242. ACHD — Atrial Septal Defects With Dr. Richard Krasuski
Highlights
- Atrial Septal Defects
Summary:
The cundum ASD is the most common type of atrial septal defect. Primum defects are associated with a cleft in the left atial valve. sinus vanosis defects account for about 5 to 10% of atrial level defects.
Transcript:
Speaker 2
Of course, there's technically four types of atrial septal defects, subcundum ASDs, primum ASDs, sinus vanosis defects, and coronary sinus defects. It's helpful to conceptualize them by location. Note that the latter two aren't actually at the atrial septum. First up is the cundum ASD, which is the most common type of atrial septal defect. They account for about 75% of atrial septal defects. So cundum defects occur in the center of the atrial septum at the fossa ovalus and can extend in pretty much any direction on the atrial septum. Primum defects account for about 15 to 20% of ASDs and are located at the inferior portion of the atrial septum and are within the spectrum of atrial ventricular septal defects, also known as endocardial cushion defects or canal defects. Primum defects are also associated with a cleft in the left atial valve, as well as ventricular septal defects and subaortic stenosis. Moving on to the third type of ASD is the sinus vanosis defects, which accounts for about 5 to 10% of atrial level defects and are located near the SVC right atrial junction or very rarely at the mouth of the inferior vena cava. (Time 0:07:54)
- Three Types of ASD
Summary:
The third type of ASD is the sinus vanosis defect, which accounts for about 5 to 10% of atrial level defects. Primum defects are also associated with a cleft in the left atial valve as well as ventricular septal defects and subaortic stenosis. A coronary sinus defect is a communication between the coronary sinus and left atrium. In essence, it's kind of an unroost coronary sinus.
Transcript:
Speaker 2
Primum defects account for about 15 to 20% of ASDs and are located at the inferior portion of the atrial septum and are within the spectrum of atrial ventricular septal defects, also known as endocardial cushion defects or canal defects. Primum defects are also associated with a cleft in the left atial valve, as well as ventricular septal defects and subaortic stenosis. Moving on to the third type of ASD is the sinus vanosis defects, which accounts for about 5 to 10% of atrial level defects and are located near the SVC right atrial junction or very rarely at the mouth of the inferior vena cava. Around 80 to 90% of sinus vanosis defects are associated with partial anomalous pulmonary venous return. Cartoon nerds, you should definitely check out episode 106 for a fascinating case from our Boston colleagues, which highlights this. The final type of ASD is a coronary sinus defect, which accounts for less than 1% of all atrial level defects. A coronary sinus defect is a communication between the coronary sinus and left atrium. In essence, it's kind of an unroost coronary sinus. (Time 0:08:33)
- Atrial Septal Defects
Summary:
Atrial septal defects account for about 10 to 15 percent of all congenital heart diseases. The incidence is approximately 1 in 1500 live first, and they're usually about 1.5 times more common in women compared to men. There are a couple of risk factors for having atrialseptal defects, including family history,. Certain maternal medications, including some SSRIs like peroxetine, as well as some antileptic medications such as Velcroac acid.
Transcript:
Speaker 2
You are definitely on the right track there, Dan. Atrial septal defects account for about 10 to 15 percent of all congenital heart diseases. And the incidence is approximately 1 in 1500 live first, and they're usually about 1.5 times more common in women compared to men. When they're diagnosed in childhood, studies have shown that nearly 100 percent of defects less than 3 millimeters will close by school age, so 3 to 5 years of age. Similarly, 90 percent of defects between 3 and 5 millimeters and 80 percent of defects between 5 and 8 millimeters will close in that timeframe as well. However, defects greater than 8 millimeters are unlikely to close. Defect size often increases as somatic growth occurs in mid to late childhood. There are a couple of risk factors for having atrial septal defects, including family history, as well as certain maternal medications, including some SSRIs like peroxetine, as well as some antileptic medications such as Velcroac acid. There's also a handful of genetic associations with atrial septal defects, including Holt-Orem syndrome, trisomy 21, and Elastin-Cravolt syndrome. (Time 0:11:35)
- Understanding the Anatomic Features of Different Defects
Summary:
An abnormal septation between the pulmonary vein on the lower end and the inferior vena cava. An unroofed coronary sinus where there's a lack of septations between the inferior left atrium and the roof of the coronary sinus. And you can have a mixing of blood at the left atrium, which causes oxygen desaturation.
Transcript:
Speaker 1
So again, much like a cleft as well. Now, those are the predominant types of defects, but you've already mentioned that there's two other types as well. You can have your sinus vanosis atroceptal defect, where this is really a failure of septation between the pulmonary veins and the superior vena cava and the right atrium. But people often forget you can also get an inferior sinus vanosis defect, where the same thing also can occur between an abnormal septation between the pulmonary vein on the lower end and the inferior vena cava as well. And finally, there's the unroofed coronary sinus where there's a lack of septation between the inferior left atrium and the roof of the coronary sinus. And this allows a communication between the left and the right atrium. And you can have a mixing of blood at the left atrium. So for this type of a defect, often we'll see oxygen desaturation as part of it, because there's so much mixing in that coronary sinus, which remembers the lowest oxygenated blood in your body, because it's coming from your coronary circulation, where you extract the most oxygen. At that point, you can get mixing and you drop your oxygen saturation. (Time 0:16:24)
- The Anatomy of Atrial Septal Defects
Summary:
People are asymptomatic for the majority of their lives if they have a small atrial septal defect. Those with moderate or a large defect may not present with symptoms until the fourth decade of life. Physical exam findings include elevated jugular venous pressure and a prominent peristernal impulse.
Transcript:
Speaker 3
Wonderful. Thank you so much, Dr. Krsucke. I think it's so imperative to understand the anatomic features of all these different defects. And it's really helpful one to understand clinically how patients present into the other milieu. For example, the left av valve cleft or the anomalous pulmonary vena turture can really be much more understandable when you understand the anatomic etiology of these defects. So Sarah, bring us back historically a little bit. Back in the 1800s, of course, they didn't have things like electrocardiograms or echocardiograms or cardiac cats. So how did they, you know, clinically diagnose atrial septal defects in these patients?
Speaker 2
Well, this is where the art of medicine really comes in. There are some classic physical exam findings, but for the most part, people are asymptomatic for the majority of their lives if they have a small atrial septal defect. Those with moderate or a large defect may not present with symptoms until the fourth decade of life. Symptoms may include shortness of breath, exercise intolerance, or recurrent chest infections. Physical exam findings include elevated jugular venous pressure and a prominent peristernal impulse. The murmur that you'll hear consists of a wide fixed split S2 with a systolic ejection murmur due to increased blood flow across the pulmonary valve. If it is a large defect, you may have a diastolic flow rumble across the tricuspid valve as well. And if there's pulmonary hypertension, the S2 may be particularly loud. One thing to note is that the murmur is not from blood going across the atrial septo. Great. (Time 0:17:36)
- Is There a Jugular Vein Pulsation?
Summary:
People are asymptomatic for the majority of their lives if they have a small atrial septal defect. Symptoms may include shortness of breath, exercise intolerance, or recurrent chest infections. Physical exam findings include elevated jugular venous pressure and a prominent peristernal impulse.
Transcript:
Speaker 2
Well, this is where the art of medicine really comes in. There are some classic physical exam findings, but for the most part, people are asymptomatic for the majority of their lives if they have a small atrial septal defect. Those with moderate or a large defect may not present with symptoms until the fourth decade of life. Symptoms may include shortness of breath, exercise intolerance, or recurrent chest infections. Physical exam findings include elevated jugular venous pressure and a prominent peristernal impulse. The murmur that you'll hear consists of a wide fixed split S2 with a systolic ejection murmur due to increased blood flow across the pulmonary valve. If it is a large defect, you may have a diastolic flow rumble across the tricuspid valve as well. And if there's pulmonary hypertension, the S2 may be particularly loud. One thing to note is that the murmur is not from blood going across the atrial septo. Great.
Speaker 3
Thank you so much, Sarah. Dr. Krasuski, anything else you look for on your physical exam for these patients?
Speaker 1
Yeah. So if you actually start with a jugular venous pulsation, which you'll notice is remember with the right side, typically you're going to get a more prominent A wave than your V wave. And in somebody with a secundumatroceptal defect, you'll see a more prominent V wave than the A wave. It does take some practice, I think, to get used to looking at the jugular veins and looking at which pulsation is greater than the other. (Time 0:18:18)
- Is There a Split Second Heart Sound?
Summary:
In somebody with a secundumatroceptal defect, you'll see a more prominent V wave than the A wave. It does take some practice, I think, to get used to looking at the jugular veins and looking at which pulsation is greater than the other. So these are all good physical exam findings.
Transcript:
Speaker 1
Yeah. So if you actually start with a jugular venous pulsation, which you'll notice is remember with the right side, typically you're going to get a more prominent A wave than your V wave. And in somebody with a secundumatroceptal defect, you'll see a more prominent V wave than the A wave. It does take some practice, I think, to get used to looking at the jugular veins and looking at which pulsation is greater than the other. But if you try to time it, you'll notice that. I think it's a great point that you brought up about the murmur that you hear. Remember it is flow across the pulmonary outflow tract. It's not flow across the atrial septal defect. And then just with regard to the splitting, I think it's important to think about how you get splitting in the first place. Remember that when you breathe in, right, what happens? You drop your intrathearacic pressure, you suck blood back into your heart, you lengthen out the amount of time that blood is pushed out into the pulmonary outflow, and you get that particular, you know, splitting in the sound. And then when you breathe out, basically the opposite happens. Now, just recall that what happens in somebody with a septal defect, you've got basically overload of that right side. When you breathe in, you get equalization between the two sides. And so, the splitting that you get will not change with inspiration. That's why we refer to it as a fixed split second heart sound. So these are all good physical exam findings. Again, if you see somebody late in their course, you might see things such as cyanosis. Let's say you pick up somebody in whom their shunt is reversed because they develop severe pulmonary hypertension. (Time 0:19:24)
- Atrial Septal Defects - What Are the Diagnostic Findings?
Summary:
The ACHD series is really highlighting the importance of a great physical exam. Many patients are asymptomatic and adulthood and may not realize that you even have a congenital defect until later in life. Your EKG findings really depend upon the type of atrial septal defect. With a primum ASD, you'll see a left axis deviation and counter clockwise looping. If the defect is large, one might see right heart enlargement or prominent main pulmonary artery on chest x-ray.
Transcript:
Speaker 4
Wow. Dr. Krasiski and Sarah, I don't think I've ever heard a more comprehensive and exciting explanation for the physical exam findings that are associated with ASD, particularly with your explanation of the splitting and also that ASD murmurs, not from the ASD flow actually, but rather, it's the associated higher pulmonary flows that you're hearing. So that was fantastic. And in general, the ACHD series is really highlighting the importance of a great physical exam, especially as many of these patients are asymptomatic and adulthood and may not realize that you even have a congenital defect until later in life. So Sarah, what are some supportive diagnostic findings that we can see on the ECG or ECHO and Cardi sex or an EKG will probably be normal.
Speaker 2
However, if the defect is large, one might see right heart enlargement or prominent main pulmonary artery and increased pulmonary vascularity on chest x-ray. Your EKG findings really depend upon the type of atrial septal defect. In secundum atrial septal defects, you may see an incomplete right bundle branch block, right atrial enlargement, and right axis deviation. However, with a primum ASD, you'll see a left axis deviation and counter clockwise looping. (Time 0:21:25)
- Atrial Septal Defects - What Are the Diagnostic Findings?
Summary:
Your EKG findings really depend upon the type of atrial septal defect. With a primum ASD, you'll see a left axis deviation and counter clockwise looping. An EKG for a sinusis venosis defect will have inverted p-waves in the inferior leith suggesting an absent or deficient sinus node. If there's severe pulmonary hypertension, RSR prime will be replaced by q-waves with deep inverted t-waves.
Transcript:
Speaker 2
However, if the defect is large, one might see right heart enlargement or prominent main pulmonary artery and increased pulmonary vascularity on chest x-ray. Your EKG findings really depend upon the type of atrial septal defect. In secundum atrial septal defects, you may see an incomplete right bundle branch block, right atrial enlargement, and right axis deviation. However, with a primum ASD, you'll see a left axis deviation and counter clockwise looping. So just to reiterate that with a secundum ASD, you'll see a right axis deviation, but with a primum ASD, you'll see a left axis deviation. Finally, an EKG for a sinusis venosis defect will have inverted p-waves in the inferior leith, suggesting an absent or deficient sinus node. In any of these, if there's severe pulmonary hypertension, RSR prime will be replaced by q-waves and tall monophasic R-waves with deep inverted t-waves. Excellent.
Speaker 3
Thank you so much, Sarah. Dr. Fursuski, anything else on ECG that you look for to glean some clinical context about these patients? (Time 0:22:15)
- Shunting Across the Atrial Septal Defect?
Summary:
A QPQS, which we get all very excited about, is a moving target in the natural history of this disease. There's really two things that determine your degree of shunting. One is the size of the defect and then secondly is the compliance of the two ventricles. So let's say you have a very compliant right side and left side. You can have a larger defect than you can have a shunt that may be moderate sized for years.
Transcript:
Speaker 3
Before we get into our first case, Dr. Kuzuski, can you just briefly touch on shunting across the atrial septal defect and what factors really determine the direction of the shun?
Speaker 1
That's a great question. And it's important to recognize that a QPQS, which we get all very excited about, is a moving target in the natural history of this disease. So remember that when you talk about a shunt, you're describing how much extra blood flow the pulmonary vasculature is receiving to the systemic vasculature. So there's really two things that determine your degree of shunting. One is the size of the defect and then secondly is the compliance of the two ventricles. So let's say you have a very compliant right side and left side. And in fact, you can have a larger defect than you can have a shunt that may be moderate sized for many years. (Time 0:26:00)
- Shunting Across the Atrial Septal Defect?
Summary:
A QPQS, which we get all very excited about, is a moving target in the natural history of this disease. There's really two things that determine your degree of shunting. One is the size of the defect and then secondly is the compliance of the two ventricles. So if you develop systemic hypertension, now your left ventricle becomes less compliant and your left atrial pressure gets higher, you may actually shunt more left to right.
Transcript:
Speaker 3
Before we get into our first case, Dr. Kuzuski, can you just briefly touch on shunting across the atrial septal defect and what factors really determine the direction of the shun?
Speaker 1
That's a great question. And it's important to recognize that a QPQS, which we get all very excited about, is a moving target in the natural history of this disease. So remember that when you talk about a shunt, you're describing how much extra blood flow the pulmonary vasculature is receiving to the systemic vasculature. So there's really two things that determine your degree of shunting. One is the size of the defect and then secondly is the compliance of the two ventricles. So let's say you have a very compliant right side and left side. And in fact, you can have a larger defect than you can have a shunt that may be moderate sized for many years. And then as you get older, your shunt can increase. So if you develop, let's say, systemic hypertension, now your left ventricle becomes less compliant and your left atrial pressure gets higher, you may actually shunt more left to right. People, I think, forget about that pathophysiology. And that explains why somebody can present at age 60 or 70 or even 80 with an atrial septal defect in a large shunt. (Time 0:26:00)
- Is There a Two-to-One Shunt?
Summary:
A QPQS is a moving target in the natural history of this disease. So there's really two things that determine your degree of shunting. One is the size of the defect and then secondly is the compliance of the two ventricles. And as you get older, your shunt can increase. That explains why somebody can present at age 60 or 70 or even 80 with an atrial septal defect in a large shunt.
Transcript:
Speaker 1
That's a great question. And it's important to recognize that a QPQS, which we get all very excited about, is a moving target in the natural history of this disease. So remember that when you talk about a shunt, you're describing how much extra blood flow the pulmonary vasculature is receiving to the systemic vasculature. So there's really two things that determine your degree of shunting. One is the size of the defect and then secondly is the compliance of the two ventricles. So let's say you have a very compliant right side and left side. And in fact, you can have a larger defect than you can have a shunt that may be moderate sized for many years. And then as you get older, your shunt can increase. So if you develop, let's say, systemic hypertension, now your left ventricle becomes less compliant and your left atrial pressure gets higher, you may actually shunt more left to right. People, I think, forget about that pathophysiology. And that explains why somebody can present at age 60 or 70 or even 80 with an atrial septal defect in a large shunt. And you often get the question, well, how could they possibly have a two to one shunt? They're 75 years of age. And in fact, what's happened is they had fairly normal compliance of their two sides. And so they didn't have that much shunting. And as they got older, the left-sided compliance got less. And therefore, their pressure got higher. And they started shunting more. I think most of us are aware of the opposite, which is somebody who develops pulmonary hypertension. In that case, your right-sided compliance starts to go down. And now your right side of pressures get higher. And as a consequence, your LA to RA gradient is not that much. And so your shunt decreases. So again, it's a moving target. A QPQS at any point in time does not necessarily tell you what it's been or which direction it's going. (Time 0:26:11)
- Atrial Septal Defect
Summary:
A young lady presenting with fatigue and exercise intolerance. It's interesting that this is a very common presentation. I've seen this a lot and I've seen a lot of misdiagnosis. We say if it's less than 10 millimeters, we refer to that as a small defect. A moderate-sized defect is somewhere between 10 and 20 millimeters. The 14 is certainly in the moderate category.
Transcript:
Speaker 1
Well, thanks, Sarah. First of all, it's a young lady presenting with fatigue and exercise intolerance. It's interesting that this is a very common presentation. I've seen this a lot and I've seen a lot of misdiagnosis. I've seen patients who are told they have asthma and are tried on inhalers with no improvement. I've seen patients who were treated for depression because in fact, they had functional limitation and they were not too happy about it. I've seen patients again treated for thyroid abnormalities, a variety of different things. It's important to recognize that if you are functionally limited and young, that that's not normal and that it's obviously important to have a good listen and make sure that you don't hear anything suggesting an atrial septal defect because this story is common. Now, as far as sizing of ASD's, typically, we say if it's less than 10 millimeters, we refer to that as a small defect. A moderate-sized defect is somewhere between 10 and 20 millimeters and then a large is greater than 20 millimeters, but 14 is certainly in the moderate category. (Time 0:30:26)
- Atrial Septal Defect
Summary:
This is a very common presentation. I've seen this a lot and I've seen a lot of misdiagnosis. It's important to recognize that if you are functionally limited and young, that that's not normal. The ejection fraction worries me a little bit that it's down, but the tricuspid regurgitation is mild. This is somebody with at least mildly dilated heart here.
Transcript:
Speaker 1
Well, thanks, Sarah. First of all, it's a young lady presenting with fatigue and exercise intolerance. It's interesting that this is a very common presentation. I've seen this a lot and I've seen a lot of misdiagnosis. I've seen patients who are told they have asthma and are tried on inhalers with no improvement. I've seen patients who were treated for depression because in fact, they had functional limitation and they were not too happy about it. I've seen patients again treated for thyroid abnormalities, a variety of different things. It's important to recognize that if you are functionally limited and young, that that's not normal and that it's obviously important to have a good listen and make sure that you don't hear anything suggesting an atrial septal defect because this story is common. Now, as far as sizing of ASD's, typically, we say if it's less than 10 millimeters, we refer to that as a small defect. A moderate-sized defect is somewhere between 10 and 20 millimeters and then a large is greater than 20 millimeters, but 14 is certainly in the moderate category. The other findings here, normal, right ventricular end-diastolic volume, we do a lot of MRIs for a variety of different congenital heart lesions, but a normal life-intricular end-diastolic volume index is going to be right around 100, somewhere between 80 and 100 milliliters per meter squared. This is somebody with at least mildly dilated heart here. The ejection fraction worries me a little bit that it's down, but the tricuspid regurgitation is mild. As far as how do we approach this from a guideline standpoint? Should this be closed or should it not? The guidelines say you need A symptoms, which she has. B, you need to have predominant left to right shunting with a QPQS that's greater than 1.5 to 1, which is the second thing we have. And then the third thing you need to make sure is that there's not significant pulmonary hypertension. (Time 0:30:26)
- ASD and Pneumonia
Summary:
You got to think about the patient holistically. And what else is going on in their cardiac structure and physiology? I can't just see a hole, plug the hole, but actually think of the patient in a very comprehensive way to get them to bank for their buck. That makes so much sense. And device embolization is obviously not ideal. It's important to recognize that that works as a pop off valve for patients with pulmonary hypertension. But you also can get a pop off valves from the left side.
Transcript:
Speaker 4
That makes so much sense. And device embolization is obviously not ideal. And of course, you got to think about the patient holistically. And what else is going on in their cardiac structure and physiology? I can't just see a hole, plug the hole, but actually think of the patient in a very comprehensive way to get them to bank for their buck.
Speaker 1
And there's one other thing I would add as you were mentioning that and that is of course pulmonary hypertension. I think you correctly noted and the guidelines have changed recently. And one of the reasons they changed because of the fears of closing a quote unquote pop off valve for the right side, you know, it's important to recognize that that works as a pop off valve for patients with pulmonary hypertension. But you also can get a pop off valve from the left side. So I've seen cases where an ASD is closed percutaneously and the patient has moderate mitral regurgitation. And that becomes more significant because now there's not a pop off for the left side as well. So either of those clinical situations is important to surmise as well and evaluate before you go ahead and just willy-nilly place a device. (Time 0:35:59)
- Trans Thoracic Echo for Pulmonary Hypertension
Summary:
Dr. Przucchi, anything to add a method point in the clinical case? Yeah, so great description of this particular case. This is a very common one. One of the things I would cue you into is that I've seen several patients get referred to me for pulmonary hypertension with almost the same clinical story. A lot of times they have slightly elevated pulmonary pressures.
Transcript:
Speaker 3
Great, thank you so much Sarah. That was a great source and an option about how you would approach this patient. Dr. Przucchi, anything to add a method point in the clinical case?
Speaker 1
Yeah, so great description of this particular case. This is a very common one. One of the things I would cue you into is that I've seen several patients get referred to me for pulmonary hypertension with almost the same clinical story and a lot of times they have slightly elevated pulmonary pressures. One of the things that's important to recognize that if you see somebody who's been followed for pulmonary hypertension and their pulmonary pressures have not changed very much over the years but their right heart continues to be dilated, think that they may have missed a sinus venousist ASD because I've seen at least two or three of these who were treated for years with advanced therapies for pulmonary hypertension in whom we eventually found this type of a defect to be present. This is a common one that can be missed with trans thoracic echo because you just can't see it. (Time 0:44:28)
- Trans Thoracic Echo for Pulmonary Hypertension
Summary:
The art of looking at pulmonary veins with a TEE is something that's been slowly lost over time. This is a common one that can be missed with trans thoracic echo because you just can't see it. It requires really somebody who's diligent to kind of go through each pulmonary vein but you can very nicely demonstrate on a TEE, the defect and you can demonstrate that the pulmonary veins drain on the wrong side.
Transcript:
Speaker 1
Yeah, so great description of this particular case. This is a very common one. One of the things I would cue you into is that I've seen several patients get referred to me for pulmonary hypertension with almost the same clinical story and a lot of times they have slightly elevated pulmonary pressures. One of the things that's important to recognize that if you see somebody who's been followed for pulmonary hypertension and their pulmonary pressures have not changed very much over the years but their right heart continues to be dilated, think that they may have missed a sinus venousist ASD because I've seen at least two or three of these who were treated for years with advanced therapies for pulmonary hypertension in whom we eventually found this type of a defect to be present. This is a common one that can be missed with trans thoracic echo because you just can't see it. A couple of things additionally to mention. I like the TEE. I think unfortunately the art of looking at pulmonary veins with a TEE is something that's been slowly lost over time. It requires really somebody who's diligent to kind of go through and look at each pulmonary vein but you can very nicely demonstrate on a TEE, the defect and you can demonstrate that the pulmonary veins drain on the wrong side. (Time 0:44:40)
- Re-Operation of an AV Canal
Summary:
Most common reasons for re-operation is residual defects either ASD's or VSD's on the top of the list cleft valve. Second most common reason these folks need re-operation are because their cleft has gotten significant and they have severe valve regurgitation. Thirdly it's important to recognize that the septum is completely shifted in these patients.
Transcript:
Speaker 1
Rings and all sorts of other stuff but you know it's important to recognize that you're doing this repair on somebody that's so small so these can be really really challenging not because the repair itself is challenging but because the size of the heart that you're working on is so small but some things to think about so when you tell me about an AV canal that's been repaired you know what are the most common reasons for re-operation well it turns out residual defects either ASD's or VSD's on the top of the list cleft valve so the clefts can be repaired early and sometimes you leave a cleft behind and that cleft gets bigger over time just kind of wear and tear and the mitral regurgitation can get worse so the second most common reason these folks need re-operation or re-intervention is because their cleft has gotten significant and they have severe valve regurgitation and then thirdly it's important to recognize that the septum is completely shifted in these patients so we often talk about the valves and how you can tell an AV canal apart from somebody who doesn't have an AV canal and one of the things we can we talk about is how there's apical displacement of the tricuspid valve normally well in an AV canal there's not so the valves kind of line up so that's one of the hints you can (Time 0:56:49)
- Re-Operation of an AV Canal
Summary:
Most common reasons for re-operation is residual defects either ASD's or VSD's on the top of the list cleft valve. The septum is completely shifted in these patients so we often talk about the valves and how you can tell an AV canal apart from somebody who doesn't have one.
Transcript:
Speaker 1
Of the heart that you're working on is so small but some things to think about so when you tell me about an AV canal that's been repaired you know what are the most common reasons for re-operation well it turns out residual defects either ASD's or VSD's on the top of the list cleft valve so the clefts can be repaired early and sometimes you leave a cleft behind and that cleft gets bigger over time just kind of wear and tear and the mitral regurgitation can get worse so the second most common reason these folks need re-operation or re-intervention is because their cleft has gotten significant and they have severe valve regurgitation and then thirdly it's important to recognize that the septum is completely shifted in these patients so we often talk about the valves and how you can tell an AV canal apart from somebody who doesn't have an AV canal and one of the things we can we talk about is how there's apical displacement of the tricuspid valve normally well in an AV canal there's not so the valves kind of line up so that's one of the hints you can have that somebody's had an AV canal repair but also because of these abnormal septum you can actually have outflow obstruction so it's not unusual to develop LV outflow tract obstruction basically related to the anatomy or to the repair so that's the (Time 0:57:04)
New highlights added February 26, 2023 at 6:11 PM
- Re-Operation of an AV Canal
Summary:
Most common reasons for re-operation is residual defects either ASD's or VSD's on the top of the list cleft valve. Second most common reason these folks need re-operation are because their cleft has gotten significant and they have severe valve regurgitation. Thirdly it's important to recognize that the septum is completely shifted in these patients so you can tell an AV canal apart from somebody who doesn't have one.
Transcript:
Speaker 1
Stuff but you know it's important to recognize that you're doing this repair on somebody that's so small so these can be really really challenging not because the repair itself is challenging but because the size of the heart that you're working on is so small but some things to think about so when you tell me about an AV canal that's been repaired you know what are the most common reasons for re-operation well it turns out residual defects either ASD's or VSD's on the top of the list cleft valve so the clefts can be repaired early and sometimes you leave a cleft behind and that cleft gets bigger over time just kind of wear and tear and the mitral regurgitation can get worse so the second most common reason these folks need re-operation or re-intervention is because their cleft has gotten significant and they have severe valve regurgitation and then thirdly it's important to recognize that the septum is completely shifted in these patients so we often talk about the valves and how you can tell an AV canal apart from somebody who doesn't have an AV canal and one of the things we can we talk about is how there's apical displacement of the tricuspid valve normally well in an AV canal there's not so the valves kind of line up so that's one of the hints you can have that somebody's had an (Time 0:56:50)
- ASD and Cardiomyopathy
Summary:
Pulmonary vascular remodeling can even be seen in individuals in their secondary than third decade of life but it may not be hemodynamically significant until many many years later. Even if you have your ASD repaired there's roughly about a 2 to 3% chance that you can still develop pulmonary hypertension.
Transcript:
Speaker 2
Kuzuski mentioned that it's particularly high in those that have trisomy 21 with unrepaired defects pulmonary vascular remodeling can even be seen in individuals in their secondary than third decade of life but it may not be hemodynamically significant until many many years later and as the ventricular compliance and pulmonary vascular resistant change over time the patients can develop isomingur syndrome where there's then increased morbidity and mortality in patients with moderate to large defects patients with ASD is also of a higher likelihood of developing atrial fibrillation or atrial flutter which can then lead to heart failure and all the other complications of strokes and they also have a lower pv02 compared to the peers without atrial septal defects and that goes into some of the presenting symptoms for ASD as we mentioned one of them was exercise intolerance. Dr.
Speaker 4
Kuzuski do you have anything to add to that?
Speaker 1
Yeah it's just to mention the pulmonary vascular remodeling because you know I think people think that if you close the atrial septal defect you're kind of out of the woods you never have to worry about it again and it's important to recognize that even if you have your ASD repaired there's roughly about a 2 to 3% chance that you can still develop pulmonary hypertension and from from what we know about pulmonary (Time 1:00:59)
- Atrial Septal Defect Repair
Summary:
Even if you have your ASD repaired there's roughly about a 2 to 3% chance that you can still develop pulmonary hypertension. Even with a successful repair the surgical and medical literature suggests that there's somewhere between a 10 and 15% chance of residual shunts as well. Those may get worse or be associated with things like paradoxical embolism and other problems so again this is a group of patients that should ideally be at least followed up some extent over time.
Transcript:
Speaker 1
Yeah it's just to mention the pulmonary vascular remodeling because you know I think people think that if you close the atrial septal defect you're kind of out of the woods you never have to worry about it again and it's important to recognize that even if you have your ASD repaired there's roughly about a 2 to 3% chance that you can still develop pulmonary hypertension and from from what we know about pulmonary hypertension if you have pH with a closed defect it's actually a worse prognosis than PAH with an open defect so it's important to know that these patients can be you know at significantly increased risk when they develop pulmonary hypertension and they probably need to at least be seen once in an adult congenital heart center and ideally they should not be lost to follow up long term because they can represent with this particular complication even with a completely successful repair. The other thing to mention is that even with a successful repair the surgical and medical literature suggests that there's somewhere between a 10 and 15% chance of residual shunts as well and those may get worse or you know over time and be associated with things like paradoxical embolism and other problems so again this is a group of patients that should ideally be at least followed up to some extent over time. (Time 1:01:59)
- Ison-Menger Syndrome
Summary:
Even if you have your ASD repaired there's roughly about a 2 to 3% chance that you can still develop pulmonary hypertension. The other thing to mention is that even with a successful repair the surgical and medical literature suggests that there's somewhere between a 10 and 15% chance of residual shunts as well. Those may get worse or be associated with things like paradoxical embolism and other problems.
Transcript:
Speaker 1
Yeah it's just to mention the pulmonary vascular remodeling because you know I think people think that if you close the atrial septal defect you're kind of out of the woods you never have to worry about it again and it's important to recognize that even if you have your ASD repaired there's roughly about a 2 to 3% chance that you can still develop pulmonary hypertension and from from what we know about pulmonary hypertension if you have pH with a closed defect it's actually a worse prognosis than PAH with an open defect so it's important to know that these patients can be you know at significantly increased risk when they develop pulmonary hypertension and they probably need to at least be seen once in an adult congenital heart center and ideally they should not be lost to follow up long term because they can represent with this particular complication even with a completely successful repair. The other thing to mention is that even with a successful repair the surgical and medical literature suggests that there's somewhere between a 10 and 15% chance of residual shunts as well and those may get worse or you know over time and be associated with things like paradoxical embolism and other problems so again this is a group of patients that should ideally be at least followed up to some extent over time.
Speaker 3
And Dr. Krsniewski is that ever too late to close an atrial septal defect?
Speaker 1
Well I would argue that if you develop Ison-Menger syndrome true Ison-Menger syndrome it's probably too late you know there there are case reports of patients getting treated with advanced medical therapies so you know what we have now are therapies that target three pathways. There's the endothelin pathway the nitric oxide pathway and then the prostaglandin pathway and so we have all these new medications 13 different drugs that have been FDA approved (Time 1:01:59)
- Atrial Fibrillation Risk
Summary:
If you were repaired before the age of 40 there was a very low risk of developing a fib going forward. The bigger the defect is at the time of presentation the larger the shunt and stuff probably the greater the risk of developing complications long-term. It's always arrhythmias heart failure pulmonary hypertension and it's that if you remember the big three arrhythmiasHeartFailurePulmonaryHypertension. yeah i think that was a good laundry list of things that you're going to look at againThe earlier the repair is the better they're going to do long-term so that means you know you mentioned 25 years of age, for example.
Transcript:
Speaker 1
Yeah i think that was a good laundry list of things that you're going to look at again the things to think about you know who's a high risk for complications long-term the earlier the repair is the better they're going to do long-term so that means you know you mentioned 25 years of age probably the biggest paper that compared atrial fibrillation risk was the gazula's paper that looked at 40 as the cut-off so if you were repaired before the age of 40 there was a very low risk of developing a fib going forward whereas if you're repaired after the age of 40 it was considerably higher risk of developing a fib so what the magical age is nobody really knows but what i usually say if it's in the childhood years you're less likely to have complications if you're in the adult years more likely to have complications the size of the defect so the bigger the defect is at the time of you know presentation the larger the shunt and stuff probably the greater the risk of developing complications long-term you know the presence of concomitant disease that's prepared at that time makes you think more of complications and then basically you know the age of the patient when you see them if you see somebody who's 50 years old and you know they had surgical repair 30 years ago they're more likely to have complications than if somebody comes back to you at the age of you know 25 and they had a repair at age you know 15 or something so it's really the the amount of years you know the the tikture of time does not help you in this case you tend to have more complications and again if you remember the big three it's always arrhythmias heart failure pulmonary hypertension and it's that (Time 1:08:44)