Pump Failure

Understanding HEF REF: Definitions and Causes Welcome back to Pulse Check, the podcast for junior doctors who enjoy learning Australian cardiology for a change rather than sifting through American and British exam questions with drugs that aren't even available here. Have you ever looked at an echo report and scratched your head wondering whether your fluid overloaded? Patient with an ejection fraction of 20% is really going to do all that well on 20 milligrams of oral furosemide and a cardiology outpatient review in three months? Well, the answer is no, and this episode is dedicated to you. Episode 2 is a whirlwind of all things heart failure that will leave your brain swollen and edematous with knowledge. Let's subtitrate your GDMT and dive into another episode of Pulse Check. An EF of 50% or more we refer to as preserved ejection fraction. EF of 40% or less is moderately reduced and 30% or less is severely reduced. So all the four pillars, the HEF REF therapy is all studied and benefit demonstrated in the patients with an EF of 40% or less. And this is the group of patients we generally refer to as HEF REF. Regarding etiology, firstly there is ischemic cardiomyopathy. So these patients that have had infarcts in the past, drugs, alcohol, chemotherapies like antrocyclins or doxorubicin, trastuzumab, checkpoint inhibitors, think of stimulants like methamphetamine and cocaine. So these drugs are meth and cocaine when used cause global vasoconstriction, but in particular coronary vasoconstriction, but also increased contractility and increase the afterload. So the heart is working harder and getting less blood supply. So overtime this is going to create a globally reduced ejection fraction, just global muscle ischemia infarction. And sometimes you can get even very unlucky with a single hit where your coronary is just clamped down and you can have even cardiac arrest and sudden cardiac death. Genetic etiologies. I consider 5 genes in particular, but there are heaps of genes that can cause HEF, ref, Titan gene, TTN, lamin, so it's LMNA, myosin heavy chain 7, MYH, 7 desmoplykin, that's DSP and filamency, FLNC. Next are the arrhythmias, atrial fibrillation. If the rate is left uncontrolled for too long and there's a general rule, rates greater than about 110 for more than two to six weeks can cause a tachycardia mediated cardiomyopathy. The way we manage this is we get the patient into sinus rhythm. So that means using amiodarone anticoagulation for more than a month, arrange your DCR, get them back to sinus rhythm and then if you've put them on the the four pillars, the HIF ref will generally gradually recover. And if you monitor them with serial echoes, you can slowly start withdrawing the HIF ref therapy. This is recent evidence published in Australia. And generally the ejection fraction will be maintained within the normal range without reducing again, they just need close monitoring. You can't use sotalol or flecainide, the other rhythm control agents if the EF is 40% or less. It's contraindicated for sotalol in EF 40% or less and flecainide you have to avoid if they've got a structurally abnormal heart PVC induced cardiomyopathy. Frequent PVCS only merit suppression if the burden is 20% or more. These patients can get PVC induced cardiomyopathy if they've got non sustained Vt, they've got LV dysfunction, or if they have persistent symptoms. Most people would start with beta blockers. If that's not effective, you can use flecainide or amiodarone. Next, think of the infiltrative conditions and that's Febris, amyloidosis, sarcoidosis, hemochromatosis. Severe valvular conditions can cause HIF refs, so that's severe ASARMRTR. When these valveolopathies get to the point that they're reducing the ejection fraction, it's generally an indication to fix the valve and then myocarditis can also reduce the ejection fraction. Optimizing Heart Failure with Guideline-Directed Medical Therapy Regarding management, we refer to the four pillars or GDMT guideline directed medical therapy. The first pillar is your ACE inhibitor, ARB or Arnie. So ACE inhibitors like ramipril and perindopril, these have been around for a long time. There are heaps of trials that demonstrate mortality benefit and better outcomes in HFRF. Regarding Arbs, candasartan through the CHARM trial and valsartan through Val heft have both demonstrated better cardiac outcomes. The other ABS like telmisartan for example haven't got the evidence that demonstrate clear benefit, but it we would probably think it's a class effect. So I wouldn't go changing someone's telmisartan over to something else, probably just maintain if they were already on it. And then Arnie's this is secubatrel, valsartan. Secubatrel is a neprolysis inhibitor and valsartan an injitansome receptor blocker. So the combination is marked as Entresto in this demonstrated mortality benefit in the paradigm HF study. The next pillar are the cardio selective beta blockers. So this is metoprolol XL, vasoprolol and the bivalol and carvedilol. Metoprolol XL can be a bit more anti arrhythmic, so patients that have high burden VTVF metoprolol XL probably better. Vasoprolol is very cheap. If you've got a patient whose EF is not 40% or less, which you need by PBS standards to get your metoprolol XL PBS subsidized and then vasoprolol might be a better choice. Nabilol. The evidence is in older patients, but there is a gentle evasive delighting effect. So you may consider it in your young patients to reduce the risk of erectile dysfunction or in some older patients with pulmonary hypertension. And carvedilol has an alpha blocking effect, so it will reduce blood pressure as well as being a beta blocker. So you might prefer it in patients that are hypertensive despite optimal GDMT. There's also benefit in esophageal varices, portal hypertension as well. Third pillar are your Mras, mineralocorticoid receptor antagonists. This is spironolactone, aplarinone and pheneronone. Spiro has been around for a long time so bread and butter MRA. It's very cheap. A pleurinone has less anti androgenic effects, less gynecomastia. The issue with a pleurinone is that it's only PBS covered if you start it within 14 days of diagnosis of ischemic cardiomyopathy. So you can't really start Spiro, wait till they get some androgenic issue from it and then switch them over to a pleurinone. The non PBS cost of pleurinone is quite expensive so I would route only started in males with here less than 40% on their index admission with ischemic cardiomyopathy so they're covered with PBS. Whereas Spiro I would generally just use in female patients. I don't think you need to bother with her pleuritone there. Veneronone is a newer drug. It has less issues with hyperkalemia. And then the 4th pillar are the SGLT 2 inhibitors. This is your depagliflozen or impagliflozen 10 milligram doses. The renal function cut off to start these drugs is an EGFR of 20 or more that was based on the KD GO guidelines in 2024. It does drop your EGFR by a little bit. So if the EGFR does drop below 20 after you've started it, you shouldn't stop it. You should keep it going until they reach dialysis. My general approach is for a new diagnosis, cardiomyopathy before they're discharged from hospital. You'd prefer for them to be on each of the four pillars at a low dose. The higher the dose that the patient ultimately gets to, the better the outcome. So you want them on the maximally tolerated dose of each of the four medications eventually. Now with the four pillars, your currency is your blood pressure and your heart rate. So with ASAP Arnie and the Mras, they're going to drop your blood pressure. Beta blockers a little bit. SGLT twos not so much. Blood pressure is usually our biggest limitation. I would generally start a bit of either candesartan or valsartan to start with. Valsartan if I think I'm going to get them to entresto before they discharge from hospital and then start a little bit of spiraid 12.5 outside, a bit of metoprolol XL or basoprolol at a low dose and then some depagliflozin. If they've still got blood pressure to spare before discharge. I would switch the AB to Entresto 2426 BDI would prefer that their blood pressure stays above 90 systolic 85 at a stretch. If they're going home with a blood pressure of low 80s, they need to be followed up very closely in the community. You want to gradually up titrate these medications. After a few weeks of being on the medication, the blood pressure will improve and that's because of cardiac remodeling, improved stroke volume, and then you will be able to increase the doses over time until you're on maximally tolerated GDMT. Advanced Heart Failure Therapies and Referral Criteria If you are at that point in the heart rate is 77 or more and they're in sinus rhythm despite maximally tolerated beta blocker, you would add in ivabridine, which doesn't have a demonstrated mortality benefit, but it does reduce hospitalizations in the trial, Ivabridine being a funny channel blocker. Ferric carboxymaltose has reduced hospitalizations in patients who are iron deficient. So ferritin less than 100 or transferrin saturation of less than 20% with the ferritin being less than 300. The next line would be category synchronization therapy, and this is indicated if your LV ejection fraction is 35% or less despite maximally tolerated GDMT, wide left bundle branch block, more than 150 milliseconds, sinus rhythm and NYH. A class 2-3 or ambulatory class 4. They need to meet all those criteria for class one recommendation for CRT, this is a biventricular pacemaker. So a general dual chamber pacemaker will be placed in RA and RV, A CRT. The biventricular pacemaker will have an LV lead and it will go via the RA through the coronary sinus into a great vein and then sit on the outside of the LV. And so the idea of CRT is that if you've got a very wide left bundle branch block, it implies that there is desynchrony between the RV and the LV as they're contracting. So a wide left bundle branch block means that the RV is going to contract sooner than the LV, the desynchronous. So if you put a BI ventricular pacemaker, then you can make them contract more synchronously and improve the ejection fraction and the outcome. We refer to CRTD and CRTP. So CRTD is when your RV lead has AD fib coil on it and connects the defibrillator as well, whereas CRTP is when you don't have that. You just use the pacing function. If you're in atrial fibrillation, if the rate is very well controlled and you meet all the other criteria, this will be Class 2A recommendation for CRT in contrast to the other pacemakers. So say single chamber RV or dual chamber RARV. Generally with those pacemakers, you want to minimize extrinsic pacing and maximize the heart's own intrinsic activity. Whereas with the CRT you actually want to maximize the pacemaker use. And they say you need more than about 92% pacing rate and that's why your AF needs to be very well controlled in order to actually get the benefit from the CRT. In HEF ref, we want to maintain sinus rhythm. So generally if someone's in AF, the first thing you would try is putting them on amiodarone to coagulate them, do an elective DCR and get them back to sinus and try to keep them there. The other option is you can do AF ablations. A Castle AF study in 2018 took these optimized HFRF patients. They all had intracardic devices, whether it be ICD or CRT and randomized them to medical rhythm control versus AF ablation. And there were better outcomes in the AF ablation group. Then once you've done all of these things, if the patient is still deteriorating or not doing so well, then you're thinking about advanced heart failure therapies like LVAD or transplant, commonly in our heart failure clinic. So I'm not at an advanced heart failure network, but we have a lot of patients in our heart failure clinic who are on optimized 4 pillars. They have CRT DS in and they're doing pretty well in the community. We just monitor them regularly in clinic, see how they're going, make sure they're tolerating the medications, the blood pressure's stable. The my HA class is pretty good, class one or two and they're coping well. And then we just regularly follow them up. They don't need advanced heart failure therapy. They're stable, living a good life on these medications. But occasionally you will encounter a patient that is not doing so well and they need referral on to an advanced heart failure therapy. The mnemonic to keep in mind for when to refer is I need help. I is need for ironotropes. N is New York Heart Association class 4. E is worsening end organ dysfunction, ejection fraction less than 20% D for defibrillator shocks for ventricular arrhythmias. H is recurrent heart failure hospitalizations. E escalating diuretic dose. L low blood pressure. P progressive intolerance of GDMT HFRF. Managing Acute Decompensated Heart Failure These patients generally live in the community in a compensated state maintained by their medications. Sometimes they will decompensate. This is acute decompensated heart failure. ADHFI think of the eyes and the A's for precipitants. So the eyes being infection and infarction. So like a new and STEMI for example, A's being arrhythmia, anemia, adherence to medications and anti inflammatories like insets. Previously we put all these patients on fluid restrictions, but the fresh up trial published in 2025 has shown that there is actually no evidence of benefit with fluid restriction in chronic stable heart failure randomized patients to a liberal intake. And that actually ended up being a median of 1.7 liters per day versus fluid restriction and less than 1.5 S. These patients had a median of 1.4 liters per day and there was no difference in outcomes in stable heart failure patients. So I don't routinely tell patients to fluid restrict, I just advise them to have some common sense and just avoid overdoing it. You know, don't go drinking 3 liters of water a day. However, it would be reasonable to fluid restrict A decompensated heart. Family patient in hospital. Sometimes these heart failure patients will present the hospital with dyspnea or any feature that suggests they could have fluid overload and NT pro BNP can be helpful. Regarding NT pro BNP or BNP, all cardiovascular risk factors will increase your baseline BNP, except for obesity which decreases it. For a new presentation with symptoms that could be considered to be heart failure and NT pro, BNP of less than 300 nanograms per litre can essentially rule out heart failure in patients with heart failure if they're less than 50 and NT probe MP of greater than 450 suggests acute decomposition of heart failure. If the age is 50 to 75, then that number is greater than 900, and if the age is more than 75, that number is greater than 1800 to suggest decompensation. And the higher the number, the worse the LV stretch, the worse the fluid overload. Regarding diuresis, oral fruzumide has a bioavailability of about 10 to 90%, somewhere within that range. It's very variable, and if they have any right side and heart failure, they may have some gut edema and that can worsen bioavailability. To use IV ferruzamide, you should aim about 3 liters of urine output per day. If you're having a good diuresis, you can expect a creatinine rise of up to 30%. That's due to hemo concentration. You should monitor electrolytes closely because too rapid a diuresis can lead to electrolyte abnormalities as well as severe cramps. We do furosemide IV boluses. There's no evidence that infusion routinely is superior to bolus dosing. If their weight isn't coming down and they're not die resing as much as you would like, I would add spironolactone as the second agent oral and then hydrochlorothiazide as the third. If despite this they're not diuresing nicely, you can then switch to a frisimide infusion. This is if you're already on 80 milligram BDIV frisimide plus Spiro and HCT. You can start say 5 to 10 milligram per hour furosemide infusion and you can up titrate up to 20 milligram per hour. In my experience, this is usually only an issue in severe right heart failure where they're 10/20 kilograms over their dry weight. Cardiogenic Shock and Mechanical Circulatory Support Sometimes in decompensated heart failure, you will have cardiogenic shock. They're hypertensive and they have features of end organ hyperperfusion. So they have rising lactate, worsening renal liver function. They're cold peripherally. They have reduced GCS. These patients need inotropes. The two main inotropes who we use are dobutamine and muranone. And you should aim as a general rule a systolic of more than 90 and a map more than 60. Regarding dobutamine, the rate we use is about two to 10 micro per kilo per minute. Dobutamine is a beta one agonist. Regarding dobutamine and muranone, RCT's like Doremi 2021 suggests that there's no significant difference in outcomes between the two. Just use whichever you're familiar with and whichever your unit is familiar and comfortable with. I think for most people that is dobutamine. Dobutamine would be preferred if the patient was hypotensive and they've got no other presses on board because miranone will generally drop the blood pressure a bit more because it acts as a pulmonary and systemic vasodilator. So it's usually Co administered with another presser like noradrenaline. Dobutamine also preferred if they've got renal impairments compared to muranone. Muranone is ACM phosphodoasterase inhibitor with a bit of pulmonary systemic vasodilation effect. But general dose range 0.125 to 0.5 micro per kilo per minute. It would be preferred if the patient has concomitant to pulmonary hypertension or RV failure just because it has a pulmonary vasodilating effect. 1/3 onotrope is levisomendin. It's a calcium sensitizer. It's generally only used in advanced heart failure settings. In the palliative setting, it's of infusion that has a prolonged onotrophic effect. It's generally just used to give to the patient, boost their cardiac output and stroke volume. It'll have that sustained effect and it'll allow the patient to get back home for a period of time. Regarding mechanical circulatory support devices, previously we used the Intra aortic balloon pump. No trial has shown mortality benefit for IABP like the IABP Shop 2 in 2012. So it's not often used. We used it at our center up until quite recently until the Danger Shock trial. An intraaortic balloon pump is placed in the descending aorta at the level of the corona. We insert it via the femoral artery and it inflates during diastole to augment the diastolic blood pressure and hence improve the coronary blood flow. And it deflates during systole to create a vacuum effect that reduced the after load permits LV unloading. So for example, if a patient came in with STEMI and cardiogenic shock, let's say you've opened up the vessel and they're hypertensive, that's when we would put a balloon pump in for a period of time. And the general weaning of the balloon pump, it started at one to one. That's where it inflates, deflates with every heartbeat and then you reduce it to 1:00 to 2:00 and then one to four. And then it can be removed after a few days as the LV stunning and ejection fraction improves. Impella is a newer device available. It's a percutaneous micro axial flow left ventricular assist device. It's inserted via the femoral artery, sits in the LV crossing the aortic valve. So it aspirates blood and pumps it from the LV through the aortic valve into the ascending aorta. So that allows LV unloading in that way. The Danger Shock trial published last year showed small mortality benefit for Impella in STEMI with cardiogenic shock. However, it is very expensive and it's only available in a few centers. ECMO is another option. The ideal ECMO candidate would be 8, less than 75. They've got an acute reversible cardiogenic pathology. For example, they might have stimulus cardiogenic shock or they might have let's say unstable myocarditis with unstable VTVF. We see that fairly commonly or just a severely decompensated dilated cardiomyopathy in a young patient. These patients should have no significant comorbidities such as like CKD stage 5 or end stage CAPD and there should be some concern for end organ deficits. You can't do ECMO, impeller or Alvad in a patient with severe aortic regurgitation. And that's because, for example, think about an impeller. You're pumping blood from the LV across the aortic valve into the SME aorta. If you've got severe Arkansas, that blood is just going to come gushing straight back through the aortic valve back into the LV. You can create kind of like a mini circuit, and Alvad is placed by a cardiothoracic surgeon. They kind of cut out the apex of the LV. Create a kind of suction pump there and then a tubing that goes around and inserts back into the ascending aorta. So it kind of bypasses the aortic valve. So it pumps LV through the pump around the aortic valve into the ascending aorta. So if you've got severe IR there, the blood just going to come straight back down the aortic valve. You just can have a mini circuit and then ECMO, you're suctioning blood out of the femoral vein and putting it back into femoral artery. So that's going to be shot back up into the aorta. And if you've got severe AR, then blood as it comes up, the descending aorta, down the ascending aorta, it's just going to come straight through the LV. And then if you've got reduced cardio contractility and output, it's just going to kind of the LV is just going to get bigger, bigger, bigger little backlog into the pulmonary circulature and the patient will die. Regarding LVAD, there are three main destinations. So there's bridge to transplant, there's bridge to decision. And that essentially means the patient may not be a transplant candidate yet, but they could be. And there might be some kind of outstanding issue that we think they can overcome, let's say like poorly controlled diabetes, for example. But if they overcome that issue and we think they can, then they could be a transplant candidate. And the third thing is destination therapy. That's just you live with your LVAD. We don't do that in Australia. It's not funded by our Medicare. They do that in the States, but we don't, we don't have DT here. There are risks with your LVAD. There's risk of stroke. And so the patients are generally on warfarin and aspirin and as such they have a high bleeding risk. So Alva has both high bleeding and thrombotic risk. It's also very kind of Labor and staff intensive and they need psychological assessment and support as well. It is a very big undertaking for a patient to have an LVAD and then finally is transplant. So cardiac transplant is contraindicated if you have a pulmonary vascular resistance of more than three wood units, IE pre capillary pulmonary hypertension. And that's because when you have pulmonary hypertension, your native RV is going to adapt to that by dilating and hypertrophying. And so if you put in a transplant heart, then suddenly the transplant RV is not going to have adapted to that level of pulmonary hypertension and so will fail. You'll develop RV failure. Other contraindications. The following are contraindications for any kind of transplant. It's a recent malignancy. The patient's critical or unstable, they have seriously limited functional status without potential for rehabilitation. They have poorly controlled comorbidities that class two or three obesity. So BMI 35 or greater. We've got a history of non adherence to therapy. They have illicit drug or substance use or dependence with no demonstration of risk reducing behaviours or they have simply inadequate social support systems. Understanding Preserved EF Heart Failure and Myocarditis Moving on to HEF, PEF or heart failure with preserved ejection fraction. These are patients that have heart failure symptoms with diastolic dysfunction on echo and a preserved ejection fraction of 50% or more. The gold standard diagnosis is an exercise right heart Cath with a pulmonary capillary wedge pressure of 15 or more. An ECHO with an E to E prime of 15 or more, either at rest or with exercise, generally corresponds to a wedge pressure of 15 or more, which generally corresponds with an LV and diastolic pressure of 15 or more. LV EDP should be equal to left atrial pressure, which should be equal to pulmonary capellary wedge pressure unless of course you have mitral valve pathology or you have a left atrial mexoma or pulmonary vein stenosis. No medication has demonstrated mortality benefit in HEF PEF nor in heart failure with mildly reduced ejection fraction either. So EF 41 to 50% SGLT 2 inhibitors reduced heart failure hospitalization in their HEF PEF study. So that was delivered for depagliflozin and Emperor preserved for impagliflozin. They have a class one recommendation for HEF PEF. So usually HEF PEF patients if they can tolerate it and we would have them on an SGLT 2 inhibitor plus a small dose of regular oral freeze amide and as their heart failure and symptoms progress, we would gently up titrate their furozumide in the community. The Fine Arts HF study showed that Feneronone and HEFPEF and HEF mildly reduced ejection fraction, reduced heart failure, hospitalization and cardiovascular mortality, but there was no difference in all courts mortality. It's possible that if the price tag for Feneron were to come down, this may find its way into regular prescribing for HEFPEF, but at the cost at the moment we don't use it routinely. The best protective therapy for HEFPEF is lifelong regular aerobic exercise. Regarding diastology, I feel like it would be very mean of them to ask about diastolic parameters, diastolic dysfunction in their basic position training exam, particularly given that's the diastolic dysfunction guidelines updated mid 2025. But I'll talk to you a little bit about diastology. I don't expect you to remember this at all. It is a hard pill to swallow and it's very complicated. I think your time is better spent elsewhere. But in a nutshell, I'm just kidding. I'm not going to talk about it because it's borderline unlistenable. I've clipped it out and I've put it at the end of the episode. If you find yourself morbidly curious, moving on. Myocarditis generally presents as chest pain with a troponin rise. They may or may not have some dyspnea or some ECG changes or some regional motion abnormality. Unstable myocarditis is when they have hemodynamic instability, they have VTVF, or they have complete heart block. These patients merit consideration of transfer to an advanced heart failure Center for consideration of endomyocardial biopsy plus minus immunosuppression or even advanced heart failure therapy. The ideology most commonly is viral infection or simply idiopathic. You might get a bit of chest pain, a small or moderate troponin rise. You may or may not have any abnormalities on echo or ECG. For these patients we would admit them serial troponins until peak, monitor them on telemetry for any ventricular tachyarrhythmias and then if the troponin is peaked and they're feeling well, they can generally go home. Other etiologies are immune mediated inflammatory myocarditis and this includes lymphocytic, eosinophilic and giant cell myocarditis. Particularly the latter is more likely to be unstable and these are the ones that need to endomycotic biopsy. Immunosuppression myocarditis can exist as a part of an inflammatory cardiomyopathy. So this includes sarcoidosis, ARVC, arrhythmogenic right ventricular cardiomyopathy and other inherited cardiomyopathies like desmoplikein or lamin cardiomyopathy. These conditions can present as myocarditis as part of a what we call a hot phase. And then finally, you can get autoimmune myocarditis such as from checkpoint inhibitors. You can also get myocarditis from clozapine. Cardiac MRI is the gold standard for diagnosis in myocarditis. Regarding late gadolinium enhancement, it is sub endocardial sparing, it's patchy, it has enhanced T2 and which is the edema. In contrast, myocardial infarction would have sub endocardial or transmural late gadolinium enhancement that is confined to a coronary territory. Ideally all patients with myocarditis would probably get a cardiac MRI, but sometimes it's resource dependent or kind of patient dependent as well. The management should be exercise restriction for a big troponin rise could be up to three months, but a small episode of myocarditis might be just a few weeks. Diagnosing and Managing Hypertrophic Cardiomyopathy Hypertrophic cardiomyopathy. So hocum, which is hypertrophic obstructive cardiomyopathy. This is when you've got thickened walls at the level of the LV septum that actually obstructs blood flow through the LV outflow tract. Often hocum comes in conjunction with some systolic anterior motion of the mitral valve, drinks systole. When the mitral valve is closing, the anterior leaf will actually flicks back in toward the LV outflow tract. So if you've got both hocum and Sam systolic anterior motion, then you've got significant obstruction of flow through the LV outflow tract. So Hockham is a subset of hypertrophic cardiomyopathy, HCM. Hockham is quite reliant on both a filled LV and a slow LV. So that means avoiding dehydration. That's preload dependent. The more dehydrated you are, the smaller the LV cavity is and the more obstructed that LV hopefully tract will be. So you want it nice and plumping and full and hydrated. Also you want the rate to be well controlled. A fast heart rate will mean a higher gradient. Essentially, keep hydrated, avoid diuretics, avoid nitrates, anything that's going to drop your preload. Hockham will have an injection systolic murmur that's loudest at the left lower sternal edge. It is accentuated by Valsalva and it does not radiate to carotids, unlike aortic stenosis, which does. Why is it accentuated by Valsalva? If you're straining against kind of a closed glottis, you're increasing your intrathoracic pressure and so you're reducing your venous feeling of the heart and so you're getting less blood flow coming through the heart into your LV and you just getting a smaller alve cavity. Hockham can be challenging to manage if the patient has acute pulmonary edema because generally in APO we manage with diuretics and CPAP. Hockham with APO is the one scenario that we manage the APO with beta blockers and generally IV beta blockers like Esmol or metoprolol, which can be carefully titrated and potentially some very cautious gentle diuresis plus or minus kind of gentle CPAP. There's a phenomenon called the broken broth phenomenon. And that's where if you have a PVC, then the beat after the PVC will have a very high LV systolic pressure, but it'll have intensified CM systolic anterior motion with a drop in the aortic pressure. So after a PVC, the gradient between LV to aorta will be very high. HCM is generally considered to be genetic, so sarcamine mutations like MYBP C3 or NYH 7 apical HCM. So thickening of the LV wall at the apex is often sporadic rather than being genetic. As a general rule, the guidelines would suggest that first degree relatives should have one to two yearly ECG and ECHO from early adolescence until age 21, and then five yearly, you know, 2 to 5 yearly. I think ideally you would perform genetic testing on the index patient and then if you find a culprit gene variant, then you can test first degree relatives for that variant. And then if they're negative for that, that essentially rules them out of screening. It's worth noting that most cardiac genetic conditions are autosomal dominant like HCM, Lonq, T syndrome, morphine, HCM. The most common arrhythmia is atrial fibrillation. We try to keep HCM patients in sinus. Same with Brugada. The management is beta blockers or verapamil. These improve the LVOT gradient and the symptoms. Plus they provide some Vt suppression which patients with HCM are at risk of. With that LV thickening that can get scar tissue and can develop Vt if they have an LVOT obstruction of 50 or more. Plus they have a wall thickness of 15 or more, and they're symptomatic with angina despite trialling each of beta blocker and verapamil. Then they meet PBS criteria for Mavicampton, which is a cardiacomycin inhibitor that requires monthly echoes for the first six months due to risk of reduced LV ejection fraction. The alternative to Mavicampton is septal reduction. There are two options, myectomy where you do open heart surgery and essentially just cut out a bit of the LV septum or alcohol septal ablation, which is a percutaneous procedure where you isolate A septal branch from the LAD. You put a catheter down that inflate a balloon and then inject alcohol ethanol into the septal branch to cause infarction of that area of the septum. So you're inducing A myocardial infarction there and then that tissue will shrivel and thin, hence reducing dalvi thickness in that area. Whether you do myectomy or alcohol septal ablation depends on the practitioners available in your area. In some areas, there are more surgeons available to do myectomy and no one to do alcohol septal ablation. In some areas it is the opposite issue. But with Mavicampton as the new kid on the block, septal reduction strategies are falling away and most people trial Mavicampton first. Patients with HCM should have a risk calculator performed for their risk of sudden cardiac death. So you just type in ACCHCMSCD risk calculator on Google and it pops up. Plug in the details there and it will give you AI. Think it's about 5 yearly risk of SCD, some cardiac death. They're high risk. They merit a DFIB in ICD, but ICD is class one recommendation if they've had prior some cardiac arrest or they've got sustained Vt. Identifying and Treating Cardiac Amyloidosis Final thing I'll talk about here is amyloidosis. So cardiac amyloid, there are three main types of amyloid ATTR, which is transthyridine amyloidosis and AL amyloid. Light chain amyloidosis both cause cardiac amyloid whereas AA amyloidosis does not. Both present rather similarly. So you get thick walls on your ventricles, severe diastolic dysfunction. You have often have a small pericardial effusion, might have some bilateral carpal tunnel syndrome, deposition of your transthyritin or light chains. Often you have a low blood pressure with intolerance to ACE or beta blockers. There'll be low QRS voltages on the ECG which is disproportional to the thickness of the walls. Usually if you've got thick LV walls, you would expect LVH criteria. But the walls aren't thick due to muscle, they're thick due to transthatin or light chain deposition. So low QRS voltages on the ECG and often you'll have a statically raised troponin and BNP amyloid will also have poor global longitudinal strain with apical sparing. So what do I mean by this strain? Is the the percentage fractional shortening of a segment of myocardium on an echo? We divide the left ventricle into 17 segments. So there's basal, mid and distal and an apical LV. So the basal segments are closest to the mitral valve. We have 6 basal segments. There's anterior, intralateral, infralateral AKA posterior, inferior, infra, septal and anterroceptor. And we have the same 6 at the mid segment. The distal LV will have anterior, lateral, inferior and separable plus an apical segments, that's 17 segments, that's the 17 segment model. And so when we do strain imaging, we look at each 17 segments, let's pick one, let's say that the mid anterior segment and we look in diastole, how long is that segment, just physical length of the segment. And then during and systole, how much has it shortened? So usually 20 to 25% shortening is normal. We express that as a minus. So let's say -20% So global longitudinal strain is just the average of the shortening, the strain across all 17 segments. Normal GLS will be about -20 to -25%. In amyloidosis, you get poor global longitudinal strain with apical sparing, IE all the basal segments. And then the mid segments will all have a very low number, let's say -5 -, 10%. But the apex will actually have good strain. Good, good, good contraction, good shortening. So that might be -20% And so when you plot that on a bull's eye, where the middle part is the apex and the outer part, so the mid and basal segments, the apex will be cherry red and all the outside segments will be white slash blue. So that's what poor GLS with epicus bearing means. When you see some of these features in a patient, you should screen them for cardiac amyloid. And the way we do that is you send off the AL amyloidosis screening. So all three of serum protein electrophoresis, urine protein electrophoresis and serum free light change should be sent. Plus at the same time you should order a PYP scan with SPECT to look for ATR cardiac hemorrhoid. The PYP scan will compare kind of the intensity of the heart compared to the ribs. It's called a Peregrini score. And so if you're if your PYP scan is positive and your AL screening is all negative, you can diagnose ATR cardiac amyloidosis if there's any uncertainty. So if they're both negative, you can rule out cardiac amyloidosis. If the tests are equivocal, the next line will be a cardiac MRI if the patients met criteria for cardiac amyloidosis. There is one PBS medication that has mortality benefit and that is tefamidis. It's transthyretin stabilizer. PBS eligibility requires that they have LV wall thickness of 12 or more and the NYHA Class 1 to 2, so relatively early in the disease. Unfortunately, if the NYHA class 3 or 4, you can't use tefamidis on the PBS. You have to use an older agent that doesn't have evidence, like diflunicil or green tea extract. There are other agents available, more advanced drugs that target transferrin at different stages of the pathway. However, these are still in clinical trials. And so if you have a patient with cardiac amyloidosis, it's probably best that they're seen in a public clinic that specializes in cardiac amyloid because then they have access to these clinical trials and potentially these better medications. Advanced Diastolic Function Concepts and Episode Summary You've just survived another physiologically demanding episode of Pulse Check. You may be experiencing some chest discomfort and I expect you'll have a small rise in your troponin. Well, if you'd like to experience full blown Takotsubo cardiomyopathy, here's my short talk on diastology. Please enjoy. Diastolic function is all about left atrial pressure and the ability of the heart to relax. The better the heart can relax, the better the next contraction will be. When we measure diastolic function, there are a few things that we look at. So there's E prime. We do tissue Doppler at the level of the mitral valve at the septal and lateral annuli. The more brisk the mitral annulus is moving, the better the diastolic function. We also look at E to E prime. So E we do pulse wave Doppler at the mitral valve level. E is the velocity of flow during early diastole through the mitral valve and we look at A. So A is the velocity of flow through the mitral valve at light diastole when the Atria contract. And then the other thing we look at is TR velocity, because if you've got diastolic dysfunction, blood is going to kind of back flow up and you're going to get increased tricuspid regurgitation. So when we assess diastolic function based on the new guidelines, we look at 3 parameters. To start with, we look at E prime velocity. So if that's reduced, that's septal E prime of 6 centimeters per second or less, lateral lesson 7 or average 6.5 or less, that's reduced. We look at E to E prime so that if that's increased, so septal 15 or more, lateral 13 or more, average 14 or more, that's abnormal. And we look at TR velocity, if it's 2.8 meters per second or higher or they have a pulmonary artery systolic pressure of 35 or more, that's abnormal. On that note, the simplified Bernoulli equation states that the pressure difference between 2 chambers I across a valve or across a shunt is equal to four times the velocity of flow across the valve or the shunt squared. So to calculate pulmonary artery systolic pressure, it's four times TR peak velocity squared plus the right atrial pressure. And on that note, the way we calculate or estimate right atrial pressure based on the guidelines at the moment is you look at the IVC on echo. A normal IVC will be less than 20mm and it will have greater than 50% collapsibility. If both of those markers are normal, you estimate right atrial pressure to be about 3 MHG. If one of those is abnormal, you say it's 8, and if both markers are abnormal you say it's 15 MHG. So PASP is 4 times trusted. Regards velocity squared plus estimated right atrial pressure. So looking at those three factors, reduced E prime velocity, increase E to E prime and increase TR velocity. And if all of those are normal, then we can say they have normal left atrial pressure and normal diastolic function. Left atrial pressure is like LV feeling pressure, IE they're overloaded, probably got some degree of pulmonary congestion. So you've got increased left atrial pressure, and if you've got increased left atrial pressure, you should have some diuresis. You've your volume overloaded. So if they're all normal, they have normal left atrial pressure, normal diastolic function. If all three are abnormal, then you have increased left atrial pressure. And then we look at the E to A. The E to A waveforms are like our secret weapon to understanding diastolic function and left atrial pressure. And E dominant waveform where E is greater than A suggests that we either have grade two or three diastolic dysfunction with elevated left atrial pressure. Or that we have a young patient with strong LV suction ability where the LV is relaxing very well and sucking blood from the left atrium through the mitral valve into the LV. Whereas an A dominant pattern where A is greater than E suggests that you have Grade 1 diastolic dysfunction, however you have normal left atrial pressure. When you combine this data with the E prime E to A and the TR velocity, you get a good understanding of whether they have normal diastolic function and left atrial pressure or whether they have grade 1-2 or three diastolic dysfunction with or without elevated left atrial pressure. Hopefully that gives you an idea about diastolic function. And with that said, I don't think it's worth remembering any of it.