Christmas 2012

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LTGA/ccTGA post published today.

Ah I just completed the post on Congenitally Corrected Transposition of the Great Arteries that I started in November and it published with November's date, so much lower on the front page.
I thought I would just put a link to it here today ;-)

http://www.care4abi.com/2012/11/congenitally-corrected-transposition-of.html?m=0

Art
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3 years post Double Switch

Yes, yesterday we went with Abi to see dr. Greene for her annual checkup and yes, this coming Sunday it will be 3 years since the first surgery.

The appointment went great. Abi was happy, active, friendly, open and very social ;).

Talked to everyone she could, was one big smile and, most importantly cooperated with every single test.















Her blood pressure and EKG were perfect.

Echo, was one of the fastest ever. She was so brave and calm and the results were great.



As for now, and we pray forever, we have nothing to worry about. Her heart looks great, all the work done in 2009 and 2010 holds up very well.

We had a good time talking with dr. Greene and then Abi got some prizes ;).

Here she is with dr. Greene picking up some stickers, bracelets and toys






Art

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And here we are

Another annual cardio checkup. Right now. Right here at the clinick




Art

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Congenitally Corrected Transposition of Great Arteries

It's time for the big one. Congenitally Corrected Transposition of the Great Arteries (ccTGA, LTGA).

In normal, healthy heart there are 4 chambers. Two upper chambers called atria and two lower chambers called ventricles. There is a Right Atrium (RA) that empties to the Right Ventricle (RV), allowing oxygen depleted blood coming back from the body to be delivered to lungs through Pulmonary Artery (PA) arising from the Right Ventricle (RV). Then, through the Pulmonary Veins blood comes back to the Left Atrium (LA) that empties to the Left Ventricle (LV) through the Mitral Valve and then the oxygenated blood goes out to the body through Aorta (AO) arising from the Right Ventricle (RV).

In case of Congenitally Corrected Transposition of the Great Arteries (ccTGA or also called LTGA for Levo Transposition of the Great Arteries) there is an atrioventricular discordance and ventriculoarterial discordance (AV and VA discordance), meaning the Right Atrium (RA) empties to morphological Left Ventricle (LV) through Mitral Valve, from which arises Pulmonary Artery (PA) providing deoxygenated blood (blue) to the lungs. Then the oxygenated (red) blood comes back through Pulmonary Veins to the Left Atrium (LA) and through Tricuspid Valve empties to morphological Right Ventricle (RV) from which Aorta (AO) arises distributing blood to the body.

As you see, both ventricles are switched performing roles they have not been designed for, but due to both main arteries (Pulmonary Artery and Aorta) arising from incorrect ventricles, the correct blood flow through incorrect path is provided.

Here's a nice picture showing both, normal and ccTGA heart described above:

The data shows that about 20% of children born with LTGA is also born with heart on the right side of the body (not left). This is called dextrocardia.

Treatment:

If this is the only defect, surgery may not be required and children with LTGA may potentially live long, normal lives without the need for surgical correction.
They remain under regular care of their cardiologist though, who needs to monitor the heart's condition, since the Right Ventricle (RV), being designed to work under much lower pressure, is now pumping blood to the body, working under approximately 3 times higher pressure. This poses a risk of blood regurgitation through the Tricuspid Valve, weakening of the heart and Complete Heart Block (CHB), in which the contractions of upper and lower chambers are not in synchrony and there may be a need for a pacemaker.
The risk of CHB increases 1%-2% each year.

In case a corrective surgery is needed (due to other defects, or to simply correct the defect and increase chance for long healthy life) a Double Switch (DS) surgery may be performed.

There are different variations of Double Switch; Mustard or Senning.

In Abi's case it was Senning procedure.

You can check out the description of Abi's surgery, including DS and VSD closure here:
Part 1
Part 2

Art

Ebstein's Malformation of the Tricuspid Valve

Today I would like to talk about Ebstein's Malformation of the Tricuspid Valve. One of the defects Abi was born with and which hasn't been corrected yet.

We pray she will never need this corrected and will live full and long life without this every affecting her again.

Ok, so what is Ebstein's Malformation of the Tricuspid Valve?

Tricuspid valve is the inlet valve of the right ventircle that is built of 3 cusps (leaflets), hence the name tricuspid.

This valve lets low-oxygenated blood from right atrium in to the right ventricle (right lower heart chamber). When this valve opens, the blood flows into the ventricle. Then the valve closes, creating a perfect seal and blood is pumped out from the ventricle to lungs through pulmonary valve and pulmonary artery.

When the tricuspid valve is malformed or displaced it means it's located lower than usually and does not seal the ventricle properly, and then we talk about Ebstein's Malformation. In this case, the valve does not seal properly and low oxygenated blood leaks back to the right atrium.

This causes atrium enlargement and leads to congestive heart failure (you will find the symptoms listed at the beginning of the Pulmonary Stenosis post from last week), and build up of fluid in the lungs.

Ebstein's Malformation often is accompanied by ASD (Atrial Septal Defect, a whole between right and left atria, upper heart chambers). This causes the blue, oxygen depleted blood and the red, oxygenated blood to mix. This leads to poor oxygenation, low oxygen levels in the body.

Treatment

Depending on severity the condition may be left untreated and not affect the patient in any major way. It will have to be monitored though.
If the condition is severe it may require surgical intervention, through an open heart surgery.

The cardiothoracic surgeon may be able to fix the valve, reposition the leaflets or may need to replace the valve completely with a cow's or pig's tissue valve, called bioprosthesis.




In Abi's case the valve is lowered and not fully closing causing some mild regurgitation.

Before the Double Switch surgery it was working on the systemic side, performing left ventricle's job, pumping blood to the body at a much higher pressure. This led to much more severe regurgitation and caused multiple problems.

Now, after Double Switch, it's back on the non-systemic side, working under much lower pressure. It is not leaking much, hence it's not affecting Abi in any way at the moment.

It was too risky for Abi to attempt to fix it during the Double Switch surgery, so it was left intact then and, so far, seems like it was a good move.

Art
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Pulmonary Stenosis

Alright, time for another post. For today I picked another of Abigail's heart's complexities, namely Pulmonary Stenosis (PS).

There are several forms of this abnormality and Pulmonary Stenosis may occur in couple different places of the heart-lung circulation system.

Let's start with a short description of what Pulmonary Stenosis means. Basically it means there is an obstruction to the blood flow from right ventricle (right, lower pumping chamber) to the lungs.

Since there's an obstruction, it is harder for the right ventricle to pump the deoxygenated blood to the lungs. Heart needs to work harder, and depending on how severe the PS is, even much harder, leading to congestive heart failure.

Here are couple possible variations of Pulmonary Stenosis.

The most popular, Pulmonary Valve Stenosis occurs when the obstruction of blood flow is caused by the tricuspid valve. The tricuspid valve has 3 leaflets (3 cusps) which open fully when the hurt pumps blood to the pulmonary artery and closes, when the right ventricle relaxes, to avoid a back-flow of blood from pulmonary artery to the heart chamber.

When the tricuspid valve leaflets are fused together, or the valve is malformed and there are 2 cusps (leaflets) instead of 3 (then it's called 'bicuspid valve'), the valve does not open fully, obstructing blood flow.

Subvalvular Stenosis, is a blood flow obstruction that occurs just below the Pulmonary Valve, in the upper part of the right ventricle called the outflow tract. The outflow tract is a muscular tunnel that normally is wide open, unobstructed and participates in pumping blood to the lungs. When the outflow tract is abnormally thickened, it can cause Subvalvular Stenosis, also called subpulmonic or infundibular stenosis (infundibular from the name of the outflow tract muscle- infundibulum).

Supravalvular Pulmonic Stenosis is a narrowing of the Pulmonary Artery just above the Pulmonary Valve.

When the Pulmonary Artery narrows after it branches off to the left and right lung then we talk about Peripheral Pulmonary Artery Stenosis or Branch Pulmonary Artery Stenosis.





Symptoms

Unless the PS is very severe there will be no symptoms, other than heart murmur.

If the PS is severe, it may lead to congestive heart failure and the symptoms I listed in my previous post, Ventricular Septal Defect, but let me list them here too:

- poor feeding
- poor weight gain
- fast breathing even when the baby is fully relaxed and in deep sleep
- excessive sweating
- congestion
- cough

In a very young child it will also lead to cyanosis, blue discoloration resulting from low blood oxygenation.


Treatment

Very mild PS may not require any treatment, but the child will have to be closely monitored by pediatric cardiologist.

Severe PS may require ambulatory or surgical treatment, depending on the severity of PS, placement and overall condition of the patient.

Pulmonary Valve Stenosis can be treated by inserting a small balloon placed at the tip of a catheter through patient's groin area, to the valve and blowing it up, opening the valve. The procedure is called ballon valvuloplasty.

Ballon Valvuloplasty happens under deep sedation and usually patients are discharged from the hospital within 1 to 2 days. If the narrowing reoccurs, the procedure may be repeated, or patient referred to surgery.

Subvalvular Stenosis (obstruction below Pulmonary Valve) or Supravalvular Stenosis (obstruction above Pulmonary Valve) is usually repaired through an open heart surgery after putting the patient on a heart-lung (bypass) machine.

During the surgery the obstructing muscle can be removed, or partially removed, or valve opened, if dealing with fused valve cusps.

When dealing with Peripheral Pulmonary Artery Stenosis, the narrowed artery can be patched, or alternatively a catheter ballon dilation can be performed to stretch the arteries.


Recovery

Unless the child is very sick, the recovery time after the surgery is usually couple days.


In Abi's case, we dealt with Subvalvar Pulmonary Stenosis, since her left ventricle was doing the job of the right ventricle and was on the non-systemic side, the blood was going out through a bicuspid valve. Also, to add to the complexity, her tricuspid valve was malformed (Ebstein's Malformation), which means it was located lower than it should, and it was close to the VSD, causing one of the leaflets to leap through the VSD and further obstruct blood flow to the lungs.

I hope this is a pretty clear and not too long description of Pulmonary Stenosis. There are many great resources out there, if you are interested in getting more in-depth information.

Art

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Ventricular Septal Defect (VSD)

We would like to write couple posts on the heart defects Abi was born with, to break down the complexity of her heart and explain one by one.

Normal Heart:

Let's start with the most common heart defect among newborns, Ventricular Septal Defect, or VSD.

To simplify, heart is built of 4 chambers. The two upper chambers are called the atria (left and right atrium) and the two lower chambers are called ventricles (left and right ventricle).

What is VSD?

It is a hole between the two, lower heart chambers.

Interventricular Septum is the wall of tissue between left and right ventricle (the lower chambers), and when there is a single hole or there are multiple holes in this wall, then we talk about VSD.

There are 2 common forms of VSD:

- Muscular VSD

- Membranous VSD or peri-membranous defect

Muscular VSD means there are holes in the muscle wall all over the septum. As long as the holes are small, there is a good chance they will close on their own, or with help of medications over time, without the need for surgery.

If the holes are large or located in the RV outflow tract then the surgery may be required to prevent the VSD from affecting the nearby aortic valve.

Membranous VSD (peri-membranous defect) occurs close to the aortic and tricuspid valves and the hole does not contain muscle tissue.

In any case, when VSD is present, oxygenated blood from the left ventricle, where there's higher pressure, crosses to the right ventricle. The larger the hole the more blood crosses over.


How is VSD discovered?

Usually within the first couple days or weeks of life, a very soft heart murmur maybe heard. Since for the first couple weeks after birth pressures in lungs and right ventricle are higher, less blood crosses from the left ventricle through VSD, hence the murmur can be very soft or even completely absent.

Once the pressures in lungs and right ventricle get lower to normal, more blood will be crossing between the chambers causing more audible murmur.

For some time the baby may be asymptomatic and if the VSD is small, there's a chance it will close on its own. Usually patients are, or at least should be closely monitored.

If the VSD is large or is not closing on its own, there is a chance the baby will develop a congestive heart failure, also called over-circulation.

Since in Abi's case we already knew about VSD, the murmur was discovered early, but it was not very loud.

The following are signs of congestive heart failure, which Abi was showing as well:

- poor feeding
- poor weight gain
- fast breathing even when the baby is fully relaxed and in deep sleep
- excessive sweating
- congestion
- cough

Depending on the size of VSD the aforementioned symptoms may be more or less severe.


Treatment of VSD

If the VSD is small and there are no symptoms of Congestive Heart Failure, there may be no medical nor surgical treatment necessary and the VSD may close by itself.

If the VSD is larger, medication to strengthen heart and diuretics to help body get rid of water, may be applied. If the medication is helping with controlling over-circulation, there's still a chance VSD will close on its own and no surgical intervention will be needed.

Also increasing calories intake, by adding high calorie formula to breast milk, or using less water with formula may be advised to help the baby gain weight.


Surgery

Surgical intervention to close VSD may be necessary when there are no signs of improvement, no signs of VSD closing on its own.

It may also be recommended when the baby is not gaining weight and the Congestive Heart Failure persist, or there is a risk of a permanent damage to other organs due to VSD.


Recovery

Recovering after surgical VSD closure takes couple weeks, but it's been said the complete recovery process is 6 weeks. After closing VSD (if it was the only heart defect) the baby is considered healthy and should not have any further problems as a result of VSD

Art

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Abi's First Vacation in Poland

We got back from our first family vacation in Poland a while back, but with all we've got going on it took a while to write this post and get all the pictures. Actually we are not going to use all of the pictures in this post ;-)

Abi and I (Art) left to Poland one week before mommy could join us, so it was our first, this long flight, just the two of us.

I can't say enough about how wonderful Abigail was all this time. Starting from the airport in the USA through Germany all the way to Poland. She was a delight to travel with. No, it was not easy, it was exhausting, but it could have been so much worse if she wasn't as wonderful as she was.

We both had a lot of patience for each other and Abi even took a 4 hour nap on the flight to Germany. Sure I wish she slept 7 or 8 hours out of the 9 hours flight, but better this than nothing, right? :-)

This is Abi at the airport and on the plane.




I took Abi to the church I used go to in Poland. She was playing with some of my friends' kids and it was so fun to watch her interact with kids that did not speak English. The conversations were priceless, but most importantly, all the kids had fun.

She also got to meet her cousins for the first time, spend some time with them and get to know them ;-)




Since Abi loves animals and zoos, we took her to this nice Zoo in the place we lived. We walked and took this fun train that she enjoyed a lot.




Since Abi and I arrived in Poland a week earlier Abi got to greet mommy at the airport. Mommy was showing a lot at the moment, but since no body had known about our next little girl, she put all the cloths on to cover the belly and not to spoil the fun of telling everyone through a fun t-shirt we had made back home.



It took everyone some time to pay attention to the t-shirt, but here you have both grandmas the moment they read the what was on the t-shirt, namely " I'll be a big sister! February 2013"




Then at my parents we made Abi's first bonfire which she loved.





And here's Abi at her first dentist visit ;-) Yes, no stress, no fear, rather fascination ;-) Luckily she has beautiful and healthy teeth.




There is so much more to tell about this trip and so many more pictures, but there's no way we could do it in one post here.

It was amazing to watch Abi interact with our families and friends, with people she met for the first time in her life, people from different culture and speaking mostly or only Polish. Before going to Poland we had to work hard on reminding Abi to speak Polish with J.

Since J was often forgetting to be consistent and require Abi to speak with her Polish only, they used to carry bilingual conversations, what led Abi to believe no matter what language she speaks, everybody understands English. It caused some challenges in Poland at the very beginning, but the change in Abi and her Polish after spending only 2.5 weeks in Poland was unbelievable.

We didn't have to remind Abi to speak Polish with J once since we got back. She switches between both languages automatically, very fast and without getting confused.

I so wish my brain had the same potential now, at my age hehehe.

I couldn't be any more proud of my little girl ;-)

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Abi growing up - playground skills :)

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Abi's project: bird house

Abi and I had so much fun making little bird houses this summer. She did most of the painting and really enjoyed the project.








Now do we hang them outside or just keep them at the house to preserve our little project :)?

~J

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Abi is 3!!!

Happy birthday my sweetness












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Day with mommy

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At the pool & lake

My silly diva! :)

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Infant and child CPR once again

I know I have blogged about Infant and Child CPR importance before and mentioned this one app, but since I was approached by someone with medical background and directly involved in CPR awareness and education, I would like to bring this subject up once again.

Dr. Raymond Wu, being as surprised as me by the fact that not all parents are taught how to perform CPR on an infant, started looking for relatively easy ways to at least introduce parents to CPR. One thing led to another and he created a really cool, interactive iPhone/iPod/iPad app called BabyCPR. 

I had the pleasure of testing this app and I need to say it's a very interesting concept. Of course, nothing can replace a dummy/doll and a professional standing right next to you to teach you CPR, but this gets pretty close. I would say, much closer than any video I've seen so far.

The app, BabyCPR, was developed by Transcension HealthCare LLC, and the developer can be contacted at contact@babycpr-app.com. Here's the link to their site.

Chicago Parents Magazine and Two Peds in a Pod talked about this app as well, so I thought I would provide the links and you can check out the articles on your own. 

As for the app, let me just use the app description below, Dr. Raymond sent me, so you know what to expect:

FEATURES:

♥ Skills Practice

Learn and become more comfortable with the techniques and critical timing of CPR by doing hands-on practice on your device. In BabyCPR, you can use the accelerometer and microphone to simulate rescue techniques such as chest compressions, rescue breaths, and more!

♥ Multiple Levels of Learning Support

BabyCPR makes it very easy to learn by walking you through the material step-by-step. This app presents the information in several different ways so that you can learn at your own pace. Go through the modes listed below and you'll be able to go from zero to hero in no time!

✓ Interactive Tutorials

✓ Demonstration Tutorials

✓ Practice Mode with Prompts

✓ Practice Mode without Prompts

✓ References with pictures and animations

♥ Real-time Feedback

The BabyCPR AI gives you detailed guidance as you learn and practice so that you can master even the trickiest parts of infant CPR. Feedback includes pacing of compressions, positioning of the baby, duration of rescue breaths, and more. It's the best way to get good fast!

♥ Unlimited Unique Scenarios

The BabyCPR simulator can create an unlimited number of unique cases so that you can be sure you're truly learning the material, not just memorizing case scripts.

♥ Scenarios Covered

✓ Mild Choking

✓ Severe Choking

✓ Unconscious - Breathing

✓ Unconscious – Not Breathing

♥ Based on the latest American Heart Association 2010 Guidelines

♥ Designed and developed by an integrated team of parents, physicians, nurses, engineers, and simulation experts

♥ Quick navigation to References and Summary Flowchart 

Infant CPR is appropriate for babies up to 1 year old.

And here are some screenshots:

OK, this post is already long without me typing too much, so to sum it up. I liked the app. I think it's a great way to learn on-the-go if you don't have the time or resources to do an in-class training. 

CPR is not a joke and when it comes to it, it is really easy to freeze, panic and feel lost, and trust me it's not the right time to freeze. 

It is always good to get at least familiar with the techniques and steps. Seeing how it's done, seeing the steps and even, I know this will sound silly, but seeing a video or reading the steps when someone is calling for help may help you respond properly, in case you find yourself in such situation. 

Art

Breakfast in bed

Today Abi woke up, came to us to cuddle for a minute (yes, she's been in a big girl bed for a week now) and said she'd be right back. She went downstairs and brought us barbecue chips for a snack in bed. It was so sweet and awesome that she almost convinced me to open the bag of chips! :)
As we both went downstairs to get her a cup of milk that she poured into a cup herself(!), she explained to me that she tried to reach for the serial but it was too high even with the chair. Needless to say, I just finished re-organizing the pantry!:)
What a lovely day today. Even despite no nap today, Abi cheerfully helped me plant flowers, went shopping, and played in the backyard running with the sprinkler on and ate hot dogs.












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Old couple...

This is something that I have to share! :-)
I just received a message from our friend who is watching Abi during the day.
This is how the conversation goes between Seth (4yr) and Abi almost 3:

Seth: " Go, go, go!"
Abi: " you don't know what you're doing!"
Seth:" just drive"
Abi: " no."
Seth: " I know what I'm doing just drive"
Abi: " fine"





That made my day!:)

JP


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Abi's 3rd Easter...

Yes, this year's Easter was all about the fun things, eggs, bunnies, crafts, family moments, and the biggest hit- chocolate treats! :).






Abi taking to her toys after a nap about bunny coming over to leave eggs in the backyard and more presents for her... :)




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Day off with mommy and daddy!

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Creativity...

After a bath and our regular bedtime routine, I put Abi to bed. She was tired today, so I made sure she's in bed by 8:15. When leaving she said: 'I got a question!'. I haven't heard her say that before, so I asked what question. She said: "I love you.."!
What a sweet end of the day.

She usually says: " look in my eyes" and when you look she says "I love you!". I Love when she does that out of nowhere.

Speaking of creativity.. Abi loves to paint and draw... Let me show you..

I'm attaching her first real painting she did with our friends for her daddy today:




The top right 'piece' looks like a bird to me. Artur can see a frog!?
Hmm. Definitely a bird!



Here's a drawing when she drew her first face with eyes and mouth.. This pic is just asking to be called some funny titles..
Presenting Abi's original drawing from December 18th, 2011 (2yrs,5 mths): "potato swimming with a whale'




Here's Abi's original drawing dated 1/18/2012. At first I didn't realize what it was but then I was blown away!


And check out Abi's easter eggs from earlier this week (4/2/12). I drew an oval and told her to draw her favorite things in it, but she started adding eyes, nose, ears, mouth and even 'pupa' (you can figure it out..:) )








We also made binoculars for Abi to help her 'hunt' for Easter eggs this coming Sunday. So fun!!




JP

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