Reflections of inflections in hypertrophic cardiomyopathy.

The almost 50-year odyssey of cardiac imaging in hypertrophic cardiomyopathy (HCM), revisited and described here, has been remarkable, particularly when viewed in the timeline of advances that occurred during a single generation of investigators. At each step along the way, from M-mode to 2-dimensional echocardiography to Doppler imaging, and finally over the last 10 years with the emergence of high-resolution tomographic cardiac magnetic resonance (CMR), evolution of the images generated by each new technology constituted a paradigm change over what was previously available. Together, these advances have transformed the noninvasive diagnosis and management of HCM in a number of important clinical respects. These changes include a more complete definition of the phenotype, resulting in more reliable clinical identification of patients and family members, defining mechanisms (and magnitude) of left ventricular outflow obstruction, and novel myocardial tissue characterization (including in vivo detection of fibrosis/scarring); notably, these advances afford more precise recognition of at-risk patients who are potential candidates for life-saving primary prevention defibrillator therapy. This evolution in imaging as applied to HCM has indelibly changed cardiovascular practice for this morphologically and clinically complex genetic disease.

The Remarkable 50 Years of Imaging in HCM and How it Has Changed Diagnosis and Management: From M-Mode Echocardiography to CMR

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.

Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy.

BACKGROUND Acute left ventricular (LV) apical ballooning with normal coronary angiography occurs rarely in obstructive hypertrophic cardiomyopathy (OHCM); it may be associated with severe hemodynamic instability. METHODS, RESULTS We searched for acute LV ballooning with apical hypokinesia/akinesia in databases of two HCM treatment programs. Diagnosis of OHCM was made by conventional criteria of LV hypertrophy in the absence of a clinical cause for hypertrophy and mitral-septal contact. Among 1519 patients, we observed acute LV ballooning in 13 (0.9%), associated with dynamic left ventricular outflow tract (LVOT) obstruction and high gradients, 92 ± 37 mm Hg, 10 female (77%), age 64 ± 7 years, LVEF 31.6 ± 10%. Septal hypertrophy was mild compared to that of the rest of our HCM cohort, 15 vs 20 mm (P < 0.00001). An elongated anterior mitral leaflet or anteriorly displaced papillary muscles occurred in 77%. Course was complicated by cardiogenic shock and heart failure in 5, and refractory heart failure in 1. High-dose beta-blockade was the mainstay of therapy. Three patients required urgent surgical relief of LVOT obstruction, 2 for refractory cardiogenic shock, and one for refractory heart failure. In the three patients, surgery immediately normalized refractory severe LV dysfunction, and immediately reversed cardiogenic shock and heart failure. All have normal LV systolic function at 45-month follow-up, and all have survived. CONCLUSIONS Acute LV apical ballooning, associated with high dynamic LVOT gradients, may punctuate the course of obstructive HCM. The syndrome is important to recognize on echocardiography because it may be associated with profound reversible LV decompensation.

Hypertrophic cardiomyopathy with dynamic obstruction and high left ventricular outflow gradients associated with paradoxical apical ballooning.

A 70-year-old male with known hypertrophic cardiomyopathy (HCM) and latent obstruction presented with new onset of cardiogenic shock. He had a new resting left ventricular (LV) outflow gradient of 90 mmHg, and new severe LV systolic dysfunction. Because of rapid deterioration despite medical management he was urgently sent for surgical relief of obstruction, which immediately reversed both the LV dysfunction and shock. A second patient, a 58-year-old male also with hypertrophic cardiomyopathy and latent obstruction presented with collapse, cardiogenic shock, 135 mmHg resting LV outflow gradient and new severe LV systolic dysfunction. His profound shock was irreversible with pharmacologic management, but surgical relief of obstruction reversed both his LV dysfunction and shock. Echocardiography plays a pivotal role in the management of these acutely ill patients. (Echocardiography 2011;28:E174-E179)

Reversal of acute systolic dysfunction and cardiogenic shock in hypertrophic cardiomyopathy by surgical relief of obstruction.

Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disorder characterised by abnormal left ventricular (LV) hypertrophy in the absence of severe aortic stenosis and hypertension. The presence of a dynamic gradient in the left ventricular outflow tract (LVOT) above 30 mm Hg at rest is associated with an increased mortality.1 Surgical myectomy or transcoronary ablation of septal hypertrophy (TASH) is indicated in patients with persistent symptoms and gradients > 50 mm Hg under resting or provoked conditions, despite medical treatment.2 We recently described a characteristic mid systolic septal deceleration (MSSD) pattern in the velocity trace of the basal septum obtained by tissue Doppler imaging (TDI),3 but the diagnostic value of the MSSD pattern in a larger patient population is still unknown.

Twenty six HCM patients (15 male, mean (SD) age 48 (17) years, septal wall thickness > 15 mm) were studied by transthoracic resting echocardiography (GE-Vingmed Vivid 7 Pro, Horten, Norway). In addition to the standard measurements, we analysed longitudinal septal motion by colour coded TDI from the apical view (frame rate 192 (40)/s). All data were stored digitally and analysed off line (EchoPac, GE-Vingmed, Horten, Norway). The MSSD pattern was defined by the presence of a biphasic systolic velocity curve with an early systolic …

https://heart.bmj.com/content/heartjnl/91/3/379.full.pdf

Mid systolic septal deceleration in hypertrophic cardiomyopathy: clinical value and insights into the pathophysiology of outflow tract obstruction by tissue Doppler echocardiography

Hypertrophic obstructive cardiomyopathy (HOCM) is
characterized by the presence of a dynamic left ventricular
outflow tract gradient. We studied septal longitudinal motion by
colorcoded tissue Dopppler echocardiography in a 69-year old
HOCM patient during and after catheterbased treatment by
transcoronary alcohol ablation of septal hypertrophy. The
presence of dynamic left ventricular outflow tract obstruction
was associated with a characteristic abrupt mid-systolic septal
deceleration pattern in the tissue Doppler velocity trace. Five
weeks after treatment, this pattern was no longer visible at
rest.

Mid-systolic septal deceleration. A new sign of left ventricular outflow tract obstruction obtained by color-coded tissue Doppler echocardiography.

by tissue Doppler echocardiography the pathophysiology of outflow tract obstruction cardiomyopathy: clinical value and insights into Mid systolic septal deceleration in hypertrophic

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Mid systolic septal deceleration in hypertrophic cardiomyopathy: clinical value and insights into the pathophysiology of outflow tract obstruction by tissue Doppler echocardiography

Mid-systolic septal deceleration. A new sign of left ventricular outflow tract obstruction obtained by color-coded tissue Doppler echocardiography.

by tissue Doppler echocardiography the pathophysiology of outflow tract obstruction cardiomyopathy: clinical value and insights into Mid systolic septal deceleration in hypertrophic