Action stations, action stations. Set condition one throughout the ship. This is not a drill.
Here’s how it works.
Popcorn. Please turn off cell phones. Dim lights. Previews…and we’re off.
Superman’s having a relaxing day as Clark Kent, lounging around the Daily Planet making moon eyes Lois when arch-nemesis Lex Luthor launches his sinister plot to defeat him and take over the world.
Caught off guard, seemly poised this time to fall to Lex and his evil henchpeople, Superman rises to the occasion, unravels the plot, finishes Lex and crew, and flies off into the sunset with Lois in his arms.
Then the lights come on, we all go out for ice cream, and head home for the rest of the day.
Two or three years later, what happens?
And two or three years later?
Hopefully nothing, because Superman III was dreadful.
This is what we were hoping for. Fix Lex and his evil aortic stenosis and scar tissue henchmen, maybe come back in a few years and to face General Zod and his wicked mitral valve sidekick.
Then hang out for a decade or two and forget we ever knew them.
Unfortunately for us, fixing the aortic valve and the mitral valve will probably not be happening in a sequence of hospital trips separated by years. Instead, it seems they’re going to give us a little Rowdy Roddy and Mr. 1-derful tag team action.
The doctors believe the aortic stenosis was masking serious issues with the mitral valve, and now it’s rearing its ugly, dysfunctional head with a vengeance.
One down. One to go.
After the Ross procedure, we expected Ari to improve steadily, but he progressed slowly and hit a wall. With the mitral stenosis reading now between 8 and 10, and his pulmonary hypertension persisting at systemic levels, the doctors are learning towards short-term surgical intervention.
The medical stuff is getting a little complicated for us to wrap our heads around, but here are some highlights:
- Right ventricle is looking a little better, and has been described to us as moderately dysfunctional. Last week it was severely dysfunctional, and he was visibly struggling: not eating by mouth, throwing up all feeds, losing weight, no smiles. It didn’t look good. Now it looks better.Doctors believe this short-term improvement may be the result of switching a few medications, mostly off propranolol and on digoxin and oxygen.
- Pulmonary hypertension at systemic level. This is bad long-term and will continue to stress his right ventricle, but as far as the doctors know, he’s probably (but not definitely) not in short-term danger. If we can fix it still over the coming months with surgery and treatment, they are optimistic it’ll get better.
- He has a “compliance” issue, which means his heart is having trouble relaxing after it squeezes. This is not new, but it’s still hanging around.
- Mitral valve has a gradient of 8 to 10, and, along with the compliance issue, is likely a significant cause of the pulmonary hypertension. We’ve been told readings of 8 and above are typically surgical, but they’re not a hard-and-fast numbers. There are a lot of complicated factors that play into the surgery / no surgery decision.
Meanwhile, the most likely plan is open heart surgery to repair or replace the mitral valve in the coming weeks.
Looks like we’ll be watching the Olympics here from the hospital. Hopefully not preseason football…
- Keep feeding Ari and see if he can start gaining weight again. A week and a half ago he started losing weight. Weight loss at his age with his condition is a very serious problem and indicative of severely depressed cardiac function. Last two days he’s gained weight (yay!), and we’re keeping his fingers crossed it continues.
- Cath lab early next week to get specific, real-time readings in his heart, and perform tests on its function. During the cath they may try balloon dilation of the mitral valve, but unlike the aortic valve, mitral valves are not often amenable to balloon dilation.
- Assuming no data to indicate a different course of action, mitral valve surgery shortly thereafter.
The mitral valve operation will either be repair or replacement. Neither is perfect option.
Repairs are only sometimes possible. The more work you do on the valve to decrease the stenosis, the more you increase the odds you create leakage. (Well, not you, unless you’re our surgeon. And if you are, put down the bottle and the bong and go look at Ari’s echo or something.)
Leakage is just as bad as stenosis. You don’t want to trade one mitral valve disease for another.
The other option is replacement. At his age, there are two replacement options. The first is a Hancock tissue valve. Comes from a pig. (There goes the bar mitzvah.) They can work fine for a while, but they don’t last long. If we go tissue valve, we’ll be back in the OR to replace it again in as short as a few months, or as long as a year or two. But not longer.
The second is a Melody valve. Comes from a cow. (There goes any chance at nirvana.) The Melody valve is newer and can last for years which would give Ari much more time before we re-replace it. But the Melody is a bigger valve and thus may be more difficult to place in his little annulus (yes, that’s annulus – stop laughing), and because it’s newer there’s little data on its ongoing efficacy and safety.
Either way, it looks like we’re giving Medtronic some money.
Condition one is set. All decks report ready for action.