Role of imaging: Insights from the cardiac catheterization?

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1 History and Clinical Presentation of Hypertrophic Cardiomyopathy Role of imaging: Insights from the cardiac catheterization? Stavros Hadjimiltiades MD, FACC, FACP Associate Professor in Cardiology Focal distribution of myocyte disarray Varnavaa AM et al., Heart 2000;84:

2 Hypertrophic cardiomyopathy ventriculogram Hypertrophic trabeculation Small chamber size Systolic cavity elimination Mitral regurgitation

3 Hypertrophic cardiomyopathy ventriculogram diastole diastole diastole systole Apical hypertrophy

4 Hypertrophic cardiomyopathy ventriculogram apical aneurysm Of 1299 HCM patients, 28 (2%) were identified with left ventricular apical aneurysms only half of them recognized by echo (Circulation. 2008)

5 Hypertrophic cardiomyopathy ventriculogram apical aneurysm Extreme mid ventricular obstruction Over years of follow up, 12 patients (43%) with left ventricular apical aneurysms experienced adverse disease complications (event rate, 10.5%/y), including sudden death, appropriate implantable cardioverter defibrillator discharges, nonfatal thromboembolic stroke, and progressive heart failure and death. (Circulation. 2008)

6 The Coronaries Compression N Engl J Med 1998;339:1201 (Myocardial bridging poor outcome in children) J Am Coll Cardiol 2000;36:2270 (Myocardial bridging no increased risk of death in adults) J Am Coll Cardiol 2003;42:889 (LVH hypertrophy and compression of intramyocardial branches may contribute to myocardial perfusion abnormalities)

7 Major events that comprise the history of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

8

9 Hypertrophic cardiomyopathy measuring the gradient

10 Characteristics of an entrapped catheter delay in the decline in LV pressure beyond the arterial dicrotic notch

11 Measuring the gradient level of obstruction LV outflow Aorta

12 Aortic stenosis The Brockenbrough Braunwald Morrow sign

13 The ill and unfit choice of words wonderfully obstructs the understanding. Sir Francis Bacon JM Criley and RJ Siegel Has 'obstruction' hindered our understanding of hypertrophic cardiomyopathy? Gauer phenomenon

14 angiographic and hemodynamic events in hypertrophic cardiomyopathy 140 ms 340 ms

15

16 LVOT obstruction

17 Myectomy and flow drag

18 The phases of the Valsava maneuver Nishimura et al. Mayo clinic proceedings. 2004;79:

19 Dynamic Nature of the Obstruction and Its Dependence on Loading Conditions and Contractility of the Left Ventricle

20 Dynamic Nature of the Obstruction and Its Dependence on Loading Conditions and Contractility of the Left Ventricle I II III

21 Dynamic Nature of the Obstruction and Its Dependence on Loading Conditions and Contractility of the Left Ventricle Base Low Dose Isoproterenol High Dose Isoproterenol

22 Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction 30% <30 mmhg gradient Maron M, Circulation. 2006

23 Improvement in diastolic filling after relief of the outflow tract obstruction

24 Low LVEDP with a continuing decline of pressure over the middiastolic period (prolonged relaxation phase)

25 What is What the is true the LVEDP true LVEDP and and why why is this this value value changing?

26 Left Ventricular End Diastolic Pressure During Dynamic Exercise in Patients With Nonobstructive Hypertrophic Cardiomyopathy J Am Coll Cardiol 2001;38:335

27

28 Invasive measurement of of diastolic function

29 Left ventricular (LV), aortic (Ao) and left atrial pressures during AV sequential pacing

30 Unchanged systolic gradient with pacing Systolic gradient Improvement with pacing

31 Pacing and Diastolic function Nishimura R, JACC 1996

32 Pacing and Diastolic function Optimal AV delay P synch 60 Optimal AV delay P synch 60 P synch 60 Optimal AV delay P synch 60 Optimal AV delay Nishimura R, JACC 1996

33 Evolution of peak left ventricular outflow tract gradient (mm Hg), before pacemaker implantation (baseline), and during early and late follow up after pacing Galve E, Heart :

34 Heart Catheterization For many years has been the only method to study the physiology of hypertrophic cardiomyopathy Certain aspects of the diastolic function can only be reliably obtained with catheterization Nowadays it is mainly limited to the anatomic definition of the coronary tree

35

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