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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