New doctor, same good story

We met yesterday for the first time with my new cardiologist, Dr. Ari Cedars, at Johns Hopkins Hospital. This was my annual checkup, complete as usual with an echocardiogram and time sitting in little kids chairs since this is, after all, pediatric cardiology.

Ann Marie and I both very much like Dr. Cedars. He's energetic, positive, deeply knowledgeable, and had thoroughly read my history.  We are quite encouraged that things remain stable. With luck, my aortic valve may last me for the rest of my life (the goal of the Ross procedure), which would be awesome. The pulmonary valve I received in 2014 won't last as long, but so far, so good.

Oh, I also ran my hospital stairs test like I've done in previous years. Sprint up as many floors as possible before I'm thoroughly out of breath. I made it the equivalent of 9.3 residential staircases, or 112 steps. I do believe I made it to the equivalent of 12 flights last year, which, if correct, is admittedly a little disappointing. I want another shot at it!  (I'll post an update here if I do :)

Thanks for reading!

Yes, no news is good news

So it's been 3 years since I last blogged about my heart health, and I thought it's about time I catch you up. The short story is that, earlier this year, my cardiologist at Hopkins again confirmed all is stable, and, earlier this week, I hiked to the top of Old Rag Mountain you see in this photo.  So yeah, I'm doing well.

I've had a checkup at Hopkins least once per year.  Each time I learn something new, whether it's the finer points of selecting the right cuff to check someone's blood pressure, or the ever-improving CT scanning equipment which now times imaging with heartbeat to measure the aortic root with less margin for error.  As always I've felt fortunate to have access to great doctors who never stop learning.

Each appointment has followed pretty much the same pattern:

1. Carefully measure my aortic root, and other key aspects of the heart, with an echocardiogram and sometimes a CT of the chest
2. Provide current and previous test results to a team of experts, including my cardiologist and others, asking everyone to user their expertise and wisdom to identify possible changes in the heart
3. Recall that the test results can vary for factors unrelated to my condition, that we're looking for changes measured in one to just a few millimeters, and that a handful of millimeters can make the difference between health and heart surgery
4. Discuss how I've been feeling, my general health, the test results, and their conclusions
5. Leave with about 75% relief that things seem stable and optimism that they'll remain so, and 25% sobriety that it doesn't always go that way

This year's appointment was bittersweet.  I had known that my Hopkins cardiologist, Dr. Ringel, would be retiring, and this year's appointment indeed turned out to be my last with him.  He has for sure earned his retirement, so I'm excited for him to reached this point.  

But looking back at his 11 years with me as a patient, along with input from other experts, Dr. Ringel and team also had an epiphany of sorts.  It turns out that the aortic root sutures from my 2008 Ross procedure, and to some degree the scarring, are precisely where the risky areas would normally be.  If there were a dissection, it would keep the blood from getting between the layers of the aorta walls, which can develop into something quite serious in other patients. 

This epiphany, along with positive results and conclusions for my valves and ventricles, left me with improved optimism for the new year.  I'll keep doing my best to remain in shape, reducing my chances of introducing a complicating condition, or improving my ability to recover from whatever procedures remain in my future.

Thanks as always for listening!

Stable

After a successful appointment in 2016, my cardiologist recommended I see him again in 12 months.  Previously it had been 6 month appointments, or even more frequent, since 2009.  In my recent 2017 appointment, he suggested I might want to go on a 2 year cycle, seeing a "routine" cardiologist in intervening years (he's a specialist).

This is good news.

It is my heart's stability that led to his recommendations. The pulmonary valve I received in 2014 is doing well. The aortic valve is mildly to moderately insufficient (i.e. leaky). The left ventricle is a normal size and not growing.  The tri-cuspid valve is not leaking. And the aortic root, while still dilated, appears stable.

I'll take this news as a sign to enjoy life, eat well, and to continually improve through exercise.  I feel quite lucky to have those options!

Great results

Good news from Johns Hopkins for me today ... Tests this spring show no sign of genetic mutations that would cause additional concerns for my heart (specifically the risk of aortic dissection).  Whew, that feels great!  I'll check back in a year to see how far genetic testing has advanced -- it may warrant additional testing.

Oh, and the picture? That's me after 55 of the 75 miles my friend and I rode this weekend. Perfect weather, super ride!

Opinions

I've been slow in writing this heart update because I've had some complex appointments recently.  Nothing urgent, mind you, just a lot of new information.  New meds.  More tests.  And some mixed opinions.

Let me explain.

I've had three appointments at Johns Hopkins since early September, two in 2015.  Most recent was with Dr. Ringel to follow up on the craziness of last summer.  The new pulmonary valve from July is performing just as we'd hoped.  The three pressures that had measured high are now all where they should be, even slightly better than they were in September.  There is no evidence that the blood clots that lodged in my lungs had any ill effect on the new valve.  And because the valve's weak spot is infection, I've followed the standard recommendation for baby aspirin once a day, no dental work for 6 months, and as I have been doing for years, antibiotics before dental work.

So far, so good. The fact that I'm feeling fine, exercising as much as ever, is a real bonus.

My two other appointments came from renewed attention on the left side of the heart, where I had surgery in 2008 (the summer of 2014 addressed pulmonary issues on the right side).  Tests, though admittedly imperfect, now show that the aortic root, where the aorta attaches to the heart, has continued to dilate, now measuring slightly over 5 centimeters.  For people with their "original equipment" tissue, a measure 5 cm, from a normal 3 to 4 cm, is the point where corrective surgery is recommended.  Not correcting this problem increases the risk of aortic dissection, where the aorta tears (not good).  

But, like other Ross procedure patients, my aortic root tissue is not "original equipment".  While post-Ross dilation is not uncommon, there is debate about the risk of dissection for these patients, and the point at which surgery is recommended.  In the "you should be talking risk of surgery vs. risk of dissection" camp is Dr. Hal Dietz, I genetic guy I'm seeing who clearly knows what he's talking about.  I'm waiting to hear back on genetic tests that might tip the scales towards that discussion.

In the "let's just check you every 6 months" camp is Dr. Duke Cameron, my surgeon.  He's comfortable with where my aortic root is at, or at least is comfortable with the risk of dissection, for me, being less than the risks of surgery.  He put it best, when referring to the debate I mentioned earlier, that there are more people talking about the problem than there are patients with a real issue.

So, while we wait for genetic results I've started on a significant dosage of a blood pressure medication.   The hope, stemming from the laboratory and theory, is that this would help slow or stop the root dilation.  As long as I can tolerate the medication, I'll stick with it.

I'll wrap up for now, so I can call this post done.  Thanks for reading, and I hope to have good news in a month or so.

Looking back and looking forward

I had an echocardiogram and consultation yesterday at Hopkins. I'm glad to know the heart issues of the summer are behind me :-)  The new valve is doing just what it was intended to do, and the pressures that had been high are where they belong.

My lungs seem to be healing well and haven't held me back. While I haven't yet returned to my normal exercise level, I did enjoy a superb 58 mile bike ride on Saturday. It's great to feel more and more like myself. I also feel lucky how relatively easy my clots were to deal with... I've met plenty of people who had more serious complications than I did.

We now resume focus on the rest of the heart (left side), especially the aortic root (where the aorta connects with the heart).   Docs will review recent and historical CT's and echocardiograms to see how things have changed.  The hope, of course, is "not much".

Next regular checkup is in March. Thanks for listening in, and for all your kind words.

Back from vacation

We're back from 10 great days in California for vacation plus 3 in New Jersey for a Krahe-Buettner family reunion. All fun.

I've been feeling great, but I do know my lungs are still recovering. Had a small amount of discomfort during a chest cold I had early in the month, then felt a bit if restriction bicycling this weekend (riding moderately fast).

Next appointment is September 4th.

Background noise

Today was amazingly one of my longer appointments. Almost 4 hours.

We saw Dr Ringel twice. After the first time when all seemed well, he heard a murmur through the stethoscope that he heard just after the Melody valve insertion a few weeks back.  At the time he had written off the sound as coming from the leaking tricuspid valve, but now that the leak is gone, he wanted an explanation. A second echocardiogram was needed.

The murmur, it turns out, is coming from my Aortic valve. It is not a cause for immediate concern. In fact it is likely this murmur has been there for some time (after my surgery in 2008), but was masked by the big sounds of the pulmonary craziness.

We will watch this over time. The question, less important than the sound made, is the valve's performance. The valve has been categorized as mildy insufficient (leaky) since it was replaced , and may have slipped into the early stages of moderate. Time will tell.

Good numbers

My echocardiogram this morning thankfully shows pressure across the Melody valve where it belongs, or at least nearly so!  While they can no longer measure the right ventricle pressure, they are comfortable that it too has improved since my tricuspid valve is no longer leaking.

This is all a dramatic improvement from just 2 weeks ago, and a wonderful feeling.  Next appointment is September 4th.  Until then I'm free to start exercising as long as I keep in mind the right lung is still recovering.

Smiles all around :-)

Testing and theories

A sonogram of my legs yesterday showed no lingering blood clots. That's of course good news, as it means there shouldn't be any new embolisms coming.  What it doesn't tell us is whether the original clots came from the legs or from the valve itself.  The leading theory is the legs, or, more specifically, from the insertion points for the catheters.

Knowing the original cause of the clots would help future patients.  Could clots like mine be prevented in the future?  Was I more susceptible this time compared to 2009?  Am I hyper-coagulative (prone to form clots more quickly)?   For my short term, knowing is moot, partially since it's behind me, and also that I'm on a blood thinner for six months.  I'll have bloodwork for hyper-coagulation sometime soon, so that may add a piece to the puzzle.

That I'm feeling better each day is a good sign.  Less pain.  No fever.  Good energy.  I even walked a mile last night with Eddie, stopping to rest now and then, but light years better than a short walk to the corner with Alli I think on Sunday.

Because nothing like what I've experienced has happened in the ~3,000 other United States Melody valve cases to date, the next step was jointly determined by Dr. Ringel and his trusted colleagues around the country.  On Monday we'll do an echocardiogram to estimate what change, if any, has occurred with the pressures in and near the heart.  It could be that my body is still adjusting to the valve and reacting to the clots, or it could mean there is something else going on.  The results from Monday will tell us if we wait longer or if we do more tests (probably a catheter echo and camera inspection).

In the meantime, thanks for reading.  Enjoy the weather if you live near us!  The Washington Post gives the rating for today a subjective 10 out of 10 ... superb for a DC July.

Taking a deep(er) breath

Saturday evening and Sunday required a healthy dose of expletives, I'll be the first to admit.  The peaks of pain from my right lung weren't as high as the 24 hours previous, but for sure, they weren't far behind.  There were periods without pain, where I could lay down and sleep, but those rarely lasted more than about 90 minutes.

Granted, I had avoided the narcotic Oxycodone as my pain med, partly because of how it makes me feel (not like myself), and partly because I didn't need more of a departure from normal digestion (one of its side effects). I relied instead on Tylenol and Tramadol.

I was ready to start Oxycodone Sunday night, but a funny thing happened. Overnight I could lay down (on my left side only), and breathe, without pain. And I could easily fall asleep. Although I was up every 2 to 3 hours, I could always return to laying down and sleeping shortly after.

As you can gather, the days previous were not comfortable. While I could always get reprieve by standing, the need was far too frequent. And while I wasn't doing anything that required more than shallow breathing, I knew that deeper breaths were important to avoiding pneumonia, something to which, for the moment, I'm more susceptible.

Today was entirely different. Alli said it first, just watching me come into the kitchen to write a note while on the phone with Dr Ringel. I was clearly feeling better.

It sure helped that Ann Marie and kids were taking care of things at home, and my teammates doing the same at work. It let me rest and take care of myself.

I'll post later about my appointment at Hopkins today, and the next one scheduled for Monday. As for now,  I'm going to take advantage of the remaining sunlight :-)

Heading home

And it's before sunset, yay!

Answers

I like it when things fit together neatly.  My diagnosis, which came a short while ago, certainly does this. It completely explains how I've been feeling since yesterday, and likely explains the high pressures identified in my Thursday followup.  

So, Hopkins docs have determined I have multiple pulmonary embolisms.  That is, multiple blood clots that likely formed in my legs during my 30+ hours of bedrest, and traveled to the arteries in my lungs sometime afterward.  Both lungs are affected, with the right lung having the larger group of clots and the largest individual clot.  The location of my pain completely matches up to the location of these clots.  My intuition about how I felt lines up perfectly.

The pain tells a story. It is unfortunate, but the pain is portions of the lungs dying off.  For what it's worth, the pain is felt in the chest wall, originating with the  inflamed, dying areas. While this may sound scary, most patients that have this don't feel any real difference afterward, so the hope is that's true for me.  Because of the great redundancy that the body provides, the affected areas are relatively small.  

My treatment plan involves a blood thinner for at least the next 6 months.  While it does not technically break up the clots that exist, the thinner gives the body the time it needs to do so naturally and helps prevent future clots. I will start on one newer-than-Coumadin medication, Rivaroxaban, that is less likely to lead to unwanted bleeding and doesn't require monitoring.  And while I am still in a fair bit of pain, but we have options to manage that, and it should subside by mid-week.  

It's a great feeling that this diagnosis very clearly matches the symptoms that started yesterday, and it quite possibly explains the higher pressures that Dr. Ringel saw on Thursday.  A conversation with Dr. Ringel will give us a better sense of that likelihood.  He may back off on his tentative plan to inspect the valve with a camera in the Cath Lab as incisions to my legs wouldn't match well with a newly started blood thinner treatment.  We shall see what he says.

We are hopeful to be going home today, and hopeful that this won't affect our travel plans later this week.  Thanks for keeping an eye on me here, and thanks for all of the encouraging words.  

Good test results so far

The pain is still quite present but the echo, EKG, chest xray, and initial blood work show no problems that would explain it. It's only the echo that looks abnormal, but it reads same as on Thursday with Dr Ringel (ie nothing new).

Still at Hopkins, waiting for next step.

Staying overnight

A combination of the late hour (can't do all tests this time of night) and my fever (100.8) means I'm here at Hopkins for the night. A good dose of Tylenol has made a world of difference, as has the always friendly Hopkins staff :-)

Got a chest xray and bloodwork. In a private room watching Full House with Ann Marie and Eddie. I think I'll sleep well later.

Waiting for doctor

Enjoying time with Ann Marie and Eddie while we wait for an ER doc. Already saw nurse. Laughing is fun, but painful!

Headed back to Baltimore

What could be a muscle pull is causing some pretty sharp pain in the right side of my abdomen. Breathing deeply is difficult (because of the pain), sneezing is out of the question. Also have some general achiness in joints, and a low grade fever. This started in the morning, and worsened throughout the day.

Cardiologist wants me checked out tonight, so I'm going in for bloodwork, echo, and x-ray. Depending on timing and my condition, I might be there overnight. I am hoping for a super awesome room like last time, if I am!

Still waiting

Had my followup appointment at Hopkins yesterday. I will share details later, but the short story is heart my pressures haven't changed yet.  While Dr. Ringel has seen a similar problem in multiple Melody valve patients, he hasn't seen or heard of mine.

The hope is that it sorts itself out, so we are going to wait it out a month.  Then, if no improvement, he'll go in with camera to hopefully see what is going on.

Better each day

Had a great meal and fun conversation last night at friends' house :-) Woke up today feeling fairly energetic and went to work. Nice catching up with concerned friends at the office, and good to start catching up on work.

After 8 hours I was ready to go, drove home, and just woke up from a nap. That is unique for me. Ann Marie even said that she never sees me yawn, but this is different :-)

Follow up appointment scheduled with Dr Ringel on Thursday. They'll do another echo to see how I've progressed (the lungs apparently take some time to adjust after this procedure). We'll also have a chance to ask more complete questions about outcomes since we have partial info now.

Thanks for keeping tabs on me!

Dr Duke Cameron

Here's a video with the super guy who did my heart surgery in 2008.  We sought him out in 2006 because of his expertise in aortic valve replacement (where it seemed I was headed) and his deep knowledge of Marfan's Syndrome (which was suspected but I do not have).

Of the three hospitals and surgeons we spoke with, Dr Cameron was the only one with the wisdom to recognize the importance of timing valve replacement surgery. His words were "you know you can have the perfect surgery, but as soon as you do  you start the clock ticking on new problems". He also had experience with the critical weakness of a new type of valve another surgeon had strongly recommended.

Here's the video: Duke Cameron, MD