Panel dyskusyjny

Ladies and gentlemen, uh, at thebeginning, I would like tothank all of the, um, speakersfor your presentations.Very complex, full of knowledgeand practical advices.

Uh, special thanks to Ana.Your, your, m- yougave us, uh, this great pleasureto, to host youhere, um, again, uh, at ourCMR workshop.And thank you very muchfor your time, for your commitmentto share out- uh, sharewith us your, um, expertise onCMR.

And, uh, m- this time,we can feel comfort about thediscussion.Uh, let me, of course,let the discussion in English, uh,due to our special guest.

Uh, on our chat, wehave only one question.Uh, it's about genetics.

Uh, so I will answer,um, that we, we do agenetics test in our hospital,University Hospital in Warsaw.So if you have, uh,any patients that you would liketo perform, uh, genetic tests,you can refer this patient toour, uh, clinic.The cost of the genetic,um, test, I think it's around1,000, but, uh, it's, uh,it- you, you- we have to,uh, check this information, but,um, I heard, uh, that isthis cost.

Um, okay, so, uhn wedon't have, uh, any more questionson our chat, but permittingme, uh, I would liketo start with, with thefirst question.

So, um, as we couldhear, uh, during, during the presentationthat in the population ofpatient with heart hypertrophy, there are,uh, diseases which are rare.Let's see Ana's case.Um, but we know thatthere are some conditions which areknown as rare because wedon't look for them the- um,when we do not performall diagnostics.For exam- uh, for example,female with hypertrophic heart scheduled forTAFI, it can be patientwith amyloidosis.

Uh, so I would liketo start with, uh, with thequestion that, um, uh, thisquestion, uh, returned to us, uh,from, uh, uh, last year.Uh, is the mapping obligatory?Um, but now concerning hypertrophic,uh, hearts, it must be includedin the protocol, uh, ofthe study or in some cases,uh, conventional, conventional techniques, uh,are sufficient to make a diagnosiswith full conviction?How it looks, uh, inyour practice?

Ana, can we start withyou, with your opinion?

Yeah, sure.Thank you very much forhaving me and hel- hello, everyone.I'm very happy I madeit to the end.

Um, so, um, what Iwould say is that typically, um,all diseases like hypertrophic cardiomyopathy,amyloidosis, um, sarcoidosis, you get morethan just clues with standardtissue characterization.So I would say thatstandard CMR, it's still enough tomake the diagnosis in mostcases.But there are some, um,conditions or some situations where, forexample, um, you cannot givecontrast.Uh, say for example, amyloidpatients, uh, m- very frequently haveimpaired renal function.In those cases, having theopportunity to still get the bestfrom CMR in terms oftissue characterization without the need ofcontrast agent, I think that'sdefinitely a plus to bear inmind.

Um, I think the pointis that when we- m- somapping is very useful inthe two extremities of the curve.So when we have veryhigh values, that's generally amyloid orhypertrophic cardiomyopathy.There have been many studiesin literature trying to also, uh,get some cutoff values orthresholds to distinguish between, for example,AL or ATTR amyloidosis andto distinguish amyloid from HCM.I'm always a bit concernedwhen I have to rely entirelyon just one number becausethere are some issues on howyou assess that.But definitely when you getvery, very high numbers, you haveto think of certain diseases,but probably where the mapping, Ifeel it's very useful, butthen I'm happy to hear th-the opinion of the otherspeakers as well, is, uh, onthe lower values of thecurve because, uh, Fabry disease typicallyhas reduced, um, values onT1 mapping.And this is very useful,for example, in the female populationbecause while male tend tohave quite early, uh, on theirdisease course, uh, LVH andyou can spot that on echoand think about Fabry andthen you get CMR, uh, andyou may get easier tothe diagnosis.Female, uh, frequently develop LVHlater on on the disease course,but CMR already shows reducedT1 values even if you havenormal wall thickness.

So in those cases, earlycases where you don't have fibrosis,so the myocardial may looknormal on postcontrast, standard postcontrast sequences,in those cases, and specificallyin the female population, having theT1 map is very helpful.But maybe not all centerscan have that, you know, straightaway.I wouldn't really panic becausewe've been working very well alsoearlier on.So I wouldn't really despairif we don't have the mappings,but I would bear inmind that there are certain situationsin which mapping is veryuseful.And in those cases, if......

Working in a center withoutmapping, I would consider referring thepatient to a center thatcan add m- mapping just to,uh, refine even more the,um, differential diagnosis.Yeah.

And I'm sure and, uh,the question from yesterday, somebody askedabout T1 values.Um, we know that T1values goes very high in amyloidosisand very down in Fabrydisease, um, is this always likethat?Uh, can we consider, uh,it as a good screening parameterfor these diseases?Maybe Justyna?

Yeah.So no, what-what- Yeah, Justyna....Important- Yeah.Yeah....

Yeah one very important thingthat I didn't mention is thatyou should always...So if you're the onedoing the report, but even moreso if you're the onereceiving the report, you need topretend to have the referencevalues, the normal values for T1and T2 mapping in theCMR lab where the scan wasperformed.Because there is a s-difference in between scans, so it'svery important to have thereference values.And I'm saying that becausein a different, completely different context,but, um, we've had manypatients referred for suspected myocarditis, justbased on slightly increased T1and T2 values.And we struggled to identifyw- what was the, um, referencevalues in the lab.So, I would really stronglyrecommend that A, we report allthe time what is normal,and secondly that we expect thatto have the normal values.

Then literature shows that thereis a wide, uh, there isnot wide, but some overlapbetween diseases and overlap between normaland diffuse fibrosis.So, I mean, I don'tlike relying on the single numberor on the single parameter.I think it's very important.The more we have, theeasier it gets.But like with echo, younever rely for judging a mitralregurgitation just on PISA radiusor just on regurgitant volume.You put pieces together.And I would do thesame with mapping as well.I don't know if theother agrees, agree but- Yeah.

...That's what I do inpractice, yeah.

Okay.Uh, thank you.Thank you very much, uh,for the answer.So maybe, uh, we will,uh, ask another speaker, so whatdo you think about theuse of mapping in hypertrophic heart?Mateusz?

Ush.Uh...So we perform- All right....T1 and T2 mapping moreoften, uh, more and more often,uh, in every patient, uh,who is referred to our, uh,lab for differential diagnosis ofleft ventricle hypertrophy.But it is not, uh,so that we always had thesesequences.Uh, we have these sequencessince 2016, I think.Uh, and before, we hadto rely on, uh, LGE, uh,and, uh, to be honest,many, many cases of, uh, hypertrophiccardiomyopathy, amyloidosis, and Fabry diseasewere detected or are detected, aredetected using only, uh, post-contrastLGE, uh, while, uh, this whatAna said that, uh, T1mapping helps us in, uh, earlystages of the disease.Uh, we, uh, cannot observe,we do not observe hypertrophy, wedo not observe, uh, anyimpairment of, uh, left ventricle strain,for example.But we still can havereduced, uh, in Fabry disease orincreased, uh, in cardiac amyloidosis,uh, T1, uh, times.

Uh, as I can...I am, uh, at, uh,the microphone I would like to,uh, have one comment anda question to each of you,uh, because, uh, we talkeda lot about extracellular volume calculation.And I would like toask you, how often do youhave hematocrit values in outpatientspa- in outpatients, uh, who arereferred for, uh, CMR dueto cardiac hypertrophy?We have this very, veryrare.Uh, in inpatients it's completelydifferent situation.And the second question is,if, uh, you don't have hematocritvalues from the blood samples,do you use this synthetic hematocrit,uh, which is described inthe literature?Uh, do you use itor is it only a researchtool?

So- Any volunteers?...Would like to start.Okay, so maybe I,I will start.Uh, so, uh, in ourhospital, um, when we regard tothe, uh, outpatient, um, theyare only, uh, referred from ourhospital.Uh, so, uh, they aregood prepared from clinicians and inthe majority of the patientthe hematocrit, uh, um, value isavailable.However, uh, when hematocrit isnot available, we, uh, at thismoment not use this, uh,systonic value.So, um, for now inour, um, for us is onlythis research tool, but probablyin the future we will useit. Okay.

And now how it isin Italy?

Um, so to be honest,very rarely outpatient come with arecent hematocrit, to be honest.Um, I tend not touse older ones.Um, um, I mean, consensuspapers do recommend to use 20,within 24 hours, if Irecall correctly.So this, it happens forinpatients, very rarely for outpatients.Um, I've used the syntheticone very rarely because it takesquite a while.So, to be honest, um,I know it's important especially fromprognostic, uh, point of viewto provide the ECV, but I'm,uh, when, you know, veryrarely when it's really mandatory toput the ECV because theyare asking for the ECV, ifthey don't have the hematocrit,I use the syntactic formula, butotherwise, I just rely onnative T1, T2, and LG, andthat's it.

Um, yes, and- And Annaor Justyna, you would like tosay here?

Yeah, so we also don'tuse the sy- synthetic values.We use either when wehave, um, our inpatients then wehave, of course, hematocrit.With outpatients, we usually onlyhave when we previously talk witha, doctor who, uh,who refer patient, and then wecan ask about this whenwe know that patient had hypertrophy,and then we ask extrafor these values.But, uh, usually, we alsodon't have it for outpatient clearly,patients.But to be honest, Idon't know how much this is,um, something we really needthis extracellular volume when we havealready T1 increase and otherpuzzles, uh, together.Uh, so, uh, but, yeah,that's, that's mi- that mi- mightbe a problem that wedon't do it in every patient.But, uh, for example,in Zurich, they, they, they u-used to do it likethis, that when patient came asoutpatient, patient, then theyperform blood gas, uh, immediately, uh,before CMR, so they havecr- uh, creatinine and hematocrit atthe same, um, quick examination.But we know that wedon't need currently, uh, according toPolish guidelines, uh, radiologic, um,guidelines to check, um, creatinine beforegadolinium contrast, so it willbe also difficult, and usually, wedon't have this machine, uh,at, um, MRI lab.

Okay.Are you, Mateusz, satisfied?

Completely satisfied.Uh- ...We have the same problemsa- around, uh, Poland and acrossthe Europe.Uh, but I agree thiswhat, uh, Justyna said and Annasaid, that native T1 mapping,uh, is providing a lot ofinformation already.So ECV, if we havehematocrit values, it's good.If not, uh, we don'tparticular care about it.Okay.

Uh, uh, uh, dear Anna,we have another question on ourchat, uh, is dedicatedfor you.Please share with us yourexpert- your experience with acute myocarditisin patients, uh, treated with,uh, immune checkpoint inhibitors.Is endomyocardial biopsy crucial insuch a cases?Recent American Heart Association expertsdocumented stated that is, um, adirect indication for biopsy.So it's- Um- CMR, buthmm.Yeah.

So, well, you know, Icome from a center where we,um, uh, I mean, histologicalvalidation is very important, 360 degrees,uh, no matter the underlyingdisease.So, you know, if, ifwe have to provide a treatment,uh, for whatever disease, weneed to have a diagnosis.And I think there arecertain situations where a diagnosismight be hard to get,uh, through other tests.And for CMR, for myocarditis,CMR is definitely very useful.It's m- the best non-invasive,um, imaging technique.But, um, it doesn't godown to, um, a cellular level,and so, um, ideally, um,well, the consensus documents also statesthat.That should be the wayto go.So, uh, having the, um,histological validation, that's a gold standardfor diagnosis.

And, um, it happened recentlyto us also because, you know,um, sometimes it's useful todo because you may have somesurprises and we've had acase of a patient, uh, hewas in the 70, 7-0,so not really, um, the properage for myocarditis and hehad a history of, um, againhe was some sort ofhematologic disorder, I don't, don't recall,uh, exactly, but he wasreceiving, um, um, immune, um, therapywith monoclonal antibodies.He came in in cardiogenicshock with complete AV block, um,very, um, difficult, um, scenarioto manage, uh, from a hemodynamicperspective and, um, he neededECMO, he needed all the medications,all the drugs to, um,help blood pressure.We decided to perform, um,endomyocardial biopsy and what it turnedout to be, it wasindeed a myocarditis, but it wasdue to, um, massive viralreplication from two different viruses andso different from what wewere expecting, but still, um, quite,uh, serious.So, uh, i- in apatient that is receiving immune suppressionbecause of a tumor andyou find that there is myocarditis,fulminant myocarditis, due to virusreally makes the management very tricky.

So I do think thatwhenever you can perform......Endomyocardial biopsy in an experiencedcenter in a safe way, oras safer as possible, andin a center where the pathologistis used to read thesamples that is the gold standard,I would say.So first, we are waitingfor, for the EC, uh, guidelines,uh, to see how, howthey will be open to dobiopsy, uh, especially when we,when the CMR, uh, isdeveloping over past years.So, so let's see.

And, uh, another question,is about the T1 values.Uh, how about reduced values,uh, uh, of T1 mapping, uh,in arrhythmogenic cardiomyopathy?So T1 mapping is, uh,going down, uh, in the, inthe fat, when, when wehave fat deposition, yeah?In the heart.But what about the arrhythmogenic?Um, it's, um, good stillto, to diagnose ACM?

I only observe fat depositionin, uh, cardiac magnetic resonance inpatients with, uh, ar- arrhythmogeniccardiomyopathy.And to be honest, uh,we perform T1 mapping as apart of a research projectin patients with, uh, arrhythmogenic rightventricle cardiomyopathy, but it isdouble-blinded, uh, study, uh, so wedon't have any, any, um,conclusions right now.But we started to investigateit.

I would, I, uh...Okay, uh......Maybe- Yeah.What we see clinically, it'susually just some spot of fat,uh, in arrhythmogenic cardiomyopathy, soit rarely causes, um, significant decreasein T1 mapping.Uh, that's why it maybealso it's not in the criteriacurrently.Uh, but, um, it's as-I w- I, I would reallylike to see, uh, conclusionsfrom, from study from Mateusz.Uh, maybe, uh, there willbe, uh, some new insight.But, uh, yeah, I, Iwould just, uh, maybe say that'sf- um, also, with T1mapping, it's, um, when we havepatients that, uh, is athletes,uh, but athletes in the- that,uh, take some anabolics.So, uh, in those patients,we see decreased T1 mapping, probablybecause of some fat,changes in the, in the heart.But this is something also,uh, which, uh, needs, uh, furtherresearch probably.

I think observation- Yeah....Of these athletes and anabolics.

Uh, we are, uh, outof time for today's meeting, butI would like a- askyou because this question is, uh,um, it's a very oftenquestioned on, on such a meetings,uh, like yesterday.Uh, we had the questionabout, um, CMR.Uh, it can be avir- virtual biopsy.So, um, it canbe a virtual biopsy to establish,um, etiology of the disease.Uh, I guess it ispossible in hemochromatosis.Uh, but what about differenttypes of amyloidosis or, or, uh,different etiology, etiologies of myocarditis.Maybe, uh, Patrycja, you can,uh, answer this question.

Uh, yeah.Um, so, um, I thinkthat, um, in the future, maybeit will be more, uh,possible, but, um, now still thebiopsy's a gold standard, uh,in this, um, diagnosis.And for example, um, whenthinking about the amyloidosis, um, Ishowed you a SAMA scale,a QOL scale, um, in whichsome authors, um, want to,uh, prove that this, um, may,um, assess the, and,um, differentiate between, uh, typeof amyloidosis.But, uh, I think this,um, should be investigated and, um,in, in the future, uh,for, um, in the more studiesbecause, uh, some, uh, authorsshow that, uh, this is possibleand another, in another studies,uh, this thesis was, um, uh,not proven.So, uh, we will seein the future, I think.

Thank you very much.Uh, to conclude.So, so we knowthat, uh, there is no doubtthat CMR is helpful, uh,and is recommended in all patients,uh, at initial evaluation, uh,when we, uh, really suspect, uh,cardiomyopathy.Uh, but, um, the diagnosticpathway has to take, uh, intoaccount in the context, whois the patient in front ofus?Uh, what is the age?Uh, what is family history,comorbidities?We are looking for otherthreats, uh, as AV blocks orother, uh, which may pointus, yes, uh, to the specific,uh, direction, understanding of theetiology in that m- particular patient.Um, this is important to,to decide on treatment, on follow-up,uh, how to monitor, uh,the disease, um, progression.

Um, thank you once againfor, for the, for all thepresentations, for your fruitful discussion.Thank Anna for connecting withus from the Italy.It, it is our greathonor, uh, to have you here.Um, thank you entire audiencefor, uh, participating in the conference,and we hope that thisnot last edition.And we, uh, we wishyou a good rest.

Goodbye, everyone.Goodbye.Thank you.Bye.Bye.Thank you very much.Bye.