Doppler Systolic Signal Void in Hypertrophic Cardiomyopathy: Apical Aneurysm and Severe Obstruction without Elevated Intraventricular Velocities

Po, Jose Ricardo; Kim, Bette; Aslam, Farhan; Arabadjian, Milla; Winson, Glenda; Cantales, Deborah; Kushner, Josef; Kornberg, Robert; Sherrid, Mark V.

doi:10.1016/j.echo.2015.08.015

Cited by 24 publications

(37 citation statements)

References 33 publications

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“…In HCM with mid-LV obstruction, the LV apex can be subjected to very high impedance to ejection, which can lead to an apical akinetic aneurysm 64 . The same midsystolic drop in LV ejection velocities is seen just before the obstructing segment of the left ventricle (FIG.…”

Section: [H3] Resting Wall Motion Abnormalitiesmentioning

confidence: 99%

Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy

2016

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Evidence indicates that anatomical and physiological phenotypes of hypertrophic cardiomyopathy (HCM) stem from genetically mediated, inefficient cardiomyocyte energy utilization, and subsequent cellular energy depletion. However, HCM often presents clinically with normal left ventricular (LV) systolic function or hyperkinesia. If energy inefficiency is a feature of HCM, why is it not manifest as resting LV systolic dysfunction? In this Perspectives article, we focus on an idiosyncratic form of reversible systolic dysfunction provoked by LV obstruction that we have previously termed the 'lobster claw abnormality' — a mid-systolic drop in LV Doppler ejection velocities. In obstructive HCM, this drop explains the mid-systolic closure of the aortic valve, the bifid aortic pressure trace, and why patients cannot increase stroke volume with exercise. This phenomenon is characteristic of a broader phenomenon in HCM that we have termed dynamic systolic dysfunction. It underlies the development of apical aneurysms, and rare occurrence of cardiogenic shock after obstruction. We posit that dynamic systolic dysfunction is a manifestation of inefficient cardiomyocyte energy utilization. Systolic dysfunction is clinically inapparent at rest; however, it becomes overt through the mechanism of afterload mismatch when LV outflow obstruction is imposed. Energetic insufficiency is also present in nonobstructive HCM. This paradigm might suggest novel therapies. Other pathways that might be central to HCM, such as myofilament Ca2+ hypersensitivity, and enhanced late Na+ current, are discussed

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Section: [H3] Resting Wall Motion Abnormalitiesmentioning

confidence: 99%

Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy

2016

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“…A midventricular peak pressure gradient of 70 mm Hg is an independent predictor of LVA formation . Interestingly, following LV apical aneurysm formation, previously significant pressure at the midventricular obstruction level may no longer be generated . Therefore, complications may still progress in the absence of high velocity and pressure gradient at the midventricular obstruction level once apical aneurysm has formed .…”

Section: Discussionmentioning

confidence: 99%

“…Interestingly, following LV apical aneurysm formation, previously significant pressure at the midventricular obstruction level may no longer be generated . Therefore, complications may still progress in the absence of high velocity and pressure gradient at the midventricular obstruction level once apical aneurysm has formed . Midventricular obstruction leads to cessation of systolic flow at midventricular level resulting in blood trapping in the apical chamber (Figure A).…”

Section: Discussionmentioning

confidence: 99%

“…Midventricular obstruction leads to cessation of systolic flow at midventricular level resulting in blood trapping in the apical chamber (Figure A). When midventricular obstruction is relieved, blood is allowed to flow from apex to base in late systole or early diastole constituting the basis for paradoxical jet flow (Figure B) . Increased pressure at the apex as a result of blood trapping may lead to apical oxygen‐supply mismatch.…”

Section: Discussionmentioning

confidence: 99%

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Left ventricular aneurysms in hypertrophic cardiomyopathy with midventricular obstruction: A systematic review of literature

Elsheshtawy

Mahmoud

Abdelghany

et al. 2018

Pacing Clinical Electrophis

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“…Doppler LV mapping in diastole, may be useful to understand the pathophysiology of HCM, particularly in complex condition like that of mid cavity obliteration [3].…”

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confidence: 99%

Left ventricular diastolic gradient in hypertrophic cardiomyopathy

Pacelli

Rapacciuolo

Giamundo

et al. 2017

IJC Heart & Vasculature

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Doppler Systolic Signal Void in Hypertrophic Cardiomyopathy: Apical Aneurysm and Severe Obstruction without Elevated Intraventricular Velocities

Cited by 24 publications

References 33 publications

Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy

Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy

Left ventricular aneurysms in hypertrophic cardiomyopathy with midventricular obstruction: A systematic review of literature

Left ventricular diastolic gradient in hypertrophic cardiomyopathy

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