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Cardiac Rapid Fire: 60 Cases in 60 minutes (2023)
R7-CCA01-2023
R7-CCA01-2023
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We're going to go through rapid-fire cases. We're going to start with cardiac masses. So with that, our first case, so we'll just talk through the case we see here. Clearly, there is a large mass that's filling the right atrium and projecting into the right ventricle. It looks fairly aggressive. We look elsewhere. We can see that we see a lot of collateral vessels. Again, we see the mass that's crossing across the valve plane into the ventricle. And when we look a little bit more superiorly, we also see that this goes into the SVC and there is a large mediastinal component. So this particular mass is actually a large malignant thymoma with direct extension into the ventricles. And the reason why I show this case to start off is that we remember that when we consider cardiac masses, we're going to see all types of masses, but we know that most cardiac masses are going to be metastatic. So if you had to place a bet, the first thing you would think of, is this a met? Have we done the appropriate steps to determine whether or not there is metastatic disease? And then based on that, we can start refining what primary diagnosis can we think of. This is just another example here. We have another mass that's in the right atrium. Looking downward in the abdomen, we can see that there is enlargement of the IVC. And certainly when we look over in the left kidney, this is a large renal mass. We have renal cell metastases into the heart. So tumor extension is very common when we see these particular lesions. Keeping going. So here we have two different patients, patient A and patient B. And what do we see here? So patient A, we see a large, fairly circumscribed lesion in the left atrium. Looks pretty close to the interatrial septum. B, another mass here, also seems closely affixed to the interatrial septum, but just has less defined morphology, kind of looks more villous. So in this particular case, patient A, patient B, these are actually both examples of cardiac myxomas. And again, just highlights the different morphologies, although we classically think of them as circumscribed, we always look for the attachment to the interatrial septum. More commonly, they occur in the left atrium, but they can occur in the right. 70 to 80% occur in the left atrium, but they can occur elsewhere. The most important thing to remember is that cardiac myxomas are the most common benign cardiac tumor. So again, if you see the appropriate features that closely associate with cardiac myxoma, again, it's a very common thing to think about. Moving on, our next case. So here we have an echo and an MRI. So patient A, when we look at the echo, we see near the apex, there is this echogenic material that we see near the left ventricular apex. Similarly, when we look at the, interesting, when we look on the MRI, we see that there is a similar lesion that looks fairly bright with this kind of India ink artifact around the lesion. And then when we also look on this T1-weighted image, we see the same abnormality, this time very increased in signal relative to myocardium, similar to fat. And yes, in fact, this is an example of a cardiac lipoma. So again, very common in many respects, like pumas are the second most common benign cardiac mass. Fortunately, the features are very characteristic when we talk about CT or MRI. You know, again, fatty attenuation on CT, increased on T1, saturations with fat saturation techniques. Again, very characteristic of lipomas. Now here is slightly a different case. We have a scout image from a CT and the actual study. When we look on the scout image, we see that there is some enhancement, or I should say enlargement of the cardiac silhouette, particularly on the left side. Certainly corresponds to the abnormality that we're seeing on CT. So again, fairly circumscribed abnormality just outside the left ventricular wall. Now, if we took a little bit of sampling of the attenuation across this, we can see that it's relatively low. This is actually a pericardial cyst, which mimics a cardiac mass. The most important thing that we remember for pericardial cysts, they're obviously predominantly asymptomatic. Less than a quarter of patients will actually present with symptoms. And typically when they present, it's because there is some form of compression or even erosion of adjacent structures. So in this case, a mimic of a cardiac mass. Now, in a similar vein, here we have another case. Again, we have marked distortion of the cardiac silhouette, this time mostly on the right side. When we look on the coronal NPR of the CT, again, we see the same abnormality. And what's interesting in this case, when we look at the cellular component of this lesion, this is actually very complex. This was actually a localized fibrous tumor of the pleura. So this is actually a very atypical presentation. Typically we think of these fibrous tumors of the pleura being plural in origin and kind of loculated in its appearance. This case, a cardiac localized fibrous tumor is very rare. Typically it's considered a pericardial type of abnormality. But as we see here, it's within the pericardial space and causing mass effect on the adjacent structures. So next case, patient A, patient B. Well, what is it that we see? So clearly on patient A, we see kind of an intramural lesion that's involving the lateral wall of the left ventricle. We see a focus of calcification in the middle. So that's one, patient B, similar kind of intramural lesion, this time more involving the interventricular septum. We don't really see the same amount of calcification, it's a little bit smaller. So in this particular case, patient A, patient B, we have two different lesions. Patient A is an example of a cardiac fibroma and patient B was an example of a rhabdomyoma. So just quickly, both of these are intramural lesions, just hallmarks to kind of help you differentiate between the two. Rhab-fibromas are typically solitary, they can grow to very large sizes and calcifications are actually quite common in the presence of rhabdomyoma. And in the presence of fibroma. Whereas rhabdomyomas are often multiple, you can have multiple rhabdomyomas throughout the myocardium, many of which will regress early in age. Typically you don't see any calcification with a rhabdomyoma. Fibromas, you'll see some mild enhancement, whereas rhabdomyomas have very minimal to low enhancement. And with that, with fibromas, you will see some evidence of late gallium enhancement on MR, whereas rhabdomyomas, typically you don't see any late enhancement. The other important hallmark for both of these lesions is that they both have associations with tuberous sclerosis. So if you happen to see an abnormality that you are considering a fibroma or a rhabdomyoma, you also want to screen for other, the possibility for tuberous sclerosis as well. All right, trying to keep on time. So here we have another lesion in this patient. We see this kind of encroaching lesion kind of around the right atrium. We see it here on CT, subsequently on MR. On MR, the lesion is relatively lower in signal relative to the myocardium. So again, could this be something that's metastatic? Not quite sure. We look for other features to kind of help us. When we look at enhancement, we see that there is some significant enhancement involving the lesion. And in particular, we see some abnormalities involving the kidneys as well. So now I'm sure all of you recognize the path in this particular case, these kind of foamy cells. This is actually an example of Erdem-Chester disease with involvement of the heart as well. Erdem-Chester, again, it's a systemic xanthomatous infiltration that's characterized by these non-Langerhans cell foamy histiocyte infiltration. The cardiac involvement as well as the real involvement is something that is characteristic of this particular disorder. So again, trying to find other features that can help you hone in on the underlying diagnosis. Our next case, again, we have a chest radiograph and a CT. On the chest radiograph, we see marked enlargement of the cardiac silhouette and numerous opacities involving both lungs. So with further determination on the CT, we see, again, a large, ugly-looking mass, a lot of central necrosis. And additionally, we see that there is an extensive involvement of the lungs. We see some kind of soft tissue density there, some pleural thickening, some nodular thickening. Certainly when we look at the underlying lung parenchyma, we see multiple nodules scattered throughout all five lobes. This case is an example of an angiosarcoma with pulmonary metastases. And it's actually not uncommon to see pulmonary mets with angiosarcomas. So angiosarcomas are the largest group of differentiated cardiac sarcomas that we see. So when we consider primary malignancies as opposed to benign cardiac malignancies, or benign cardiac disorders, angiosarcomas make up the largest differentiated group of primary sarcomas of the heart. And up to 80% of them may have pulmonary metastases at the time of diagnosis. Okay, our next case. Again, patient A, patient B. Patient A, we see this kind of very small, nodular type of soft tissue mass that's along the aortic valve leaflets. In patient B, we see a similar looking type of mass, this time in the left atrium. Basically, again, looks like it's kind of attached to an endothelial surface. In this case, patient A and patient B, these are both examples of a papillary fibroblastoma. And again, classically, we associate these lesions with being on the valve, but they really can occur on any endothelial surface, as we see here. The aortic valve and mitral valve are the most common locations. But again, 10% can occur on any endothelial surface. We can see them in the ventricles, we can see them along the papillary muscles, the other leaflets, and this, here we're seeing within the left atrium. Again, all of these are common presentations, or I should say varied presentations of papillary fibroblastomas. All right, here we go again. Patient A, patient B, both of which have significant masses. So the first thing you think about, yeah, metastases. But let's look at this a little bit closer. So patient A, so we have this MR, we see this large mass, pretty aggressive looking, it's eating into the right atrium. The mass itself has multiple kind of heterogeneous signal, areas of increased signal. Whereas patient B, this is a CT, we see again, very large, fairly aggressive looking lesion, eating into the right atrium, right ventricle. We see that there's a pericardial effusion associated with this. So in this particular A, patient A and patient B, patient A is an example of an angiosarcoma. Patient B is a primary cardiac lymphoma. And as you can see here, again, both have very similar type of presentations. But the context in which they present is also very important to consider. You know, patient A, or I should say angiosarcomas, again, they're very common, the most common differentiated cardiac sarcoma. They're gonna be highly aggressive, so you can see aggressive activity. You're gonna see robust contrast and enhancement, as well as late gamelan enhancement. Typically the areas of the heterogeneous signal indicate areas of necrosis as well as hemorrhage within the mass itself. Whereas primary cardiac lymphomas, most lymphomas that you see with cardiac involvement will actually be secondary. There'll be a mediastinal lymphoma with involvement of the heart. But a true cardiac lymphoma, it's gonna be isolated. And typically you're gonna see this in someone who's immunocompromised. Patients with HIV, this has also been classically described. These patients will commonly have pericardial effusions. About 2 3rds of patients that pericardial effusion, the cytology of which can actually give you the diagnosis. And as opposed to enhancement, as opposed to angiosarcomas, which tend to have robust enhancement, lymphomas can have very mild and heterogeneous enhancement as well. But again, both of them tend to be infiltrative lesions that have a predilection for right-sided cardiac chambers. Okay, moving on to our next patient. This was an interesting patient. Patient came in through our ER. As we see here, we see that there is some ventricular hypertrophy. There's a small pericardial effusion. Do you see any abnormalities? I guess it depends on how it projects. Now it just so happens that this person also had an abdomen as well as the CT. And when you look at the delays, you see multiple masses involving the left ventricle. So this came to quite the surprise of the radiologist who was reading the study. So subsequently this patient went on to get an MRI. And again, we see multiple lesions involving the left ventricle. We can see here on the scout, we see multiple areas of abnormality, T2-weighted images. We see extensive amount of edema involving the myocardium. So this was actually metastatic hepatocellular carcinoma. And I did not include the liver on this, but you could see direct extension from the liver in to involve the heart as well. Most commonly when we think of hepatocellular carcinoma, we think about the lung, abdominal lymph nodes, and certainly in the bone, but we can see direct tumor invasion of the IVC. And with that, see continuous extension into the right heart. This particular case, we see direct extension involving the ventricle as well. All right. All right, I gotta speed this up. So here we have another patient. We see on CT this kind of well-circumscribed lesion, low attenuation with some alternating areas of abnormality. On MR, we see T1 post-GAD. We see that there is some enhancement of the outside of the lesion. We see some different areas of signal inside, but overall, bright on T2-weighted signals. This is an example of a cardiac teratoma. Obviously, teratomas, embryonic origin, composed of the multiple germ cell layers. About 90% of these are found in the pericardium, and with that, they're grouped within the primary benign cardiac tumors. Okay. All right, hopefully I don't run out of time. Here we see a large lesion along the right heart. Kind of looks like it's crossing across the tricuspid valve, and in fact, we look a little bit lower. We see that there is this component here. This is contiguous with the lesion. This is the top. This is the bulk of it. This actually here, when we look at other images, we see that this was actually a giant aneurysm of the right coronary artery. So again, another mimic presenting as a cardiac mass. So that was an aneurysm. Another case here, patient, we see a large area of decreased attenuation, pretty much on the roof of the left atrium. When we look at other views, we can see that there is this eccentric layering density. This patient had presented with multiple CVAs, right upper extremity weakness. This was actually just a large left atrial thrombus, which we can, again, clearly see here involving the left atrium. Majority of these patients are gonna have AFib or some other valvular disease adherent to the wall, and the decision in these situations is anticoagulation versus underlying surgery to get the lesion out. All right, coming down to the last couple here, we see this patient with salt and pepper lesion kind of interspersed between the aorta and pulmonary artery. We see it here as well. So this kind of heterogeneous enhancement, this is an example of a cardiac paraganglioma. Paragangliomas, 20% of patients who have cardiac paragangliomas will have paragangliomas in other places, and very similar to paragangliomas, systemic hypertension is another hallmark that they can present with. All right, rounding out the last couple cases, this one, a pretty ugly looking lesion in the left atrium. These are two different patients, patient A and patient B. We see the lesion kind of extending into the pulmonary vein in patient A. Patient B, we see, again, a large ugly looking lesion, multiple areas of calcification. So particularly the calcification, the left atrial origin of this, one thing to consider is a left atrial osteosarcoma. Now this is a rare lesion, but typically can occur within the left atrium. The left atrium has many pluripotent cells that can differentiate this, which contrasts with metastatic osteosarcomas, which typically favor the right atrium. So if you see a left atrial, what appears like an osteosarcoma, it's possibly that this is a primary osteosarcoma of the left atrium. And finally, my last case here, again, a large lesion involving the right heart. First off, if we're gonna make a wager, oh, gotta worry about a MET. Okay, but is there anything else that can help me further differentiate it? Well, I don't know, not really. It's a large mass. It's large necrotic. We see it as a pericardial effusion, a pleural effusion. So this one was quite a quandary to us. The path came back. This was actually a pericardial mesothelioma. And this was actually quite rare, extremely rare. Like all mesotheliomas, the subtypes, epithelial, spindle-cell mix, these have a very poor prognosis. And with that, that was really a rapid fire of multiple cardiac masses. Again, the idea is that you consider most are gonna be metastatic. Think about the primary benign lesions, and if it looks aggressive, then you know you're dealing with some type of sarcoma, and you're looking for other elements that can kind of help hone you in on the final diagnosis. And with that, I thank you all for your attention. Thank you very much for the introduction. In this 20 minutes, and in a case-based approach, we're going to review the role of advanced imaging in the evaluation of valvular heart disease, and due to the matter of time, only focus in aortic valve disease, and the main findings that can help us to make the right diagnosis in those cases. We know that valvular heart disease is a significant clinical problem, which has associated high morbidity and mortality, and metronal and aortic valve diseases are the most common sources of disease. Generally, worldwide, the main cause are its rheumatic disease, whereas in developed countries, the most frequent forms are secondary to degenerative for congenital heart disease. And also, we know that echocardiography is going to be the first-line imaging modality that help us to detect the valvular heart disease, to make the diagnosis, for severity grading, and also for reconstruction and treatment planning. CT has a complementary role when echocardiography is not conclusive, helping us to rule out coronary artery disease, diagnosing and grading native and prosthetic valvular heart disease, but also has a tremendous value for treatment planning and for ruling out possible complications. We also know that cardiac MR is the ultimate technique in valvular heart disease, help us to, it's a gold standard technique for assessing ventricular volumes and function, has excellent properties for tissue characterization, and with phase contrast imaging, we can deal with hemodynamic parameters, but we can also dive more into the world of hemodynamics and apply 4D flow imaging, not only for evaluating common hemodynamic parameters, like peak velocity, regurgitant volumes, or regurgitant fraction, but also for focusing on more advanced parameters, like wall shear stress, like particle traction or kinetic energy. Also, due to the conservation of mass principles, we could also evaluate these parameters without then avoiding the vertical flow, which can be a potential source of underestimation. So let's start with two cases, the two-year-old male and 64-year-old male. The first one has a heavily calcified aortic valve with irregular aortic valve opening area, and the second one, we could see there's a concentric remodeling of the left ventricle with a regional wall thickness of 0.6. There's a signal void in the aortic valve, and we could see an irregular valve opening area with increased peak velocity on the aortic valve of three meters per second, and these are a moderate and severe aortic stenosis. And aortic stenosis is defined as the obstruction of the forward flow through the left ventricle flow tract, the most frequent cause in worldwide rheumatic disease, and again, in developing countries, degenerative disease. We can classify aortic stenosis either in supravalvular, valvular, or subvalvular, and CT, when echosynconclusive, has good sensitivity and specificity for detecting and grading aortic stenosis. In those cases, we are going to see, in several cases, less than one square centimeter valve aortic area, and we are going to see a thickened, irregular, and calcified gasps. These are other two cases, and with this, we could see that the higher amount of aortic valve calcium, the higher severity of aortic stenosis, and in very several cases, we could see also partial fusion of the aortic gasps, giving up to functional pectus valve stenosis. So, calcification, it's a big issue in aortic stenosis, and it's different between genders. In fact, lower thresholds has been described in women among men, and this is due to, because women have lower calcium load for the same aortic stenosis severity compared to men. Also, the pattern of aortic valve calcification, it's important that we could see that in degenerative disease, we are going to see a heavily calcified aortic valve progressing from the annulus towards the gasp that give us an irregular valve opening area, whereas in rheumatic disease, there's a symmetric calcification and fusion of the commissures, giving up to a symmetric valve opening area. Another thing that we have to take into account is that we have to detect extension of this calcification towards other structures, like in the mitral, or the mitral curtain, and this has impact in the preoperative treatment planning. We also know that aortic valve calcification is strongly associated with severity, and may detect severe aortic stenosis independent of flow, and this is particularly important in specific cases of low-flow, low-grain aortic stenosis, where we could find a cubical echocardiographic findings, and aortic valve calcification quantification could help us not only to detect this aortic stenosis severity, but also is a predictor of aortic valve replacement and death. This is another case of a 78-year-old male with ischemic heart disease and pericardial effusion. Again, we could see there's a concentric remodeling and a double aortic lesion with aortic regurgitation, but also, I want to show you that there are also an irregular valve opening area with severely reduced size. There's a signal void of a testamentic jet in the aortic valve, and this is important. This is a finding of aortic stenosis, and this is important because help us to place adequately the phase contrast through plane imaging perpendicular to this jet. So findings that we could observe on MRI, it's a severely reduced valve opening area, in severe cases, less than one square centimeter, and also increased peak systolic velocity, more than four meters per second in severe disease. In plane evaluation, either with 2D phase contrast or with 4D flow imaging help us to detect what is the site of aortic involvement, either supravalvular, valvular, or subvalvular. And indeed, not only we can detect the area of peak velocity, but also retrospectively in 4D flow we could go there to calculate the peak velocity and the peak systolic gradient. And indeed, 4D flow imaging is more accurate than 2D phase contrast due to the capabilities for 3D orientation and retrospective analysis. Also has an excellent agreement for net flow compared to 2D phase contrast, and in terms of peak velocities, better than 2D phase contrast because avoids underestimation attending to the appropriate quantification in eccentric jets. Also has been shown that has excellent correlation with Doppler echocardiography. Moreover, can help us to know and to approach to abnormal patterns of flow in descending aorta and see abnormal flow patterns in it and quantify elicitive or DCT or wall shear stress, and therefore to detect aortic stenosis-related aortopathy. Left ventricular flow obstruction is a common cause of subvalvular aortic stenosis. It's more common, it's common in up to, it's seen in up to 70% of classical hypertrophic cardiomyopathy, and has increased risk for related heart failure and related death. There are several, sorry, there are several factors that are involved in left ventricular flow obstruction, not only elongated anterior mitral valve, but also abnormal papillary muscles or asymmetric thickening of the septum. And these give us a paradoxical movement of anterior leaflet and or corded tendon towards the interventricular septum, and therefore, reduced co-optation of the mitral valve and secondary mitral regurgitation. Another case, 64-year-old female with ascending aortic aneurysm we could see. There's an increased size of the aortic root, but also of the ascending aorta. And on the aortic valve, there is a central hollow diastolic rotograph flow towards the left ventricle, and produces a fluttering of the anterior leaflet of the mitral valve. If we focus on the aortic valve, we could see that there's incomplete co-optation of this aortic valve and hollow diastolic rotograph flow. The regurgitant fraction is 46%, and this is in keeping with type 1 aortic regurgitation. An aortic regurgitation is defined as incomplete co-optation of the pulmonary gas with back flow towards the left ventricle. Mild aortic regurgitation is common, and it's been seen in, it's been described in up to 67% of people of more than 65-year-old, but contrarily significant, clinically significant aortic regurgitation, it's only seen in up to 15% of aortic regurgitation population. Again, the most frequent cause worldwide is rheumatic disease, but in developed world, the most frequent cause is bacuspid aortic valve. We could differentiate the acute aortic regurgitation that's commonly seen on infective endocarditis, but also can be seen in trauma and aortic dissection, or chronic aortic regurgitation that is most frequently seen in vulvar degeneration, rheumatic disease, or aortic ectasia. Again, there's a functional classification in similar fashion as in carpenter classification for middle valve disease that could be differentiated due to aortic root enlargement, cause prolapse, or poor cost tissue quantity or quality. Location of the 2D phase contrast in aortic regurgitation is important, and we have to place it in the tubular junction in order to assess directly the presence of regurgitant volume and regurgitant fraction. In fact, evaluation with 2D phase contrast has better reproducibility than echo, but lower cutoff values have been described compared to the echocardiography due to several geometric assumptions that echocardiography has. However, better correlation with 3D echocardiography has been described. Moreover, cutoff value of 33%, it's considered as a marker of severity because in those patients, there's a higher progression to surgery. Another factor that we could evaluate is the presence of holo-diastolic retrograde flow in the descending aorta. And it's a very reproducible and robust parameter that we could evaluate and has been related to a higher risk of death and hospitalization. For tube flow imaging, help us to increase our confidence in aortic flow, in aortic regurgitation quantification. And for that, we could apply retrospective valve tracking. And it's especially useful in when we saw multiplanar and eccentric jets or vertical flow imaging, avoiding this vertical flow and avoiding this underestimation of the aortic regurgitation. Another two cases, 61-year-old male, 3-athlete. We could see a two bicuspid aortic valve open area with secondary two fusion of the right coronary and non-coronary cusps with mild calcification. And on the right side, you could see a 39-year-old male with known bicuspid aortic valve and ascending aortic ectasia. In this patient, we could see a double aortic lesion with severe aortic stenosis. We could see there's an increased peak velocity, more than four meters per second, but also a severe regurgitation with a regurgitant fraction measured on 40 flow of 47%. If you could see, there's a large ascending aortic ectasia and there's increased wall shear stress in the right and lateral portion of descending aorta. And we know that bicuspid aortic valve is a most frequent congenital valvular heart disease. It's been described in up to 2% of population. And it's secondary to commissural fusion, given unequally sized cusps. Could be associated with aortic stenosis, aortic regurgitation, mixed lesion with aortic stenosis and aortic regurgitation or non-significant valvular heart disease. Again, the pattern of calcification is different from rheumatic or degenerative disease. And bicuspid aortic valves, we're going to see less severe calcification, usually in the commissural edges or outlining the rafae. There are several things that we have to mention when we deal with a bicuspid aortic valve, not only the pattern of fusion of the commissures, but also the size of the valve opening area, if it's symmetric or asymmetric, and the size of the aortic root and ascending aorta. And this is important because bicuspid aortic valves have higher risk of aneurism and dissection. And the flow eccentricity in the ascending aorta and the site of the aortic dilatation has been related to bicuspid aortic valve phenotype. Another important thing to take into account when we are dealing with valvular heart disease is not to have a valvular-centric approach. We have to look at the left ventricle in order to see adverse remodeling. When we are, when we have an increased afterload initially, the left ventricle is going to have a compensatory remodeling and developing concentric remodeling or concentric hypertrophy. The pattern of hypertrophy could be asymmetric and has been described several patterns of hypertrophy secondary to aortic valve disease that has inherent worst prognosis. When the insult persists over time, then a maladaptive response overcomes and there's secondary dilatation of the left ventricle with progressive reduction of the left ventricle ejection fraction and heart failure. When we focus on, when we deal with aortic regurgitation, there's an increased preload and then the ventricle progressively dilates and progressively turns into a dysfunctional ventricle. But not only we could observe the world changes, we can further drive into the world of hemodynamics and try to differentiate patterns of adverse remodeling attending to these intercavitary hemodynamics. And it's been described in literature focused on dilated cardiomyopathy that in patients with adverse remodeling, we are going to see an increased vortical size on the left ventricle and displaced over the apex. Moreover, we can separate the four components inside the heart and differentiate left ventricle adverse remodeling by detecting lower residual volume and, sorry, increased residual volume and decreased direct flow as a marker of inefficient ventricular pump function. Another case is this is a 55-year-old male with fever and left flank pain and positive blood cultures. You could see there's hypotenuse region in the lower part of the left kidney and also sphenic infarct. And when we focus on the aortic valve, we could see soft tissue lesion located in the low pressure side of the aortic valve. And with mild thickening of the adjacent leaflet. This is a patient that has infective endocarditis. And infective endocarditis is a severe disease associated with high morbidity and mortality that results from infection of the endocardium, prosthetic valves, or intercardiac devices. Vegetation are commonly seen as mobile soft tissue lesions with irregular shape and size that occasionally can produce functional impairment. They can be located not only in the valves but also in the chordae tendinea or in the walls or intercardiac devices. And usually when they are located in the valves, are located in the low pressure sides. That is the ventricular flow track of the aortic valve or the middle side of the middle valve. When they are large, they have risk of embolization. And it's an indication of urgent surgery. Other feature of aortic valve of infective endocarditis in valvular involvement are leaflet perforation or leaflet aneurysms. Cross-sectional imaging techniques can be helpful for detecting pre-valvular extension or extracardiac embolic events. This is another case of 47-year-old female with solitary pedicular lesion in the aortic valve incidentally detected on echocardiography. As we could see, there's an echodense lesion located, pedicular lesion, highly mobile located in the downstream portion of the aortic valve. It's this lesion is, has a small pedicle and it's attached away from the free edge of the aortic valve in the downstream. And it's compatible with a papillary fibroblastoma. As nicely shown, Professor Judy, papillary fibroblastomas are the second most common benign tumors. And it's the primary cardiac valvular neoplans of the heart. Commonly, the most frequent site is the aortic valve but also can be seen in the mitral to conspital pulmonary valves in the walls or in other portions that have endothelium. They are located usually downstream, away from the free edge. And they usually are small, very highly mobile with a small pedicle that attach these lesions to the involved valve. And in certain portions, it's been associated with embolic events. Other less frequent locations of a papillary fibroblastomas are the Eustachian valve or the pulmonary aortic valve where we could see habit enhancement of these lesions. As I said, some of these lesions can have associated embolic events. And it's thought to be secondary to abnormal flow patterns adjacent to the papillary neoplans. And now with 40 flow, we could see these abnormal patterns. We could see there's a vertical flow adjacent to the papillary neoplans. And therefore, there's theoretically with increased risk of embolism. And this complex intracardiac features could also be seen in other primary neoplans like in this actual myxoma which produces dynamic mitral stenosis. And this mitral stenosis, not only we could see it on conventional imaging, but also with 40 flow, we can quantify, we can select and detect the peak velocity and show the abnormal EA ratio in the inflow area. So hopefully in these 20 minutes, I've been able to show you the role of advanced imaging in the evaluation of valvular heart disease, the impact and the main features that we could observe not only in aortic stenosis, but also in aortic regurgitation. The importance of quantification of aortic calcification in especially in low flow, low gradient stenosis. And not only to have a valvocentric approach, but also to look at the left ventricle in order to assess concentric and adverse remodeling. Finally, the value that we could, the promising value that we could obtain with 40 flow imaging in order to not only precisely detect the aortic valve disease, but also to evaluate adverse remodeling. Thank you very much. I'm Lisa Bergman. I'm an assistant professor of radiology and also medicine at the University of Kentucky, which is in Lexington. And I also have the honor to be the medical director of our multidisciplinary 3D imaging lab in our Department of Radiology. So we're going to review some congenital cardiovascular cases. I'm going to, I've selected these cases in part to emphasize our persistent role as generalists, no matter how specialized we become, because we are indeed responsible for everything we see on the edges and outside the designated anatomy of interest. And also to emphasize multimodality evaluation, the importance of comprehending fluid dynamics and hemodynamics in all congenital cardiac cases, as well as genetics. And then also to think of the vasculature, in particular the arterial walls, as an organism themselves. And historically we've very much focused on the lumen and the lumen size, but the walls themselves are tissues. So just a little quote I always talk about when I talk about cardiac anatomy, since we don't respect our coronal, sagittal, and axial plane so much in the heart. So this first patient is a 42-year-old male, post-congenital heart disease repair. Images, these are the axial CT of the lungs. In the lung window, there has been a known lung cancer that's been resected. And there is a recurrent, presumably neoplastic, or pleural fusion in this axial soft tissue window. This is arterial phase. We can begin to see just why the cardiac silhouette is so large on the lung window. So we can see especially enlargement of the right side of the heart, as well as some post-operative repair in the pulmonary valve region. And then there's some heterogeneity in the density of the liver, as well. So our delayed images are most revealing. We see that pulmonary valve baffle. We see numerous hepatic metastases, as well as a right adrenal met. So this is indeed metastatic stage four lung cancer in a patient who had tetralogy of fallot repaired in childhood. So tetralogy of fallot, the most important, you know, of course, there's four parts of it. But I think if you remember any one thing that can kind of help you derive the other aspects of tetralogy of fallot is that the RV outflow tract is obstructed. And so tetralogy is one of the five T's of the major cyanotic congenital heart diseases. It does have typically decreased pulmonary vasculature. RV hypertrophy is one of the four features, but this does not develop until after birth. So presumably in response to the obstruction of the right ventricular outflow tract. There's also VSD and overriding aorta. And so kind of think of it like the blood can't get out of the right ventricle via its normal pathway. So there has to be that VSD and or that overriding aorta so the blood can exit the right ventricle somehow. There can be a pentalogy with an atrial septal defect as well. Twenty-five percent of TEP patients have a right-sided aortic arch, which is not the case in this patient. And they can also have coronary abnormalities. So tetralogy is considered the most common cyanotic congenital heart disease in those infants who survive beyond the neonatal period. About 10% of all congenital heart defects. So estimated about just up to .1% of all live births. So three to, and some studies also say three to five per 10,000. So historically we thought there was more tetralogy in males, but now there's some thinking that it's equal between male and females. In the USA, per the most recent data I found in 2010, there were about 1,660 patients born with tetralogy. And that was one out of just over 2,500 live births. So now we repair the right ventricular outflow tract obstruction primarily. And pulmonary regurgitation develops post-repair in nearly 100% of patients. So it's highly important to follow these patients, typically with cardiac MR for most accurate flow quantification. And once that regurgitation is either symptomatic or sometimes pre-symptomatic, but it's increasing at a rate that's concerning, then this data is used to time the repair. Also the coronary artery position in these patients is of utmost importance as noted on a prior slide. It can be somewhat aberrant. And sometimes the pulmonary valve repair is done endovascularly. That's a new developing technique used more and more in the US. And if the LAD in particular is right there at the site where the pulmonary valve will be placed endovascularly, we definitely don't want to injure or cause a dissection or somehow otherwise occlude or adhere our replacement prosthetic pulmonary valve to that LAD. Also note based on this case in which we saw stage four metastatic lung cancer in a 41 year old, which is quite young, congenital heart defects, they are the most common birth defects. And then we expect now nearly 100% of these patients to live to be adults. But this interesting and recent study out of Sweden found that patients with congenital heart defects have a 1.23 times higher risk of obtaining cancer. And that confidence interval, 95% confidence interval does not cross one. So it might be even closer to 1.3 times higher. So this is an area of developing research. Our next case is a 27 year old female with a known syndrome and with hypertension. So this is a chronal cardiac MR focusing on the left ventricular outflow tract. This is seen as a SFP or bright blood image. And I want to draw your attention to this. Let's see if you can see my mouse. Okay, great. So here we have our sinotubular junction and we have a narrow jet coming through there. So we have some narrowing at the sinotubular junction resulting in turbulence and stenosis, turbulence distal to that stenosis. And so this is considered supravalvular aortic stenosis. And I'll draw your attention inferior to the diaphragm. Again, we're responsible for everything we see. And here we see a pulsatile vessel, presumably an artery given the high pulsatility in the expected path of the abdominal aorta. It's very small. So here we have our axial MR and this is a predominantly T2 rated image. And we see, this is even above the diaphragm, we see a very small caliber descending throughout the aorta. And here it was confirmed on CT. And so the caliber was measured about maximum eight millimeters just below the diaphragm. So this patient was known to have Williams or Williams-Burin syndrome. There is a micro deletion and there are various types of micro deletions that can cause Williams syndrome in the elastin gene, which is in chromosome seven, Q11.23. And so this results in a loss of function. And so when there's a heterozygous, there's approximately 50% less elastin production throughout all cells in the body. So these patients are known to have a facies called elfin. They have shorter stature. They have sometimes mild cognitive impairment. And they're also known to have pulmonary arterial stenosis, supravalvular aortic stenosis, rarely mid-aortic coarctation, which is also called middle aortic syndrome, and renal artery stenosis as well. And so just here's two references regarding the mid-aortic syndrome or the mid-aortic coarct. So now we're gonna move on to another adolescent, technically pediatric female, 16-year-old. She's pregnant, and she presented at 32 weeks to an outside hospital due to premature labor as well as hypertension with systolic pressures up to 171 millimeters mercury. So she is transferred to our academic center. And due to some echocardiogram findings as well as persistent hypertension, she was induced into labor at 34 weeks gestational age. So she had a successful delivery. And then she underwent a cardiac MR with a gadolinium-based contrast agent shortly afterwards. And just a reminder, as far as we know still, to the best of our knowledge, gadolinium is a class III teratogen. So of course, in every case, you need to do a risk-benefit analysis when the patient is pregnant. But if it can be avoided either by delaying imaging until after pregnancy or with the off-label use of faramoxetol, which is an FDA-approved, basically IV iron treatment for iron deficiency anemia, which is FDA-approved in pregnancy. And there's more and more safety data on use of faramoxetol as an MR contrast even in pregnant patients. But so if we can, we would like to avoid the gadolinium-based contrast agent during pregnancy. So unfortunately, there are quite a few artifacts. But this is the candy cane oblique sagittal view of the thoracic irida in our MRA. And you can see there's absolutely almost no gadolinium viewed here in the proximal descending thoracic irida, which is distal to the left subclavian artery. Here we can see some mild LV hypertrophy as well as some dilation as well as of the LA. And here we have a video. And perhaps you can see the sort of fish mouth appearance of a bicuspid aortic valve as well. So a CT is also performed. This is our 3D labs reconstruction. And the CT very well demonstrates that near total coarctation in the descending thoracic irida as well as extensive collaterals along the ribs. Here's our 3D reformat. And then also, of course, the patient did not get the chest X-ray until after she had already been taken to the OR and had her coarct repaired and stented. But there is some rib notching. So just another instance of even our bread and butter chest X-ray, very quick to obtain, very low ionizing radiation exposure exam might have given a clue as to what was going on if it was the first exam done in the first place. So bicuspid aortic valve and coarctation. A bicuspid aortic valve, as Dr. Marcono mentioned, it's the most common congenital cardiovascular anomaly at one to 2%. It's more common in males than females and also in Turner syndrome. Question of whether a second X chromosome is somehow protective. And also, it can be associated with aortic dilation, as Dr. Marcono very well illustrated. And that's found in about 40% of patients with bicuspid aortic valve. But there is also some association with coarctation. And that's also more common in males than in Turner syndrome. And historically, before surgical intervention for coarct was commonplace, dissection in coarct with bicuspid aortic valve could have a mortality of up to 50%. Whereas in patients without a bicuspid aortic valve, it was only about 20%. Okay, so this is a next case of a 22-year-old female. So here we have a coronal CNA SSFP or bright blood imaging. And here we're seeing our IVC going directly into our right pulmonary artery. And we again see that here. And we also, in this image on your right, we see the SVC also contributing directly to the right pulmonary artery. And so here is a short access CNA SSFP. And you can see that there is a very, very large ventricular septal defect within the musculature. And this was just considered basically a single ventricle. It was functionally a single ventricle. So it was repaired as a Fontan. And here's a companion case in a 40-year-old male. So we have our post-contrast CT. We do have a left-sided aortic arch. And I'm going to go to the delayed images, which are a bit more illustrative. But you can see there the nodular hepatic contour. So hopefully this video file gives me a bit more control. So again, we can see here, we see a baffle. So we see the systemic venous return coming to the pulmonary arterial system. And so this is another post-Fontan patient. And we can see also here by the distal esophagus, there are rather large caliber curvilinear enhancing structures consistent with varices. And here we see a nodular contoured liver with a start, kind of starting to get shrunken and as well as an enlarged spleen. So single ventricle is considered again one of the five T's if you call it a tingle ventricle, just as a mnemonic for remembering. So it is a cyanotic heart disease. It's considered to be about just under 8% of congenital heart disease. The Fontan repair is a multi-step repair with the end result being baffles. So that the systemic venous return goes directly to the pulmonary arterial system bypassing the single ventricle. This way the single ventricle serves as a systemic arterial or the subaortic ventricle. Patients, it's imperative that patients be followed using MR or ultrasound elastography or even biopsy because hepatic fibrosis occurs in nearly 100% by adolescence. And currently there are no definite screening guidelines. However, screening may be considered starting as young as age 12 and some people consider even earlier. So here's a great cartoon showing the surgically corrected heart after diagnosis of a single ventricle. And the physiologic consequence of the hepatic fibrosis. And again, virtually 100%. And I'm a big fan of saying never say always and never say never. But this is basically as close to always as we get in medicine. And so this, any kind of screening guidelines are currently under development. And again, starting by age 12 is not a consensus or a definite guideline at this point. Another note, sort of in relation to our prior case with Clark. I do know of one patient who recently, she had a post-Fontan patient who ended up conceiving twins and carried them both nearly to term successfully without any cardiac complications to that patient. And so this is per personal communication from the radiology and Fontan team at Cincinnati Children's who we collaborate with at University of Kentucky. But just as our post, our congenital heart patients are aging and they're becoming adults. And this is all very new and we don't know how, you know, how their hearts will hold up. Oh boy. So this is a 4D flow image showing the path lines through the right ventricle and the repaired pulmonary artery or the neopulmonary artery in a patient with dextro-TGA post-arterial switch. So dextro-TGA is often simply called TGA. It is a cyanotic disease also. One of the T's, the five T's, just under 1,200 birth annually in the USA. And it's typically repaired via arterial switch. It could also be repaired via atrial switch. That is less commonly performed now but it was historically performed. Levotransposition of the great arteries is also called congenitally corrected transposition. And this is one example of where two wrongs do make a right. So this patient is a 27-year-old male. The first time I opened this patient's chest x-ray, I thought, oh, the text flipped it. But you can see it is labeled left on our right, so on the radiology left, so it's correctly labeled. And you can also see here the shadow of the aortic knob and the descending thoracic ureter, which are also on the right. And then also I'll draw your attention to all these mediastinal clips, which are consistent with a surgery this patient had prior to this film. So here we have just two axial slices showing the right side or the dextrocardia, the right-sided heart with the apex directed also to the right. And we have the right-sided ventricle on the patient's right is morphologically the left ventricle. And we have the patient's left-sided ventricle, the morphologic right ventricle. So when I'm working with trainees, I always talk with them about, you know, I say use whatever side and then whatever morphology. Because once you can start sort of dividing the sidedness from the morphology, even though we typically use the sidedness to describe the morphology, to me that's the most clear and is a bit wordy. I generally prefer great concision. But this patient also had a cytosine versus totalis. You can see the liver is on the patient's left. The spleen is on the patient's right. The stomach is on the patient's right. The descending thoracic ureter or descending thoracic slash upper abdominal ureter is on the patient's right. So cytosine versus totalis is only about 1 in 10,000. It does have a slight male predominance, about 50% more often in males. But in patients with primary ciliary dyskinesia such as this patient, about 1 in 2 do have cytosine versus totalis. And so an eponym sometimes used would be cartesianers and this patient had the surgery, had a bilateral lung transplant due to that primary ciliary dyskinesia and that's why they have all those mediastinal clips. Okay, so I think this is my last case. Here again just our, you know, our perhaps hospital handshake of a simple chest x-ray, still our bread and butter in radiology and you can see a device near the right border of the spine in superimposing the right aspect of the cardiac silhouette just to the right of the line. And so this is an atrial septal defect repair device. And we can see its position between the atria and the axial CT in the bone window so as to avoid the, to minimize the metal artifact. And then on the sagittal, you can also see it between the atria as well. So atrial septal defects typically close within the first year of life, estimated just under, just over 1 and a half of 1,000 livers. There are five types. The most common is the ostium secundum. Ostium primum ASD, you can kind of think of it as an atrioventricular septal defect. So it's very kind of low towards the ventricles or towards the base of the ventricles, if you will. Superior and inferior sinus venosus ASDs and coronary sinus ASDs where there's actually an opening between the coronary sinus and the left atrium. A patent-framed ovary is not technically considered an ASD. So that is why it's not listed as a sixth type. So just again, to go over our objectives, I wanted to review some congenital cardiac cases that reemphasize our role as generalists and reminding us to focus on anatomy that's not the focus of the exam, to keep your fluid dynamics in mind because that will help you remember the congenital cardiac diseases. Don't hesitate to use multiple modalities as needed. Don't forget about genetics, which we're still learning about as a growing body of research knowledge. And then the importance of the vasculature, for instance, the elastin gene affecting the aortic wall. The vessel wall is very important and we're just kind of starting to image it historically having done angiograms under fluoro and not really having been able to see the walls that well. Thank you so much for attention.
Video Summary
In the transcript of several rapid-fire medical cases presented at a conference, a diverse array of cardiac conditions and imaging techniques are explored. The discussion begins with cardiac masses and tumor identification using imaging methods, highlighting a large malignant thymoma and a right atrial mass due to renal cell metastasis. The focus then shifts to cardiac myxomas, emphasizing their commonality as benign cardiac tumors and their typical occurrence in the left atrium.<br /><br />Further discussion delves into various benign cardiac masses such as lipomas characterized by fatty attenuation, and pericardial cysts, which can mimic cardiac masses, often being asymptomatic. Other rare conditions like localized fibrous tumors of the pleura are noted for their atypical presentation in the cardiac area.<br /><br />The speaker addresses specific cardiac lesions with examples: cardiac fibromas and rhabdomyomas, differentiating these based on calcification patterns and associated conditions like tuberous sclerosis. Erdheim-Chester disease is identified by foamy histiocyte infiltration affecting the heart, and angiosarcoma's aggressive nature is underscored by its association with pulmonary metastases.<br /><br />Cardiac valve abnormalities are also examined, such as papillary fibroelastomas, primarily found on valves but capable of occurring on any endothelial surface. The session wraps up with insights into advanced cardiac imaging, particularly in valvular heart disease, emphasizing echocardiography, CT, and the evolving role of MRI, including 4D flow imaging, to assess and manage conditions like aortic stenosis and regurgitation, reflecting the significant developments in cross-sectional imaging for cardiac diagnosis and treatment planning.
Keywords
cardiac conditions
imaging techniques
cardiac masses
tumor identification
cardiac myxomas
benign cardiac tumors
Erdheim-Chester disease
angiosarcoma
valvular heart disease
advanced cardiac imaging
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