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Thin-filament mutations, hypertrophic cardiomyopathy, and risk [Comment]

Sherrid, Mark V; Arabadjian, Milla; Koulova, Anna
PMID: 25524338
ISSN: 1558-3597
CID: 1570852

Vector flow mapping in obstructive hypertrophic cardiomyopathy to assess the relationship of early systolic left ventricular flow and the mitral valve

Ro, Richard; Halpern, Dan; Sahn, David J; Homel, Peter; Arabadjian, Milla; Lopresto, Charles; Sherrid, Mark V
BACKGROUND: The hydrodynamic cause of systolic anterior motion of the mitral valve (SAM) is unresolved. OBJECTIVES: This study hypothesized that echocardiographic vector flow mapping, a new echocardiographic technique, would provide insights into the cause of early SAM in obstructive hypertrophic cardiomyopathy (HCM). METHODS: We analyzed the spatial relationship of left ventricular (LV) flow and the mitral valve leaflets (MVL) on 3-chamber vector flow mapping frames, and performed mitral valve measurements on 2-dimensional frames in patients with obstructive and nonobstructive HCM and in normal patients. RESULTS: We compared 82 patients (22 obstructive HCM, 23 nonobstructive HCM, and 37 normal) by measuring 164 LV pre- and post-SAM velocity vector flow maps, 82 maximum isovolumic vortices, and 328 2-dimensional frames. We observed color flow and velocity vector flow posterior to the MVL impacting them in the early systolic frames of 95% of obstructive HCM, 22% of nonobstructive HCM, and 11% of normal patients (p < 0.001). In both pre- and post-SAM frames, we measured a high angle of attack >60 degrees of local vector flow onto the posterior surface of the leaflets whether the flow was ejection (59%) or the early systolic isovolumic vortex (41%). Ricochet of vector flow, rebounding off the leaflet into the cul-de-sac, was noted in 82% of the obstructed HCM, 9% of nonobstructive HCM, and none (0%) of the control patients (p < 0.001). Flow velocities in the LV outflow tract on the pre-SAM frame 1 and 2 mm from the tip of the anterior leaflet were low: 39 and 43 cm/s, respectively. CONCLUSIONS: Early systolic flow impacts the posterior surfaces of protruding MVL initiating SAM in obstructive HCM.
PMID: 25440093
ISSN: 1558-3597
CID: 1570762

Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy

Sherrid, Mark V; Swistel, Daniel G; Olivotto, Iacopo; Pieroni, Maurizio; Wever-Pinzon, Omar; Riedy, Katherine; Bach, Richard G; Husaini, Mustafa; Cresci, Sharon; Reyentovich, Alex; Massera, Daniele; Maron, Martin S; Maron, Barry J; Kim, Bette
Background Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic features of mild septal thickening, outflow gradients, and distinctive mitral abnormalities differentiate these patients from others with Takotsubo syndrome, who have normal mitral valves and no outflow obstruction. Methods and Results We analyzed 8 patients from our 4 HCM centers with obstructive HCM and abrupt presentation of cardiogenic shock with LV ballooning, and 6 cases reported in literature. Of 14 patients, 10 (71%) were women, aged 66±9 years, presenting with acute symptoms: LV ballooning; depressed ejection fraction (25±5%); refractory systemic hypotension; marked LV outflow tract obstruction (peak gradient, 94±28 mm Hg); and elevated troponin, but absence of atherosclerotic coronary disease. Shock was managed with intravenous administration of phenylephrine (n=6), norepinephrine (n=6), β-blocker (n=7), and vasopressin (n=1). Mechanical circulatory support was required in 8, including intra-aortic balloon pump (n=4), venoarterial extracorporeal membrane oxygenation (n=3), and Impella and Tandem Heart in 1 each. In refractory shock, urgent relief of obstruction by myectomy was performed in 5, and alcohol ablation in 1. All patients survived their critical illness, with full recovery of systolic function. Conclusions When cardiogenic shock and LV ballooning occur in obstructive HCM, they are marked by distinctive anatomic and physiologic features. Relief of obstruction with targeted pharmacotherapy, mechanical circulatory support, and myectomy, when necessary for refractory shock, may lead to survival and normalization of systolic function.
PMID: 34634917
ISSN: 2047-9980
CID: 5061922

Disease Expression and Outcomes in Black and White Adults With Hypertrophic Cardiomyopathy

Arabadjian, Milla E; Yu, Gary; Sherrid, Mark V; Dickson, Victoria Vaughan
Background There is limited research on hypertrophic cardiomyopathy (HCM), which is the most common inherited cardiac disorder, in diverse populations, including Black individuals. Current literature lacks comprehensive data on HCM disease expression, comorbidities, and outcomes in this historically disadvantaged group. The purpose of this study was to examine structural HCM characteristics, comorbidities, and outcomes in a Black and White cohort with HCM. Methods and Results The study was a subgroup analysis from a longitudinal, prospective study on HCM, with supplemental chart review. The sample included adults (≥18 years) with a clinical diagnosis of HCM, who self-identified as Black/African American or White. The study sample comprised 434 individuals; 57 (13.1%) were Black, and 180 (41.5%) were women. Black patients were younger than White patients, 54.6 (13.4) versus 62.5 (14.8) years, P=0.001. Black patients were more likely to have sub-basal and diffuse hypertrophy, 22 (38.6%) versus 56 (14.9%), P<0.001, 6 (10.5%) versus 15 (4%), P=0.017, mid-LV obstruction, 7 (12.3%) versus 21 (5.5%), P=0.025, and cardiac fibrosis ≥15%, 10 (22.2%) versus 19 (8.8%), P=0.009, than White patients. Black patients were more likely to experience appropriate implantable cardioverter defibrillator interventions, 5 (38.5) versus 5 (6.8), P<0.001 and were more likely to have ≥2 sudden death risk factors. Comorbidities were largely similar between groups, though more Black participants had Class II obesity, 12 (21.8) versus 30 (8.1), P<0.001. Both groups had similar rates of genetic testing usage. Conclusions This study underscores the need for continued research of HCM in Black populations, including tailored approaches to diagnosis and precise evaluation of cardiac anatomy.
PMID: 34431363
ISSN: 2047-9980
CID: 5011112

Mavacamten Favorably Impacts Cardiac Structure in Obstructive Hypertrophic Cardiomyopathy: EXPLORER-HCM Cardiac Magnetic Resonance Substudy Analysis [Letter]

Saberi, Sara; Cardim, Nuno; Yamani, Mohamad; Schulz-Menger, Jeanette; Li, Wanying; Florea, Victoria; Sehnert, Amy J; Kwong, Raymond Y; Jerosch-Herold, Michael; Masri, Ahmad; Owens, Anjali; Lakdawala, Neal K; Kramer, Christopher M; Sherrid, Mark; Seidler, Tim; Wang, Andrew; Sedaghat-Hamedani, Farbod; Meder, Benjamin; Havakuk, Ofer; Jacoby, Daniel
PMID: 33190524
ISSN: 1524-4539
CID: 4875082

Three-Dimensional Imaging and Dynamic Modeling of Systolic Anterior Motion of the Mitral Valve

Vainrib, Alan; Massera, Daniele; Sherrid, Mark V; Swistel, Daniel G; Bamira, Daniel; Ibrahim, Homam; Staniloae, Cezar; Williams, Mathew R; Saric, Muhamed
Left ventricular outflow tract (LVOT) obstruction in hypertrophic cardiomyopathy (HCM) is often caused by systolic anterior motion (SAM) of the mitral valve caused by the interplay between increased left ventricular (LV) wall thickness and an abnormal mitral valve anatomy and geometry. Three-dimensional (3D) echocardiographic imaging of the mitral valve has revolutionized the practice of cardiology, paving the way for new methods to see and treat valvular heart disease. Here we present the novel and incremental value of 3D transesophageal echocardiography (TEE) of SAM visualization. This review first provides step-by-step instructions on acquiring and optimizing 3D TEE imaging of SAM. It then describes the unique and novel findings using standard 3D TEE rendering as well as dynamic mitral valve modeling of SAM from 3D data sets, which can provide a more detailed visualization of SAM features. The findings include double-orifice LVOT caused by the residual leaflet, the dolphin smile phenomenon, and delineation of SAM width. Finally, the review discusses the essential role of 3D TEE imaging for preprocedural assessment and intraprocedural guidance of surgical and novel percutaneous treatments of SAM.
PMID: 33059963
ISSN: 1097-6795
CID: 4641632

Availability and Utilization of Automated External Defibrillators in New York State Schools

Arabadjian, Milla; Serrato, Stephanie; Sherrid, Mark V
PMCID:8514828
PMID: 34660480
ISSN: 2296-2360
CID: 5037232

Indications for Surgery in Obstructive Hypertrophic Cardiomyopathy [Editorial]

Sherrid, Mark V
PMID: 33342223
ISSN: 2047-9980
CID: 4726052

The Mitral Valve in Hypertrophic Cardiomyopathy: Other Side of the Outflow Tract [Editorial]

Sherrid, Mark V; Adams, David H
PMID: 33153585
ISSN: 1558-3597
CID: 4664372

Analysis of three-chamber view conventional and tagged cine MRI in patients with suspected hypertrophic cardiomyopathy

Chitiboi, Teodora; Kanski, Mikael; Tautz, Lennart; Hennemuth, Anja; Halpern, Dan; Sherrid, Mark; Axel, Leon
OBJECTIVES/OBJECTIVE:To investigate the potential value of adding a tagged three-chamber (3Ch) cine to clinical hypertrophic cardiomyopathy (HCM) magnetic resonance imaging (MRI) protocols, including to help distinguish HCM patients with regionally impaired cardiac function. METHODS:Forty-eight HCM patients, five patients with "septal knuckle" (SK), and 20 healthy volunteers underwent MRI at 1.5T; a tagged 3Ch cine was added to the protocol. Regional strain, myocardial wall thickness, and mitral valve leaflet lengths were measured in the 3Ch view. RESULTS:In HCM, we found a reduced tangential strain with decreased diastolic relaxation in both hypertrophied (p = 0.003) and remote segments (p = 0.035). Strain in the basal septum correlated with the length of the coaptation zone + residual leaflet (r = 0.48, p < 0.001). In the basal free wall, patients with SK had faster relaxation compared to HCM patients with septal hypertrophy. DISCUSSION/CONCLUSIONS:The 3Ch tagged MRI sequence provides useful information for the examination of suspected HCM patients, with minimal additional time cost. Local wall function is closely associated with morphological changes of the mitral apparatus measured in the same plane and may provide insights into mechanisms of obstruction. The additional strain information may be helpful when analyzing local myocardial wall motion patterns in the presence of SK.
PMID: 32152793
ISSN: 1352-8661
CID: 4348832