PVL Closure

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Dana

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Sep 27, 2011
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Westwood MA
Anyone had a paravalvular leak (PVL) repaired?

After complaining of anemia, SOB on exertion, and hemolysis, a TEE was performed and a significant leak around the outside of my mechanical mitral valve was found allowing a jet of blood to gush back up into the left atrium resulting in inefficient pumping action among other things. This seems likely to be causing most if not all of the hemolysis, which causes anemia, which causes reduced hemoglobin, which causes shortness of breath and cascades into a host of other symptoms as well, most insidiously, difficulty sleeping. During mitral valve replacement, evidently, a lot of calcification prevented the surgeon from completely securing the valve's sewing skirt to the tissue substrate. The leak may have been there ever since the OHS because, while improving a lot, I never quite reached my previous exercise tolerance after the surgery six years ago. And over the last two years symptoms have been getting progressively worse. We've managed to keep the anemia somewhat under control with weekly or biweekly Aranesp injections, which stimulate RBC production in the bone marrow, hence improved hemoglobin and hematocrit. But heart failure symptoms are occurring as a result of the inefficient pumping in spite of my normal blood pressure, normal weight, normal blood sugar, good diet and regular exercise, although my exercise performance has diminished correspondingly.

Because of the leak's size and location, the interventional cardiologist thinks there is a good 70% chance the leak can be repaired percutaneously (using a catheter through a vein). I'm not up for another OHS at 71. He will guide and release a plug into the hole where the plug expands to fill the void. It's a compression fit, and my hope is that it won't pop like a champagne cork under the pressure from the left ventricle. I asked the doctor if that ever happens and he says, he's seen it once in a patient (not one he had repaired) where the plug had loosened, traveled and ended up having to be dislodged from a vein in the patient's leg.

Conceptually what these guys do is not much different than what a plumber would do except the tools and techniques are so much more complicated.

The PVL closure is schedule for Sept 25, 2023. Just wondering if anyone can share positive outcomes.
 
I had the leak closed on Sept 25, 2023 and it was none too soon as I was even waking up short of breath at night. It took three valvular plugs of 12mm, 8mm, and 6mm in diameter to diminish the leak from 'borderline severe to trace'. Do I feel better? A little, but there was about over three pints of blood loss during the procedure and blood counts ended up being lower than they have ever been. Going in my hematocrit and hemoglobin were 35 and 11.4 respectively due to the Aranesp injections I've been getting. After the procedure the hematocrit and hemoglobin were 25 and 8.8; ouch! So my first lap around the floor was disappointing. But if the leak was responsible for most if not all of the hemolysis, then I should start to rebuild those blood counts and feel better. They transfused one pint (could have easily used three) before sending me home.

Now just trying to keep the vein puncture in groin closed. My previous catheterization left a hole in the jugular vein and it would not stay closed. There's a funny (now that it's over) story about that time which I'll share for some levity. Woke up to go to bathroom. Saw in mirror that my white tee shirt was completely red. Should I try to change the dressing myself? They told me to go to ER if it started leaking. Went back to the bedroom and saw that the pillowcase and bed were soaked in blood. I tried to change the pillow case but with one hand holding pressure on the wound, couldn't accomplish that so I threw it on floor and got a new pillow and got back in bed to think about what to do. I woke up my wife, Sue to drive me to ER because that's what they said to do in that situation. She said, let me see. I showed her the wound leaking and she said, okay let's go. She got up walked around the bed saw the pillow, all the blood and passed out completely unresponsive! Not knowing why, I got out of bed after a struggle. (With one hand compressing the wound with my bloody tee-shirt and with her body pinning the covers to the floor I was trapped for a while.) I checked Sue's pulse but saw she was breathing. A stroke I thought. But I managed to rouse her and got her to repeat a sentence clearly, so no stroke. So I told Siri to call 911 and after explaining our situation to the operator and giving the code to our house door lock, she sent two ambulances, a fire truck, police car, four EMTs, firemen and cops all with their flashing lights illuminating the whole neighborhood at 3:30 AM. At least they kept the sirens quiet. You know the 911 operator keeps you on the phone with question after question, so in a few minutes, there were four EMTs in our bedroom who I asked to help me get my pants on. Sue felt she was okay so refused a perfectly good ambulance while I headed off to the nearest emergency room, which was at a closer hospital than the one that did the procedure. So when Sue awoke my daughter who lives nearby and they drove in together to pick me up, they didn't know to which ER I was taken. Sue had to call 911 again and ask where they brought me. At the ER they just put in a couple more stitches and sent me home.

So that's why I need to keep this new vein puncture closed.
 
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there was about over three pints of blood loss during the procedure and blood counts ended up being lower than they have ever been. Going in my hematocrit and hemoglobin were 35 and 11.4 respectively due to the Aranesp injections I've been getting. After the procedure the hematocrit and hemoglobin were 25 and 8.8; ouch!
Thanks for the story. Hope you recover well. Would be great to know how well the plugs work out.

I also a significant blood loss during the surgery. Many blood parameters got normalized quickly, within a couple of days. The one outlier was read blood cells. (It seemed they didn't want to give it along with the blood transfusion, due to some kind of complications.) The attending physician thought it would take a month or two to get them back. Maybe that's a typical time scale. Would imagine the exercise capacity might be reduced until you get them back.

Sorry to hear about the midnight ER adventure. Glad it ended up well in the end!
 
Over time there have been demand for better flowing prosthetic heart valves. How do you do that? Make the cuff thinner thus allowing the "inside" of the valve to grow. This makes sealing (paravalvular leak) the valve all that much harder. Then along comes TAVI with the potential for leakage-city as there is not a sewing cuff as "usual".

Moral of the story: find a valve with a big fluffy cuff - your surgeon will be much happier - as well with you. I (editorial) would sacrifice EOA (effective orifice area) for a better sealing valve.
 
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I hope your procedure works and the hemolysis goes away. Thanks for the funny story!
I just saw my hematologist again and he’s taking me off of Trental. It’s not worked as he had hoped for my hemolytic anemia.
He still wants me to take the folic acid.
I forgot to ask about the aranesp but I could email him about it. My hemoglobin is 9.1, now.
My latest visit with cardiologist left me w 2 referrals. One for an interventional cardiologist (mitralclip), the other for the thoracic surgeon for robotic surgery. They’re in the same office so I see them on same day. It’s to consult to see my options.
All the best for your outcome!
 
Gail, we have an online portal where patients can request appointments, see test results, view visit notes and compose/receive messages to/from our doctors. I use it quite frequently probably to the annoyance of the doctors but if I have something I would like to ask I don't wait for the next appointment, I just fire off a message. They always respond in a day or so. We as patients need to self-advocate.
If you have such a online portal, or even if not, call or email your doctor asking the question about Aranesp. They set my threshold at 11.3 for hemoglobin. Under that allows me to receive an injection. So by that measure you easily qualify. It a costly (around $700 I think) drug but I'm on medicare and they cover it when it's prescribed by doctor for the right reason. I believe the drug was originally designed for chronic kidney disease not hemolytic anemia. Medicare initially balked because the diagnosis of hemolytic anemia didn't fit their coverage criteria but because I wan't told that up front, I am not responsible, the hospital is. If your diagnosis is kidney disease, Medicare covers it and your insurance should as well. I mentioned this to my hematologist and she corrected the diagnosis because kidney disease does accompany hemolytic anemia, mild at first but will get more severe if hemolysis is not checked. The free hemoglobin that is released by sheared red blood cells is the culprit and it has a deleterious oxidative activity on kidneys. Renal impairment is a known outcome of hemolytic anemia. Your body does have a backup plan, though. It's a protein called haptoglobin and is produced by the liver. Haptoglobin has a high affinity for free hemoglobin and it binds it up for excretion like a mop. However the liver can only produce so much haptoglobin and when it runs out, kidneys are vulnerable and damage can ensue. There is a blood test for haptoglobin that your hematologist may have already ordered for you. Normal haptoglobin levels are for me (adult male) 32-197 mg/dL. The blood test cannot detect haptoglobin below a certain threshold like 5 or 10 mg/dL. It will just report the level as < 5 mg/dL meaning it wasn't able to detect any haptoglobin. That's what my tests show. An unmeasurable level of haptoglobin means it's all used up and there's a risk of renal impairment. Of course blood checks of the kidney function like creatinine, EGFR (globular filtration rate), or a urine test checking for albumin in the urine are indicators of issues with the kidneys.
Anyway, be your own advocate unless you have someone who can do it for you. I have been a patient advocate for my father (cardiology and renal cell carcinoma), and five different other people, all women with breast cancer including my mother, sister and sister-in-law accompanying them to all their appointments. I'm just an engineer but I do research and ask questions. It helps patients get a better understanding of what their doctors are saying and sometime prods the doctors into doing some thinking rather than the cook-book approach often seen because, I think, of fear of being sued if they do anything outside the realm of the AMA's "standard-of-care". I expected doctors to be problem solvers. They are not. Instead they follow rules for treatment.

I take it you have mitral valve regurgitation. I watched a YouTube video on the MitraClip procedure. Amazing. That's exactly how they deployed my vascular plugs. And on the video, of course, it looks like it would work great. I hope it does if that's what you choose to do. Best of luck.
 
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Thanks. I do messsge my Doctors through the Cedar-Sinai app. They always quickly respond.
It will be interesting to find out if I have options going forward. I’m not sold on mitraclip. There’s only a decade on it.
 
Having had a thru the vein valve repair (valvuloplasty not mitraclip) the decision making process was would it be safe to try it before proceeding to ohs if needed. We decided yes and that repair has lasted 21 years so far. Still have native valve and no ohs which I feel very lucky that the valvuloplasty was an option for me.
 
Thanks for the story. Hope you recover well. Would be great to know how well the plugs work out.

I also a significant blood loss during the surgery. Many blood parameters got normalized quickly, within a couple of days. The one outlier was read blood cells. (It seemed they didn't want to give it along with the blood transfusion, due to some kind of complications.) The attending physician thought it would take a month or two to get them back. Maybe that's a typical time scale. Would imagine the exercise capacity might be reduced until you get them back.

Sorry to hear about the midnight ER adventure. Glad it ended up well in the end!
Would be great to know how well the plugs work out.
About to find out. My paravalvular leak is back as confirmed by a right heart catheterization yesterday. The pressures in the right atrium are the same as they were just prior to the leak repair. This may indicate that the exact same leak is back, that the leak grew worse despite or because of the repair or that a new leak has erupted with the same flow as the previous leak. If it's the same old leak again, what happened to the three plugs that were installed, lodged in a vessel somewhere?
At this point another TEE is planned to better image the mitral valve and locate the leak to answer those questions and also plan a repair, the most invasive of which is another OHS. My doctor said a direct repair is the most straightforward but that's a second OHS, which I'm not looking forward to at 72. One of the main reasons we went with mechanical valves, despite their required life-long anti-coagulation was their ability to last, obviating a second OHS.

Meanwhile the leak exacerbates hemolytic anemia plunging the Hgb and making daily activities slow and difficult.

I'll keep you posted...
 
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My paravalvular leak is back as confirmed by a right heart catheterization yesterday. The pressures in the right atrium are the same as they were just prior to the leak repair. This may indicate that the exact same leak is back, that the leak grew worse despite or because of the repair or that a new leak has erupted with the same flow as the previous leak.
Thanks for posting. I would query the doctors after TEE about the repeat leak, if it's confirmed. Such possibility sounds unusual (to me). There must be a reason for it, that could be relevant for the next course of action. Good luck!
 
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