Short Version:
Since the last post a lot has happened! Ross got his implanted cardioverter/defibrillator (ICD) on Thursday and we have answers and next steps after Arthur's procedure on Friday. Arthur has an extra electrical pathway in his heart - this is not terribly uncommon (about 1 in 100 or 1% of people), but his is in the 20% of these that is "hidden" from regular tests. Doctors can only find it with an electrophysiology (EP) study. That's a procedure that uses heart catheterization, so it's not without risk. Generally speaking, with the EP study, when they find an extra pathway, they can "ablate" it (i.e., burn it out with a laser) and it cures the arrhythmia. But since our boys can't be satisfied with common medical issues, Arthur's extra pathway is among the 1% of the 20% that is so close to the main electrical node inside the heart that it can't be ablated during an EP study. That main node manages communication between the brain and the heart, so any damage to that one and Arthur would end up relying on a pacemaker just to be alive. So they didn't do the ablation. The good news is that they also confirmed that his racing heartbeat, while distressing, is not life-threatening. This is great because they can put him on a medication that addresses his heart rhythm and monitor him while we wait for genetic tests to give us next steps. All of it is a big relief, and we left this morning for home!

Long Version:
Ross is now a full-on cyborg with his ICD installed and working. He went in early Thursday morning and was out of surgery and in a room by 1:00pm. They put in a newer type of ICD, known as an "S-ICD", where "S" stands for subcutaneous. Unlike with a traditional ICD which is placed just below the collar bone on the left side above the heart, the S-ICD is placed under the skin against the rib cage about halfway between the waist and the left armpit. The traditional ICD has lead wires that run through the veins and are embedded in the heart muscle tissue itself - this means a doctor can connect a pacemaker or a defibrillator to those wires depending on what a patient needs. They can also place the "double-duty" (that delivers both pacing and defibrillation) device with that arrangement. With an S-ICD, on the other hand, the leads go under the skin across the rib cage on the left-hand side, making a 90 degree turn at the bottom of the sternum. The lead wire then gets placed parallel to the sternum and never enters the chest wall. The electrodes that deliver the life-saving shock, then, are attached very close to the heart, but on the outside of the rib cage.

These are good for people like Ross, because the intravenous ICD does have risks of infection which also may spread to other parts of the body through the bloodstream. With the S-ICD the risk of infection is only at the incision sites and in the skin. But with an S-ICD a patient gets only cardioversion/defibrillation, and currently there is no way to deliver pacing with that device since the electrodes are on the sternum, not in the heart muscle. While we were there at our appointment on Wednesday morning, though, the doctor showed us a very small little titanium device that is in development and, while not commercially available, should hit the market in a few years. That small "medicine capsule sized" device delivers pacing AND communicates with an S-ICD. In other words in a few years, if Ross needs pacing, they will be able to stick this little thing into his heart and he'll pacing that too, with less risk of infection that currently exists for traditional ICD patients. So, things turned out pretty well!

Currently Ross is still quite sore, but doing well and looking forward to telling all of you in a robot voice things like "Take me to your leader" and "Resistance is Futile".

The doctors were able to get Arthur scheduled for an EP study via heart catheterization (cath) for Friday morning. He went in with Erika and as Ross was being released, we were all able to see each other and spend a little time together. They took Arthur back for sedation at about 12 noon. He was all done by 2:00 and spent the night in the hospital just for observation. They released him by 9:00 and we were able to get on the road to get home. 

A heart cath is usually performed under sedation, not anesthesia, with a very thin, flexible tube that they insert into the major vein in your right leg. They push the tube up the vein all the way inside your heart and from there they can do a whole bunch of tests. For the EP study they use a wire with an electrode on the end of it to create conditions inside the heart that will reveal all the electrical pathways you have in your heart tissue. They can find extra pathways, interrupted pathways, and a whole host of issues. If you have an extra pathway, like Arthur does, they can usually use that electrode, or a laser, to "burn out" the extra pathway. But in his case, his extra pathway was too close to other parts of the electrical system of his heart, and it was not possible.

Nevertheless, the EP study gives a lot of information, and in Arthur's case the doctors were able to find a whole lot of news. The best thing is that the racing heart rhythm he is experiencing is not life-threatening. It's a form of supraventricular tachycardia (SVT) which is a racing heartbeat that originates with electrical signals above the ventricles. This is important because if the arrhythmia is ventricular tachycardia (VT), its signals originate in the ventricles themselves, and that IS life-threatening because it can convert to ventricular fibrillation (VF) which means your heart doesn't pump blood at all. Normal heartbeats originate with a signal from the brain to the sinoatrial (SA) node which is a cluster of nerves that function as the "natural pacemaker" of the heart. The SA node "fires", sending the signals from the atria (the upper chambers) to the ventricles (the lower chambers). Under normal circumstances, this functions as one system. In Arthur's case he has an extra electrical pathway in the atria called an "anteroseptal concealed accessory pathway," which means it's a hidden "extra piece" to the system which repeats the signal and creates a "feedback loop". This causes a kind of SVT called "orthodromic reciprocating tachycardia" (ORT) which means the extra pathway creates an extra signal that loops around back to the SA node and sometimes finds its way down to the ventricles causing them to beat at 200-250 beats per minute. He definitely feels it when that happens.

The standard treatment for ORT is an ablation during the EP study, but since Arthur's accessory pathway is so close to the SA node, they could not do that. What they are doing instead is giving him arrhythmia medications (generally very good at controlling SVT), and having a genetic study done to see what other steps we need to take for the future. Once the genetic tests are back, they can decide whether an ablation procedure using cold might be called for. In that one, they would need to put him all the way under a general anesthesia so they can control his breathing, and the movement of his heart. In addition, with burning ablation, they destroy whatever the laser touches, but with a freezing ablation it takes a little time to destroy the tissue and if they are not exactly precise, they can stop it quickly as it's in progress and the heart will heal as long as they have not been freezing too long. This is also good news, because Arthur may eventually need an ICD himself, and if he has ORT, that will likely fool and ICD into giving him a shock when he doesn't need one. This means it's particularly important to resolve this rhythm issue before proceeding with measures to address the "family heart disease." 

All in all, pretty good movement and good timing. Since we got all this done in September, and our benefit year at Ross's work is from Oct. 1 to Sept. 30, we won't have to pay our deductible again to get all these procedures paid for by insurance. That's gonna help a lot! We are so grateful to all of you for the prayers, the good vibes, the positive thoughts, and the well-wishes. We will get through this because we have all of you. May God bless you all.