New way to create an ASD!

Here’s a report of a novel procedure that will hopefully come into use soon: creating (or enlarging) an Atrial Septal Defect (ASD) without breaking the skin.

I had a small ASD at the time of my first surgery but it needed to be larger. Back then (1967), ASDs had to be created or enlarged the old fashioned way – with a scapel. This was known as the Blalock-Hanlon Procedure, and it was pretty straightforward: cut into an Atrium, look for the ASD, and enlarge it. If you couldn’t find an ASD, make one.

Surgeons don’t perform the Blalock-Hanlon very often these days. (When I read my operative notes just a few months ago I had never even heard of the Blalock-Hanlon, much less knew that I had one!) Today the majority of ASDs are created by a balloon atrial septostomy, which is done by a catheter.

But this new procedure wouldn’t cut the skin at all. Histotripsy is “tissue liquification by ultrasonic wave” – the same technology they use when your uncle has his gallstones crushed. The suffix “-tripsy” is a Greek word that means “to massage” or “to crush.”

This was a small study of only ten dogs. Ultrasound was used to locate an appropriate point for an ASD and then the defect was created by Histotripsy. In nine out of ten cases, the shockwaves created an ASD, and later examination showed that there was “minimal damage” to surrounding cardiac tissue and no damage to the outside of the heart.There is also evidence that Histotripsy could be used for some cardiac ablations.

Much more research needs to be done before this becomes an accepted procedure, but the initial results are promising!

What really happened

Back in August I blogged about faxing a HIPPA compliance form to Johns Hopkins Hospital to get some medical records from my 1967 heart surgery. I already had the records, actually, but I had managed to misplace several of them. So I filled out another compliance form and faxed it to the Medical Records office.

Hopkins responded by sending all of my medical records from that first surgery, not just the 12 sheets I already had. I returned to Hopkins several times for check ups until I was about five years old, but none of those records are included. So now instead of 12 sheets, my stack is about an inch thick. This was a lot more information than I expected, and every bit of it is interesting.

I have Catherization reports, – one of which destroys a family legend; I always thought that we arrived at Hopkins at roughly 10:30 PM, but the Cath report says that I was brought into the Cath Lab at 6:16 PM. Daddy says we arrived at Hopkins about 3:30 pm, and the surgery itself began at 10:30 PM.

There are Radiological reports but no X-rays. I wonder if they deteriorate over the years. Possibly they are filed away in a different place, since all my records are copies, most likely made from microfilm.

Quite a few pages of Nurse’s reports (I can – and probably will – make an entire post about those!) and -

Oh my goodness…

The title of the sheet is OPERATING ROOM REPORT. And this is the entire report, not just the summary and the little bits and pieces of my operation that I assume from what I already know.

After the temperature was down to 32 degrees we used inflow stasis and during a three-minute period of inflow stasis dissected a sizable portion of the Atrial Septum.

I was too small for the heart/lung machine, so the surgical team stopped my heart temporarily by hypothermia – They literally put me in ice and cooled me until my heart stopped. During a three minute period of no blood flow doctors cut into my heart and enlarge an Atrial Septal Defect (ASD). For the heart to work (and its owner to survive) blood has to be able to complete the Cardiopulmonary circuit: flow from the body into the heart, move from the heart to the lungs, then back to the heart, and once again out to the body.  Because my normal pathway is blocked my blood “escapes” through an Atrial Septal Defect (ASD), and that earlier Catherization had revealed that my ASD wasn’t quite big enough.

…at the end of this part of the operation we proceeded to prepare the vessels for a right Pulmonary Artery anastomosis after the technique of Glenn.

This is a phrase that I type often and Funky Heart readers are probably tired of seeing: I don’t have the Glenn Procedure that is currently in use. That’s known as the Bi-Directional Glenn, because blood is rerouted to both lungs. I have the original version of the operation, the “Classic Glenn” or sometimes called the Unidirectional Glenn. In my version, the right branch of the Pulmonary Artery is cut and sewn into the Superior Vena Cava, so the majority of my blood flows into my right lung. CLICK HERE to see the best drawing I have ever seen of the Classic Glenn Procedure.

Chest was closed in layers with #00 catgut and skin with Dermalon. The patient tolerated the procedure well.

(Signed)

Vincent Gott, M.D.

Wow. Just…wow.

Lies, Damn Lies, and Statistics

Here’s a “tweet” that’s been retweeted (copied and passed on) a lot lately:

Downs Syndrome: 1 in 1000 births, Autism: 1 in 100, Congenital Heart Defects: 1 in 85. Which one have you heard the most about?

Say what?

Well, first things first, I can tell you that the incidence of Congenital Heart Defects (CHDs) is not 1 in 85. It affects 1 live birth per every 125, and has stayed at that rate for quite a few years. A jump to 1 in 85 would be a 32% increase, Cardiologists would freak out and the news media would be talking about a “Heart Defect Epidemic!” That’s not happening.

But what has happened is the rate of Autism occurrence has changed: from 1 out of 150 children up to about 1 in 91. That’s a huge percentage change, too: 39%. When you see a huge jump in a number such as this, the first thing you do is ask, What’s changed?

What has changed may not really be an increase in the number of cases of Autism, but rather a better understanding of the disease. Autism is an extremely complicated disease probably (it is not certain) caused by changes that occur at the cellular level.

Relatively speaking, Heart Defects are easy to identify: There is only one basic heart design, and any variation can be considered a defect, even if it has no effect on the body. Thirty-five different Congenital Heart Defects have been identified, though it is not unheard of to have more than one. With Autism no one seems to know how it occurs, exactly what occurs to cause it, and it can manifest itself in many different ways.

But researchers have now identified another possible “key” to the Autism puzzle, and the general understanding of how the brain develops is improving.

But that really doesn’t address what is so irritating about that “tweet” that keeps going around. I’d really like to know where that “1 in 85 have a CHD” statistic came from, because it just doesn’t make sense. There has been no trend that shows that kind of increase.

Changing the numbers to highlight “your” cause doesn’t help, and is embarrassing when that fake number bumps into the facts. Both Autism and Heart Defects are debilitating illnesses, and campaigning for more research into either is a worthy cause. Don’t cheapen either one by playing fast and loose with the numbers.

Migraine Headaches and Heart Defects

Is your migraine headache caused by a heart defect? It’s possible!

Recent research has uncovered an interesting tidbit of information:  People with an Atrial Septal Defect (ASD) or a Patent Foramen Ovale (PFO) who suffer from migraines can see a reduction in the number and severity of headaches when their defect is closed.

Many people may go their entire lives with a ASD or PFO and never know they have it; or if it is detected, their doctor reports that the opening is so small that the best course of therapy is just to monitor it.  With advancing technology and improvement in Catheters, repair of the defect became more practical. The fact that migraine headaches faded or went away completely was a happy side effect of the repair.

It doesn’t always work – one study showed a small number of patients (4 out of 97) actually developed migraine symptoms (their headaches disappeared after several months) and there is at least one known case of a person who developed a migraine every day – with no relief of symptoms.

When I was younger, I averaged a “dizzy spell” per month – just a few moments when everything seemed to take a few steps to the right. Usually it cleared up in less than a minute, but at least once a year I could count on having a really bad bout of dizziness.

Everything would start spinning and it just wouldn’t stop. And it didn’t stop until I had thrown up. So I learned that rather than suffer until things happened naturally, it was best to shove a finger down my throat. Sorry if that thought turns your stomach, but I’d rather do that than to suffer.

Then one day while I was in college it hit me: Dizzy spell, and a bad one. But a friend and I had made plans to get dinner at one of our favorite restaurants that night, so I had a light lunch. A very light lunch – a soda and a pack of Nekots, if I remember correctly.

In case you didn’t know, you can’t throw up on an empty stomach. Finally after about two hours of the world spinning, I think my body just decided it had had enough and the dizzy spell let me go. I got back to my room and slept fourteen hours!

After I had my pacemaker installed, the dizzy spells went away. I haven’t had one since, not even a mild one. No one has ever figured out why, it’s just “one of those things!”

So if you are having migraines, ask your doctor about the possibility of you having an ASD or a PFO. A few days in the hospital getting the repair could end them!

The Advocate: Kim, Heart Mom, Writer of The CHD Blog

A little more than a week ago I volunteered for The Blogger Interview, an exercise intended to help bloggers learn more about our fellow writers and perhaps introduce our readers to other blogs that they may find interesting. One of the rules of The Blogger Interview is that you offer others a chance to participate, and Kim from The CHD Blog has risen to the occasion. So below, you’ll have the opportunity to meet her. Kim is a Heart Mom; her daughter was born with Transposition of the Great Arteries with an associated Atrial Septal Defect and Ventricular Septal Defect.

The Challenge is STILL open… we’d love to hear from any Survivors or Heart Parents (or Medical staff!) who would be willing to take the challenge!

And now, Kim. My questions are in bold, and her answers are in plain text:

Were you a blogger before you created the CHD Blog?

Yes, I have a personal blog, Herding Cats, that I created immediately after my daughter was diagnosed with a CHD. The hospital I was at didn’t tell me about Caring Bridge or CarePages, so I winged it – I created a blog to keep family and friends informed. It was a great way to vent, to organize my thoughts and to share news without having to repeat myself. Since then, it’s grown into a sounding board and a way to stay in touch with friends and family outside my immediate area. I started and just can’t stop! (Kim doesn’t mind if you stop by, but she reminds us that it is her personal blog, so not everything is CHD related. There are “lots of brain droppings there!”). It’s the cheapest form of therapy I’ve found to date.

The CHD Blog was born last year on a whim. I realized only a couple of weeks before February that there was a CHD awareness week. I wanted to do something. When my daughter was diagnosed, I felt alone in a vast sea… even the Internet couldn’t provide the answers I craved. The connections I wanted. Or a quick resource to get the information I needed. I thought that perhaps a portal could be created that was not affiliated with any organization, completely (or mostly) unbiased and for the general public’s consumption. That’s why the CHD Blog was born.

What have you learned from other Heart Moms and Dads?

I’ve learned how to advocate for my child. Coming into the world of acute care for the first time, I still held the ancient belief that the medical community knew much more than I did. While they do know more about specific diseases, organs, etc., they do not know my child. Only I can truly advocate for her needs.

I’ve also gained a support network I never knew I needed. I’m not much one for “support groups.” I don’t like group activities per se. Yet I have met incredible men and women both virtually and in person that have walked down similar paths. It’s hard to relate all of your feelings about “that” road we parents (or you patients) have been down. I didn’t even realize many of the emotions I harbored. Having friends that have walked similar paths in life really does make a difference.

What’s a routine day around your house like?

Honestly? Loosely organized chaos. I own a Montessori preschool, as well as an association management firm. I manage two full time clients (their accounting, marketing, public relations, event planning, administration, etc., etc.). I am married, have two daughters (six and two), two dogs, two guinea pigs and somewhere around 30 fish to care for. My day starts somewhere around 5:30 and ends somewhere around 1:30-2. I spend some time in the preschool, a lot of time behind the computer or phone working with scientists and business professionals around the world, try to make sure to have some one-on-one time with my girls, and try to cram some volunteer work (usually CHD related) into the few remaining hours of my day.

Describe the moment that your doctor told you that your daughter had a heart defect?

It was a moment of pure, unadulterated shock. We knew something was wrong, but had thought it was minor at best. As the doctor drew a crude drawing of a heart on a napkin, his hand was shaking. He would not look me in the eye as he discussed her emergency intubation, that she needed to be medivaced out immediately, that surgery was her only option, and it was dire that intervention happened immediately. Until that moment, I had never even fathomed the possibility of a heart defect. I didn’t even know what CHD was.

In that moment, I asked stupid questions. One of the first, which I still cringe at, was if she would have open heart surgery, and if there would be a scar. Well, yes, dummy. What a stupidly naive question, so irrelevant to the life and death situation we were in. But I couldn’t connect the dots. I’d just had my daughter a few hours before, only 7 minutes after flying through the ER doors. And now they were telling me she was struggling for her life. I hadn’t had time to hold her. I hadn’t even really seen her. It was all just too much to take in.

After a long night of trying to wrap my mind around the situation and my reaction, I decided to change immediately how I was dealing with the trauma. I refused to let myself go down that “shocked” road again. My mode de operand since has been to push all emotions aside and to get analytical and realistic when faced with life-death situations. Of course, there are a lot of drawbacks to ignoring emotions and refusing to allow shock to take over – it only gives them opportunity to fester and grow, and it’s often ugly to deal with their neglected mutations after the emergency has passed. But it has helped me to become a better advocate in traumatic situations. Some people comment on this being a strength, but in fact it is not. It’s a total weakness – I admire other parents and patients that can cope with the emotional aspects of medical trauma while it is in progress.

What is your wish for your daughter?

That technology is always ahead of her in terms of treatment and her medical needs. That she lives a full and happy life. And that she never doubts for a second how much I love her.