday, along with a brief Caring Bridge blog that she wrote while I slept for a couple of hours on the futon in her ICU room, but for the most part, I'm not oversharing.

In that spirit, in this post I won't be telling any colorful anecdotes. There will be no clever puns, no similes or metaphors. And you should definitely not expect any inspirational wisdom. This post will be as dry as....something really, really dry. To quote Joe Friday from Dragnet, "Just the facts, ma'am." (Kids under the age of 45, ask someone old.)

Andrea received a phone call around 12:30 PM on Saturday. She spoke to the transplant coordinator for a few minutes, and got some bare-bones information about the donor: the person was "young" and not "high-risk." This latter designation refers to people who may engage in IV drug use or other behaviors that may expose them to serious illnesses. At any rate, that's all we knew, and made the determination to go forward with the transplant early on Sunday morning.

We did some things around the house for a couple of hours to prepare, then jumped in the car. In an odd twist, Katy Perry...

[paranoia-inducing story about iPod's shuffle mode stricken from the record]

We checked into the hospital a few hours later, and...

[colorful anecdotes about a nutty evening in the cardiac ICU omitted for now]

...so much sushi...

[funny story about weird coincidences removed for discretion]

...what are the odds we would have been at Duke...

[Just get on with it already. Nobody's laughing. This isn't supposed to be funny.]

Fine. Whatever.

At 6 AM on Sunday morning, the anesthesiologist wheeled Andrea out of the ICU and onto the service elevator, taking her to the OR. And then we didn't hear very much for a bit. This was, in part, because of our decision not to wait in the designated family waiting room. Instead, we headed down to the cafeteria for some breakfast. I had a couple of scoops of scrambled eggs...

[OMG, no anecdotes!]

Right.

After about 3 hours without having received a call on my cell phone with status updates, Andrea's father (Larry) and I headed back to the ICU to see if we could learn anything. It seems they had been trying to contact us, but were calling the waiting room and that's not where we were. They took down my cell number (again) and maybe an hour, had one of the operating room team nurses give me a call with information. Things were going as planned, they had received the donor heart, she was on the bypass machine, and her heart had been removed. 

Another hour after that, we received news that the new heart was in and was pumping normally. The procedure then is to let the new heart rest a bit before closing the patient back up. After a couple more hours, they closed her up, and they wheeled her back up to the ICU. 

At this point (around 2 PM), I was allowed to see her. She was intubated (had a breathing tube inserted into her throat), covered with wires and tubes, and had puffy, air-filled "boots" on to keep her ankles at an angle that would be more comfortable. And, of course, she was completely unconscious.

Over the course of the next two hours, she would come to wake up a little bit. The staff brought her off the medication so that they could conduct neurological tests, checking whether she would respond to verbal commands and demonstrate a pupillary response when a flashlight was shone in her eyes. She made eye contact with me and was responding to my voice. It was clear that she knew what was going on, even if she was still very groggy. She was squeezing my hand and could wiggle her toes.

Everything was going perfectly...and then not so much.

Wanna learn some anatomy? Ok, good!

The mammalian heart has four chambers, with paired right and left atria (singular atrium) and ventricles. The left side of the heart receives oxygenated blood from the lungs and then distributes it around the body via the aorta and the smaller arteries branching off of it. The right side of the heart receives deoxygenated blood via the vena cava, and then pumps that blood to the lungs to get oxygenated again.

Andrea's underlying disease, the whole reason she's in the hospital at all, is a condition called left ventricular noncompaction cardiomyopathy (LVNC). This is a congenital disease that manifests during fetal development. It is a very rare disease, but also very challenging to diagnose properly, as only an echocardiogram will show it in a living person. In one study, the rate was estimated at 1.2 cases in a million.

Andrea was diagnosed in her mid-30s, and has since been on drugs to prevent clotting and improve her cardiac output, and she received an implanted defibrillator to shock her in the event of significant arrhythmias. (N.B., The device was removed yesterday, along with her old heart, but she was shocked on two different occasions. It likely saved her life at least once.)

In people with LVNC, the left ventricle has atypically developed trabeculae inside the heart chamber. Trabeculae are normally the shallow muscular "ridges" inside the heart. This disease impacts the formation of them, leaving the person with much deeper invaginations. This greatly increases the risk of stroke, allowing blood to pool inside the heart.

This different anatomy impacts the efficiency of the heart. Heart output is generally measured with an ejection fraction (EF). This is expressed as a percentage of blood that the heart pumps out with every beat. A normal, healthy heart might pump 50-70% of its blood out with every beat. In Andrea's case, her EF was down in the 20-25% range. As the heart has to work harder and harder to accommodate the lower efficiency, the muscular walls of the heart thicken, making it more rigid and further decreasing efficiency and increasing the likelihood of valve disorders and arrhythmias, both of which Andrea also developed.

So this is where we were leading into the transplant yesterday morning.

The new heart is, by all appearances, in very good shape, and undoubtedly way better than what Andrea was dealing with before. But cardiac transplantation is of course a complicated and tricky procedure. Up through 4 PM yesterday, again, everything was looking great. Then we started to see some decreased performance of the right side of her heart. Again, this is the part of the heart responsible for pumping blood to the lungs. The staff did some imaging of Andrea's new heart and concluded that the best option was to take her back to the operating room, re-open her chest, and take a better look at what was going on. 

It's a good thing they did.

The location where a blood vessel is sewn to another blood vessel in an operation is called an anastomosis. It seems that one of the vessels between Andrea's heart and lungs had become kinked; there was too much slack in it and it managed to get a slight bend in it. That inhibited blood flow a bit, and it started to back up into her heart and her circulation grew less efficient. The surgeons were able to remove the slack from the anastomosis and get everything closed back up. Her circulatory vitals immediately showed improvement. I got the call around 11:30 PM last night that things looked good.

Some time around mid-morning, her right ventricle (RV) function started to decline again. I suppose it is worth interjecting here that just about every heart transplant patient has some degree of RV dysfunction. Along with organ rejection, it is the most frequent issue that they have to contend with. One of the reasons this occurs is myocardial stunning. The term myocardial refers to the heart muscle. Stunning is just exactly what it sounds like - the muscle gets stunned due to the trauma of the operation. This is a temporary, reversible condition, and generally can be managed with medication. The right ventricle is especially susceptible to it because it is smaller and less muscular than the left ventricle.

So what we're dealing with today is a fair bit of impairment of the right side of her heart, perhaps in part caused by stunning, and also possibly impacted by the amount of fluid she is retaining in her body as a consequence of the procedure and all the medicines that she is getting pumped into her. She has been unconscious most of the day, but has been brought out of it a couple of times.  When she's awake (but still very groggy), she is responding to our voices, and doesn't seem to be in too much pain. They are managing her heart function with diuretic drugs and inotropes to improve muscular function.

And that's pretty much where we are. If all goes well, the breathing tube might be coming out tomorrow.