Brian Goldstein

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I placed the systolic function in gray because typically systolic function is preserved, at least preserved until very late in the clinical presentation with difficulties, and so I think most of these problems boil down to problems with the vascular resistance across the pulmonary bed, probably in the systemic bed and diastolic function of the systemic ventricle.

quote · Single Ventricle / HLHS

So, um, we did, but it didn't prove meaningful in our cohort, and I think that unfortunately may be a lesson from. You know, Fontaine patients in general that the easy biomarker has proved difficult to identify because their EDP is probably quite low and it's not straining their atrium if you like.

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Brian's statements about Single Ventricle / HLHS 115 statements

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Case Review Dynamic Assessment of the Fontan Part I: New Horizons in Medical...

Quotation I'm happy to have learned that my left ventricular and diastolic pressure is not 20, uh, facing my 40th birthday in a couple of weeks. Open

Quotation In any circulation, blood flow per unit time is dependent upon the drop in pressure across the vascular bed. And the vascular resistance, of course, Ohm's law. Open

Statement In any circulation, blood flow per unit time is dependent upon the drop in pressure across the vascular bed and the vascular resistance (Ohm's law). Open

Quotation In any circulation, blood flow per unit time is dependent upon the drop in pressure across the vascular bed. And the vascular resistance, of course, Ohm's law. Open

Statement In any circulation, blood flow per unit time is dependent upon the drop in pressure across the vascular bed and the vascular resistance (Ohm's law). Open

Statement Biventricular circulation is characterized by low right ventricular pressure, low pulmonary arterial pressure, and low pulmonary vascular resistance. Open

Statement During exercise in biventricular circulation, cardiac output can increase to approximately 5 times baseline cardiac output. Open

Statement The pulmonary vascular resistance falls characteristically during exercise in biventricular circulation, allowing for substantial augmentation of pulmonary blood flow. Open

Statement In the Fontan circuit, the pulmonary vascular resistance is typically both fixed and elevated. Open

Quotation Thus, even with a moderate elevation in central venous pressure, the pulmonary blood flow is limited, which limits systemic blood flow. Open

Statement In Fontan circulation, baseline cardiac output is typically near normal but a bit reduced, 70 to 80%. Open

Statement Central venous pressure in Fontan patients is chronically elevated because there is no ventricle to do the work of the subpulmonary blood flow. Open

Statement With exercise in Fontan patients, cardiac output increases quite a bit more limited than in the two ventricle population, characterized by increased central venous pressure and increased pulmonary vascular resistance. Open

Statement At rest with zero exercise, cardiac output in typical or good Fontan patients is 70 to 80% of a normal biventricular patient, and identifying differences between these two populations may be subtle. Open

Quotation However, with augmentation of cardiac output or with stress or exercise, the difference between the Fontan patient and the biventricular patient becomes substantially increased, and identifying abnormalities or limitations in the Fontan circuit becomes quite a bit easier. Open

Statement With augmentation of cardiac output or with stress or exercise, the difference between the Fontan patient and the biventricular patient becomes substantially increased. Open

Statement The limitations of cardiac output in the Fontan circulation can be distilled down to pulmonary vascular resistance, diastolic function of the ventricle, and systolic function of the ventricle. Open

Quotation I placed the systolic function in gray because typically systolic function is preserved, at least preserved until very late in the clinical presentation with difficulties, and so I think most of these problems boil down to problems with the vascular resistance across the pulmonary bed, probably in the systemic bed and diastolic function of the systemic ventricle. Open

Statement Systolic function is typically preserved in Fontan patients, at least preserved until very late in the clinical presentation with difficulties. Open

Statement Most or all variables in Fontan circulation may be pretty typical or near normal at rest, thus to understand limitations, one must evaluate these variables in a dynamic or stressed state. Open

Quotation To the extent that rest and cardiac output is near normal, as we just talked about, most or all of these variables may be pretty typical or near normal at rest. And thus to understand these limitations, one must evaluate these variables in a dynamic or stressed state. Open

Statement In a series of Fontan patients who underwent supine exercise with invasive hemodynamics, pulmonary vascular resistance was elevated at rest and with exercise, the pulmonary vascular resistance was quite static and did not decrease as expected with maximal exercise. Open

Statement In patients with biventricular circulation, pulmonary vascular resistance begins lower and falls with exercise. Open

Quotation Importantly, if you compare this graphic to patients who have biventricular circulation, their pulmonary vascular resistance begins lower and falls with exercise. Open

Statement The Pediatric Heart Network Fuel trial is a randomized trial of 400 Fontan patients, with 200 receiving udenafil (a PDE5 inhibitor) and 200 receiving placebo, evaluating exercise capacity after 6 months of therapy. Open

Statement With echocardiographic assessment of diastolic function, nearly 3/4 of Fontan patients demonstrate abnormalities of early relaxation or elevated atrial filling pressure, suggesting problems with diastolic function are quite prevalent. Open

Statement In a study comparing Fontan patients with normal diastolic function versus those with diastolic dysfunction by echocardiography, patients with diastolic dysfunction had reduced peak VO2 and peak work with both supine and upright exercise. Open

Quotation Now the the diagnosis of diastolic dysfunction, as we've heard earlier from Doctor Reddington, may be difficult. Um, first, we don't have population specific validated non-invasive measures. Open

Statement Echocardiographic measures of diastolic function have not been validated in a Fontan population. Open

Statement Invasive assessment of end-diastolic pressure in symptomatic Fontan patients is frequently unrevealing in the resting state because patients are often preload starved from being NPO, lying supine, and not undergoing stress. Open

Quotation Invasive assessment of the end diastolic pressure, the gold standard for diastolic dysfunction, even in the symptomatic Fontan patient, it is frequently unrevealing in the resting state. Open

Quotation So locally we generated a protocol of rapid volume expansion to perform what we'd call a ventricular stress testing to evaluate for what we've termed occult diastolic dysfunction, which is just diastolic dysfunction. It's not revealed at baseline under those non-stress conditions. Open

Statement In a protocol of rapid volume expansion (ventricular stress testing) in 46 Fontan patients, ventricular filling pressure (end-diastolic pressure) was significantly increased after exposure to volume, while transpulmonary gradient, pulmonary vascular resistance, and cardiac index were not significantly affected. Open

Quotation On the whole, about 35% of patients demonstrated what we termed occult diastolic dysfunction, which was defined as a post volume challenge and diastolic pressure of greater or equal than 15 millimeters of mercury. Open

Statement About 35% of Fontan patients demonstrated occult diastolic dysfunction, defined as a post volume challenge end-diastolic pressure of greater than or equal to 15 millimeters of mercury. Open

Statement In univariate analysis, higher baseline end-diastolic pressure, longer duration of Fontan circulation, and lower baseline cardiac index were associated with higher fluid challenge end-diastolic pressure. Open

Quotation And this makes some intuitive sense that the longer duration of exposure, just like the longer duration of life in a standard biventricular circulation, is associated with a progressive increase in filling pressure and diastolic stiffness. Open

Statement Longer duration of Fontan circulation was associated with a greater change in filling pressure during volume challenge. Open

Statement In multivariable analysis, baseline end-diastolic pressure was the only finding associated with the final end-diastolic pressure after volume challenge. Open

Quotation Um, we've published the 46 patients, we've now performed testing in over 100 patients, and we've shared this protocol with a number of other large congenital heart centers, uh, who are interested in utilizing this to really tease out who has and who does not have that degree of diastolic dysfunction. Open

Statement The volume challenge protocol has been shared with a number of other large congenital heart centers and has now been performed in over 100 patients. Open

Quotation And we know that Fontaine patients can have the presence of mechanical dissynchrony without electrical dissynchrony. This contributes to overall ventricular dysfunction, both systolic and diastolic, and reducing that dissynchrony could improve mechanics, which could improve symptomatic patients. Open

Statement Fontan patients can have the presence of mechanical dyssynchrony without electrical dyssynchrony, which contributes to overall ventricular dysfunction, both systolic and diastolic. Open

Quotation And in theory, acute improvements in ventricular mechanics could translate to long-term clinical benefits. Of course this is an unproven assertion. Open

Statement Acute improvements in ventricular mechanics from pacing could translate to long-term clinical benefits, though this is an unproven assertion. Open

Quotation And in theory, acute improvements in ventricular mechanics could translate to long-term clinical benefits. Of course this is an unproven assertion. Open

Statement Acute improvements in ventricular mechanics from pacing could translate to long-term clinical benefits, though this is an unproven assertion. Open

Statement In the catheterization laboratory, a multimodality setup with conductance catheter feeds, electrical anatomic mapping, fluoro, ECG, and voltage mapping can identify ideal pacing sites and demonstrate ventricular mechanic changes at each site. Open

Quotation Dynamic assessment of the Fontan circulation might provide substantial insight into what can be beyond what can be obtained in the resting state. Open

Statement Dynamic assessment of the Fontan circulation might provide substantial insight beyond what can be obtained in the resting state. Open

Quotation Dynamic assessment of the Fontan circulation might provide substantial insight into what can be beyond what can be obtained in the resting state. Open

Quotation By combining disciplines and expertise across uh cardiology domains, we may be able to offer complex Fontan patients improved insight into the mechanisms of Fontan limitation and Fontan failure and hopefully uh translate these mechanisms into potential solutions to improve both functionality and quality of life. Open

Statement Pathologies in pulmonary vascular resistance and diastolic function are likely to play critical roles in the limitations of Fontan patients. Open

Quotation I think one of the issues with large scale trials, for example, the fuel trial, which has 400 patients, is that we haven't, uh, uh, made efforts to identify those patients who specifically have problems with pulmonary vascular resistance before inclusion in the trial. Open

Statement In the Fuel trial with 400 patients, efforts have not been made to identify those patients who specifically have problems with pulmonary vascular resistance before inclusion in the trial. Open

Statement Patients with relatively low pulmonary vascular resistance but substantial ventricular noncompliance may not respond to PDE5 inhibitor therapy if the drug does not have lusotropic effects to overcome ventricular fibrosis and noncompliance. Open

Quotation So I think it's incumbent on us at this stage to to treat patients as individuals and utilize a combination of non-invasive and invasive testing with stress to tease out if the primary problem is a vascular resistance problem through the lung circulation, if the primary problem is ventricular stiffness, or if the primary. The problem is a substantial combination of both and target their therapies towards what we have and what we can use to those specific problems in our future. Open

Quotation Yes, absolutely true, um, we did look at both overall change in EDP per unit time and everyone's unit time. is the same, so that reflects slope. We also looked at those who got to an absolute higher number. Both populations are known to us Open

Statement BNP was measured after exercise in the invasive exercise cohort, and almost universally it was a meaningless measure, with every patient's number between 15 and 40. Open

Quotation Yeah, so this is a great question. We did measure BMP. We measured BMP after exercise, and almost universally it's a meaningless measure. Um, we, every patient's number was between 15 and 40, and it's hard to tease out substantive differences in relatively modest sized populations, 50, 60 patients between numbers that are that small. Open

Statement BNP was measured after exercise in the invasive exercise cohort, and almost universally it was a meaningless measure, with every patient's number between 15 and 40. Open

Quotation So, um, we did, but it didn't prove meaningful in our cohort, and I think that unfortunately may be a lesson from. You know, Fontaine patients in general that the easy biomarker has proved difficult to identify because their EDP is probably quite low and it's not straining their atrium if you like. Open

Statement The easy biomarker has proved difficult to identify in Fontan patients because their end-diastolic pressure is probably quite low and not straining their atrium. Open

Quotation Yeah, stay tuned. I think we have access to all of these data with this specific cohort of 100 patients, so we have all these invasive stress data for us, so we'll see how that plays out, what we identify. Open

Summaries Brian gave as host · 1 summary

Recaps of other experts' statements, not Brian's own clinical position.

Summaries Brian gave as host · Single Ventricle / HLHS 1 summary

Open the Single Ventricle / HLHS collection →

Case Review Dynamic Assessment of the Fontan Part I: New Horizons in Medical...

Host summary Brian Goldstein summarizing the discussion: With echocardiographic assessment of diastolic function, nearly 3/4 of Fontan patients demonstrate abnormalities of early relaxation or elevated atrial filling pressure, suggesting problems with diastolic function are quite prevalent. Open