- Living with congenital heart defects, coming up next on "Call The Doctor".

(upbeat music) - [Narrator] The region's premier medical information program, "Call The Doctor".

(upbeat music) - [Announcer] One in four babies is born with congenital heart defects or CHD's.

These defects can range from a small hole, to missing or poorly formed parts of the heart.

Experts don't know what causes congenital heart defects, but research shows the mother's health condition such as obesity or pre-existing diabetes may play a role.

Symptoms of CHD's include blue-tinted lips or nails, or tiredness when feeding.

Many children born with CHD's are now living into adulthood and leading independent lives with little or no difficulty, thanks to medical advancements.

However, people with CHD's should see a cardiologist regularly for checkups.

Learn more about living with congenital heart defects now on "Call The Doctor".

(upbeat music) - Welcome to "Call The Doctor" here on WVIA.

I'm Paola Giangiacomo, and thank you for joining us for our discussion on Congenital Heart Defects.

Joining us on the panel are Dr. Tapan Buch, he is a cardiology fellow who trains with the Wright Center for Graduate Medical Education.

Dr. Shirah Shore, she's a Pediatric Cardiologist at Geisinger.

And Dr. Pradeep Adumala, he is a Pediatrician and Primary Care Doctor at Lehigh Valley Health Network.

And I would like to remind our viewers that you can participate in the conversation at wvia.org/ctd.

And you can also submit your questions for future shows by emailing ctd@wvia.org or using the hashtag #WVIACTD.

And be sure to check our website for a listing of future programs and watch "Call The Doctor" episodes any time on the WVIA app.

And now we'll begin our discussion.

First of all, thank you all for joining us to talk about congenital heart defects.

I'll start with you Dr.

Shore, can you explain what a congenital heart defect is?

- Sure Paola, a congenital heart defect is a problem with the heart that a baby or a child is born with.

So it can range from anything from as a hole between one of the chambers of the heart.

It can be a narrowing of one of the blood vessels of the heart, or a problem with one of the valves of the heart, or it can actually be something quite complex where parts of the heart are missing where there's only one or two chambers of the heart.

So it's a variety of problems that a child can be born with.

- And Dr. Adumala, how would you detect whether a baby has a heart defect, right at birth?

- Yes, first of all we examine the baby, how the baby is acting in the first 24 hours of life.

And depending on the oxygenation status, like we check for the pulse ox and see how the baby's pulse ox is.

And is the baby turning blue, or is the baby's pink in color?

That's the best parameter we look in the babies.

And if the baby is pink and doing good and the examination of the heart sound give us a little bit of a musical sound, we'll say, it's just a murmur.

But the baby is not in a pink color but it's more of a bluish discoloration and we try to give the oxygenation and the baby's color is not improving, then we have to think about the cyanotic congenital heart disease.

- And how common is a congenital heart defect, Dr.

Shore in an infant?

- So one out of 800 babies born will have a congenital heart defect.

Again, there's a whole range of severity of the defect, but it is the most common congenital heart problem that any baby can be born with.

So it is fairly common in the neonatal population.

- And Dr. Buch, are congenital heart defects common in adults as well?

- Oh, for sure.

So, you know, the interesting thing is in adults that we either through our pediatricians know that the patient has a congenital heart defect or they're asymptomatic from it.

And over time we find out that these adults do have congenital heart defects.

Whether it may be through incidental findings, through testing or their body says, hey, I have a symptom I'm not used to, now it's showing up, this something is wrong.

So they go to their primary care provider and the primary care provider order some tests, and they find a congenital heart defect.

- [Paola] And what are some of the symptoms?

- Some of the symptoms are very common type symptoms.

So what's common is common.

So, Oh, I'm feeling more tired on just routine activities.

So I'm walking from here to the bathroom or here to the end of the driveway to get my mail and I'm feeling more tired.

It's just getting a little more progressive and a little more progressive than I used to be able to do.

That's a common sign.

Oh, I'm sleeping, I'm laying more flat at night, and I have some shortness of breath when I'm feeling that way.

Or I just noticed that my color isn't what it used to be.

I'm just doing less, I'm more fatigued.

So those are some common symptoms that start to pop up as we go through our adulthood life that can be a sign or symptom of, Oh, maybe something at play here with your heart.

- Hmm, and Dr. Adumala, what treatments are available for heart defects in infants?

- There are various treatments depending on the condition of the heart.

There is like very advanced technologies that have been developed, depending on each condition.

There is no specific procedure for all of them, every heart condition depending on the level of chambers involved, level of the vessels wall, level of the side of the heart in wall, they have a procedures that are developed.

And with this life expectancy has been increased tremendously, and it has been shown a success data, around 95%.

- So Dr.

Shore, if left untreated can a congenital heart defect as a baby progress into adulthood?

- So yeah, so nowadays we really are fairly aggressive about treating patients with congenital heart disease.

Because we know that the sequelae of leaving an untreated congenital heart problem later on in life can really affect not just length of life, but quality of life.

So we do, if we've identified a patient who has a congenital heart disease that needs to be taken care of, we usually do take care of it.

We do have patients that have congenital heart problems that can't really be fixed by what we call palliated, meaning that they still have issues with their hearts.

And those patients are now living into the adults years and need to have special people taking care of them.

Mainly adult congenital cardiologists that are specially trained to deal with patients that are now adults and also have congenital heart disease.

- It sounds like there have been advancements in congenital heart defects, Dr.

Shore?

- Yes, absolutely.

There are actually more adults with congenital heart disease living now than there are babies that are being born with congenital heart disease.

So it's actually an exploding new field that we are learning about now.

And we're really excited about in terms of dealing with adults with these residual congenital heart problems.

- Dr. Buch, do we know what causes these problems?

- So, you know, there's a multitude of reasons out there.

Can it be genetics?

That's a possibility, there are certain congenital heart diseases that are due to genetics.

Can it be lifestyle of the mom?

Yes, it can be, so obesity, high-risk behaviors can produce congenital heart disease.

Diabetes is another one, that in pregnancy or even before.

Could it be, there are reasons congenital heart disease can spring up later on in life with your own lifestyle.

So, are you not eating well?

Are you not sleeping well?

They can all trigger some congenital heart defects or diseases that we didn't even know about.

And they present with symptoms of varying degrees that lead us to find these congenital heart defects.

So there's a whole list of things that start from even before the baby is born, in utero, in the mother, to all the way to adulthood that we find.

- So Dr. Adumala, if a problem is found while the baby is in utero, can you treat that defect while the mother is still carrying the child?

- I'm not an expert of that, but usually when the babies are born, we try to classify which is, is it a cyanotic baby or acyanotic baby?

Depending on the lived expertise knowledge.

But most important is, we have to identify what exactly at the birth of the baby.

Like, is it a cyanotic heart problem or acyanotic?

- What does that mean, cyanotic?

- So usually, the oxygenation status.

We look at the oxygenation status of a baby.

Usually after a couple of minutes of the life of the baby, usually oxygenation status is 100%.

But if it is not improving about 85, 90, we call say, oxyhemoglobin saturation.

Which is very less and we try to give oxygen and it is not improving, that we consider as a cyanotic heart disease.

And depending on what kind of cyanotic heart disease is, there are like five most common.

First of all it's Tetrology of Fallot.

And then the second is, transmission of the great arteries.

And the third is, total anomalous pulmonary venous connection.

And fourth is, hypoplastic left heart syndrome.

These are the most common thing in a cyanotic.

- And what about acyanotic?

- Acyanotic usually, it's a defect in the chambers.

Like RSD, it's between a connection between two chambers of the low, like left chamber and the right chamber, called left ventricle septum, left ventricle and right ventricle, that's a VSD.

Or it can be a defect between two upper chambers called ASD, Atrial Septal Defect.

These are the two most common septal defects we come across.

And usually it causes acyanotic congenital heart disease, unless otherwise it is not treated in a certain age period.

It may turn into cyanotic heart disease.

- Okay.

And Dr.

Shore, is a heart defect something a parent or a mother can prevent while pregnant?

- Unfortunately, there really is no way to prevent these things.

Obviously, you know, keeping yourself healthy as Dr. Buch mentioned.

You know, PCP is definitely a high risk for having congenital heart problems.

Diabetes, particularly if it's not well controlled is a risk for having congenital heart problems.

So there are some modifying factors that some moms can do to try to prevent this, but you gotta remember that the heart is formed in the first 10 weeks of your pregnancy.

So for a lot of people, you may not even realize that you're pregnant when the heart is being formed.

And when these defects are first being formed.

So as much as I would like to say, yeah, you can modify your behaviors and prevent these things.

Unfortunately, a lot of times they are not preventable, at least in this stage of our technology.

- And what about prenatal tests?

Isn't there any other tests available that can detect any abnormalities?

- So yeah, fetal cardiologies are also very big and growing subspecialty of pediatric cardiology.

We can actually detect congenital heart problems as early as 13 weeks of your pregnancy in some instances.

For most of these complex congenital heart problems there's not a lot we can do about them.

However, with some of them there are some fetal interventions that can be done to try to prevent worsening of some of these very complex congenital heart disease.

And in addition it is very helpful to make these diagnosis prenatally so that the families can know what they're dealing with in a very calm and quiet setting rather than having the diagnosis made right after birth in a very hurried manner.

And also it helps to prepare where the delivery is going to be made.

What sort of management issues may come up during and after the delivery.

So we do like to make these diagnoses prenatally because it really makes the course of the baby much more smooth than if it is done sort of postnatally.

- And Dr. Adumala, does the baby have to be followed more closely throughout his or her life if he or she is born with a defect?

- Yeah, absolutely, absolutely, there is no question about it.

Most important, when somebody has any heart defects the most important thing is, the baby has poor feeding issues.

And you have to make sure where exactly the defects are.

And depending on the baby, we give high calorie foods and make sure the follow-up what kind of medication they needed.

Depending on the age of the babies also, because most of the surgeries are do according to the age, and complexity of that heart condition.

So as a primary care pediatrician we always make sure proper followup of the baby to the specialist is very, very important.

- And as an adult Dr. Buch, what are some of the complications that someone as an adult may have with a congenital heart disease?

So, you know, I like to take a second to tell you about how I was trained at the Wright Center for Community Health, okay?

So my attendings always telling me this, they say, listen, pretend your heart is your house and it has four rooms in it, okay?

And just like a house with four rooms there's a pipe leading into the house.

It's two pipes or multiple pipes.

And that feeds the house, let's pretend it's water, blue.

And just as your house has four rooms, your heart has four rooms typically, okay?

In a typical adult patient.

And it has valves and doors and just like a house.

And let's pretend that on your way out of your house you have a couple of pipes that lead out of your house that pump all the good stuff away.

And in your house itself you have a water pipe and you have a gas pipe.

And in your heart itself, and in your house itself you have electrical, circuitry and wires.

So any one of those areas, as you can imagine over time if you don't find it as your baby or as your child into adolescents--- - [Paola] right?

- Or even if you do find it and intervene on it, right?

With our pediatric, cardiologists and surgeons and pediatricians.

Let's say you find it in adulthood, you're gonna have wear and tear in your house.

You're gonna have wear and tear and those congenital defects will show up.

Or the ones that are fixed are going to say, hey, do we need to do something about it?

So, you know, in the world of adult medicine we have so many tools available to us to fix those issues.

We have all sorts of screening tools so we can do a simple echocardiogram and an EKG.

Let's see how the electricity in your house is running.

We can do an echocardiogram, let's see how the structure and the valves are doing.

Let's see how it's pumping.

Let's see how that fix is doing.

We can do more invasive tests.

We can do a catheterization which is taking a wire, going into your RS and taking a picture of your arteries, seeing how your pipes are doing.

So there are all sorts of tests that we can do to see if the fix is still working, or do we need to intervene on it and call our surgery friends or our inner invasive friends to help fix the problems.

Any house needs fixing after time.

It's just a matter of catching the symptoms before it falls apart.

- What if they ignore the symptoms?

Can it cause stroke, heart failure, delayed breathing?

- Right, so unfortunately a lot of times, you know, we, of course, you know, we don't really take notice of the symptoms.

Or if we have the symptoms we say, hey, it's nothing I'm just gonna keep going about my business and that's it.

I just toughen up and I say, I continue on my way, cause we have to, we have no choice.

That's our daily in and out rat race, right?

So, you know, over time it may lead to symptoms of heart failure exactly.

It may lead to symptoms where all of a sudden you have a stroke.

Or because a clot passes through that septal defect that we didn't know about or we knew about, and it just opened up again or we didn't know about it at all.

So unfortunately these are things that happen and we're seeing it in more and more prevalence.

However, our technology and the ability to treat has gotten so much better.

We have advanced rapidly in the field of medicine.

We're gonna keep making advancements.

And our guidelines are forever updating.

We are able to handle a lot of the situations that we were not able to handle 10, 15 years ago.

We can handle it safely now.

So do seek help, come to the hospital, see your family doctor, that's where it starts.

See your pediatrician, that's where it starts.

They can help you lead to the specialist and keep you out of the hospital.

If you need the hospital, we're there for you as well.

- Yeah, it's scary cause sometimes you hear stories about people running a marathon and all of a sudden they drop dead in the middle of the run.

And you wonder, did they have a defect, like what's going on in that situation?

Did they miss the signs or it was just their time.

- So let me tell you a story.

So I had a patient, you know, bless his soul.

He ran a marathon every single year, a couple of marathons every single year, 72 years old now.

All of a sudden he says, hey, I'm having shortness of breath that I never used to have.

I'm running these marathons and now I can't complete a marathon.

That's a brand new thing, up until two years ago he was running marathon.

So we did our routine tests.

We got an EKG, looked good, the electricity of the house is fine.

We take a look at the echocardiogram.

We notice, hey, something, an echocardiogram is just like I said, evaluating the structure and how your valves are doing.

And it said something was a little off.

So we took a further look with another, a special type echocardiogram where we take a scope and we go into your food pipe and we take a look.

He had a four-leaf clover aortic valve.

four leaflets on your valve, normally you have three.

That was causing him to have these delayed symptoms.

So yes, there are, that's one on the extreme.

Luckily we were able to catch it, he's a marathon runner and we caught it in time.

- So don't ignore any signs--- - Don't ignore any signs.

If you can't do what you're able to do and it's starting to get progressive and worse don't ignore it.

- Okay.

Dr.

Shore, can congenital heart defects be prevented at all?

- I mean, generally speaking they can't be prevented, unfortunately.

In general as I mentioned before, they are formed very early on in pregnancy.

So therefore there's not a lot you can do about them.

But again, maintaining, you know, mothers, you know, good health, general good health.

If it is the baby, we like the babies to be full term, we like the babies to be growing well.

Any other complicating factors, with a baby with complex congenital heart disease, will make any sort of surgery or any sort of intervention that we need to do with these children much more complex.

So if we can get these babies to term, if we can get these babies in a good nutritional state before they need any surgeries or any interventions then the baby will do much better than if they were otherwise.

- We have a Facebook question.

A viewer would like to know Dr. Adumala, if a baby is born with a heart murmur, can they outgrow that?

Or is that something that would need surgery?

- That's a good question.

Depending on how much is a murmur, like what kind of murmur it is?

Most of the murmurs are innocent and they will close by itself.

But some murmurs, usually if you have a opening of the wall, as my friend said, there's a gap between two chambers, the blood flow goes from high pressure to low pressure.

So as the baby grows, sometimes the wall closes by itself, or the chamber closes, develops itself to normal.

But sometimes it can then, reverse of the flow can happen.

So usually when there is a reverse of flow from right to left chamber, then you can look at the symptoms develops.

So most of the murmurs are treated, goes by itself, but some of the murmurs can get worse.

So most of the pediatricians, whenever they hear a murmur they try towards symptoms.

Is the baby's eating good, is the baby having shortness of breath or any sweating happening, any leg swelling happening?

We look at, is the baby's gaining enough weight or not?

We all look at the symptoms depending on the report of the cardiologist.

That's the way usually we do it.

Most of the murmurs, like 90% of the murmurs usually are innocent murmurs.

But some murmurs which have a really high grade murmurs, we are to refer to the cardiologist.

- Okay.

Thank you.

Once again, I would like to thank our panels for participating in our discussion on congenital heart defects.

We're all out of time.

For more information and resources on this topic, visit wvia.org/ctd.

I'm Paola Giangiacomo for "Call The Doctor".

Thank you for watching.

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