Andrew Lotz
77 statements · 1 topic
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if you don't label them destination, you're always thinking you're trying to get them to transplant candidacy, which maybe would be a longer um life, prob probably better quality of life, although that's yet to be determined.
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Single Ventricle / HLHS
the reason is it will allow flow into the vat where the restrictive or small cavityed ventricle sometimes clamps around it and not, does not allow it to flow. We've definitely seen that phenomenon.
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Single Ventricle / HLHS
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Andrew's statements about Single Ventricle / HLHS 77 statements
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Transplantation and Ventricular Assist Devices: New Horizons in Medical and...
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we don't have, um. A ton of experience at each center and we are getting patients as fast as we can think about them.
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when you think about how I'm presenting this, just remember there's a part to each slide that is probably another hour.
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We're trying to prevent those end-stage patients from coming to us and more focusing on that late-stage, um, patient and what we can do for them.
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In the Bernstein 2006 pediatric heart transplant study group multi-center analysis, risk factors for poor Fontan transplant outcomes included mechanical ventilation at listing, younger age (0-4 years), status 1 listing, and shorter time interval from Fontan (less than 6 months).
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If Fontan patients are selected appropriately, they do just as well as dilated cardiomyopathy patients post-heart transplant.
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if you pick the right patients, they do just as well as your other dilated cardiomyopathy patients, um, post-heart transplant.
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Protein-losing enteropathy causes accumulating risk factors including edema, low albumin, poor nutrition, electrolyte disturbances, low IgG with increased infections, malnutrition, and prothrombotic state.
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if you have a patient that's been sitting with PLE for 5 years and then we wanna put a VAT in them, they're very prothrombotic
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A patient with PLE for 5 years becomes very prothrombotic, which is important for VAD outcomes, and becomes malnourished, making them a poor transplant or VAD candidate.
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you've had a patient that's been malnourished, now wants a transplant, now thinking about a VAT, and they're in very poor shape.
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The 1998 Mertens study showed approximately 50% survival at 5 years after PLE diagnosis.
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Schumacher from Michigan showed 50% freedom from death or transplant at 5 years in patients diagnosed with plastic bronchitis.
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having taken care of a handful of these patients that have gone through to transplant, they do do very well post-transplant.
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Plastic bronchitis patients who go through to transplant do very well post-transplant.
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the interesting part is how they wait and they're waiting at home with these casts having respiratory insufficiency at times that's pretty severe.
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Current pediatric transplant survival across all centers is 92% at 1 year and 86% at 5 years.
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Fontan transplant survival, aggregating available data, is approximately 80% at 1 year and 75% at 5 years.
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Multi-center Fontan post-transplant studies show higher waitlist time and mortality compared to dilated cardiomyopathy.
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Fontan transplant patients have increased risk for early graft failure post-transplant.
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Bleeding is more common in Fontan transplant patients since they are multiple-time redo surgeries.
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while you, um, as congenital physicians may hold on to your PLE patients and want them transplanted in a certain time point, they will sometimes wait for years.
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Under recent pediatric allocation system changes, congenital patients on one inotrope can be status 1A only if they are in the hospital; Fontan patients with PLE or plastic bronchitis who are not on inotropes will be status 2.
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Approximately one year ago, patients could be sent home on low-dose dopamine or milrinone and remain status 1A; this is no longer the case under current allocation rules.
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all of those patients that we've, you may have heard about that small, um, low-level dose dopamine helping PLE can no longer go home like that.
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if it's an urgent placement, which this child seems that he needs some urgent mechanical support, um, we are now for the most part using continuous flow.
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For urgent VAD placement at Cincinnati Children's, continuous-flow short-term devices (CentriMag in particular, some institutions use Rotaflow) are used to support patients for weeks to months to transplantation.
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The Berlin Heart is still available for small children, but most Fontans are bigger, so HVAD is increasingly used.
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HVAD can be safely placed in 25 kg single-ventricle patients, and many institutions (including Cincinnati) may push down to 15 kg.
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continuous flow for these single ventricle physiologies really seems to be working. Very well compared to our Berlin data.
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Continuous-flow devices for single-ventricle physiology seem to be working very well compared to Berlin Heart data.
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For patients over approximately 35 kg, SynCardia total artificial heart is an option, removing all ventricular mass and supporting the patient.
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that's something that we as a bad community are working very hard at trying to figure out the best way to support these patients, and we're working very quickly, um, since it's a growing population.
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In the Excor (Berlin Heart) registry, there were only 5 stage 3 (Fontan) patients, with 3 survivors; days on support were 1, 3, and 229 days, representing a very limited dataset.
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The Intermacs adult registry shows only 17 single-ventricle patients, and the device type used is not clear in the registry.
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If a patient does not need respiratory support, supporting an organ that doesn't need support should probably be avoided, which is why ECMO is not the preferred option when lungs are functioning well.
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if you don't need respiratory support, we really feel like supporting an organ that doesn't need support, we should probably veer away from.
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the real question is not only what devices should we put in these patients to support them both long and short term, but are we going to make them better?
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For SynCardia placement in single-ventricle patients, a capacitant chamber must be built because two AV valves are needed or the anatomy must be addressed in some fashion.
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A smaller 50 cc SynCardia device is now available, making it an option for smaller Fontan patients.
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there is a smaller device now, the 50 cc Synchardia device, so the smaller fontans will have that option as well.
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Among all adults listed as status 2 (where PLE adult Fontan patients would be), only 30-40% get transplanted within one year.
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Among congenital heart disease patients on medical therapy (not all Fontans), one-third would be transplanted at 1 year.
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I think we really need to think about the surveillance of end organs and early referral.
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since there is a paucity of organs, we really have to think about what patients are ready to go towards transplantation.
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The advanced cardiomyopathy learning network is now operational and working to share experiences with Fontan and VAD to understand which patients benefit from VAD and what the best support strategy is.
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we do have a um advanced cardiomyopathy learning network that's now up and running that we're trying to share our experiences with Fontan and VA to understand which patients will benefit from a VA and what the best support strategy is.
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The upcoming adult allocation system changes will be more favorable for Fontan patients, but the recent pediatric allocation changes were not favorable for Fontan PLE or plastic bronchitis patients.
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The adult allocation system is going to be a little bit more in our favor. The changes that are going to be made, but the pediatric was just changed and was not in the Fontian PLE plastic bronchitis favor.
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I think we need clear criteria for transplant referral at each center and then across centers, and then a seamless collaboration between our transplant teams and our congenital teams.
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In pediatric VAD practice, 'destination therapy' has been reframed as 'chronic therapy,' with the concept that patients can transition in and out of transplant candidacy status.
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as pediatric bad physicians, we've really used destination theory more. We've kind of changed the lingo to chronic therapy, thinking that they can. You know, go in and out of their status.
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if you don't label them destination, you're always thinking you're trying to get them to transplant candidacy, which maybe would be a longer um life, prob probably better quality of life, although that's yet to be determined.
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If a patient is not labeled as destination therapy, the goal remains getting them to transplant candidacy, which may result in longer life and probably better quality of life, although that is yet to be determined.
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I think then you can place a device with hopes to make their end organs better, resolve their PLE, get them better nutrition. Um, with the hope to get to transplant candidacy.
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There are only about 20 Fontans in all of Intermacs and Pedimacs (the two VAD registries) available to study.
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will it help them and will it improve their quality of life or get them, give even get them out of the hospital.
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there's only 20 or so fontans in all of Innermax and PDMx, which is the two VA registries that we have, um, to even study.
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We've been talking a lot about that in our restrictives as well as our fontans. If you have a small left ventricular cavity or single ventricle cavity, can you put the vat in the atrium?
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A handful of Fontan patients have been successfully supported with atrial cannulation.
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There is a handful of patients that have been. Successfully supported with Fontan's, um, with an atrial cannulation
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learning from our atrial cannulation in the restrictives with the Berlin data, um, they do not do as well as a ventricular a canulation.
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In restrictive cardiomyopathy, Berlin Heart data shows that atrial cannulation does not perform as well as ventricular cannulation.
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I think that's yet to be determined in the Fontan and we are extrapolating data from our restrictive experience
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Atrial cannulation allows flow into the VAD where a restrictive or small-cavity ventricle sometimes clamps around the cannula and does not allow flow; this phenomenon has been observed.
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the reason is it will allow flow into the vat where the restrictive or small cavityed ventricle sometimes clamps around it and not, does not allow it to flow. We've definitely seen that phenomenon.
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