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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

Mitral Leaflet Shortening as an Ancillary Procedure in Obstructive Hypertrophic Cardiomyopathy

Swistel, Daniel G; Massera, Daniele; Stepanovic, Alexandra; Adlestein, Elizabeth; Reuter, Maria; Wu, Woon; Scheinerman, Joshua A; Nampi, Robert; Paone, Darien; Kim, Bette; Sherrid, Mark V
BACKGROUND:Mitral leaflet elongation is common in hypertrophic cardiomyopathy (HCM), contributes to obstructive physiology, and presents a challenge to dual surgical goals of abolition of outflow gradients and mitral regurgitation. Anterior leaflet shortening, performed as an ancillary surgical procedure during myectomy, is controversial. METHODS:This was a retrospective study of all patients undergoing myectomy from 1/2010 to 3/2020 analyzing survival and echocardiographic results. We compared outcomes of patients treated with myectomy and concomitant mitral leaflet shortening with patients treated with myectomy alone. Over this time technique for mitral shortening evolved from anterior leaflet plication to residual leaflet excision (ReLex). RESULTS:Myectomy was performed on 416 patients age 57.5±13.6 years, 204 (49%) female. Average follow up was 5.4±2.8 years. Survival follow-up was complete in 415. Myectomy without valve replacement was performed in 332 patients, of whom 192 had mitral valve shortening (58%). Mitral leaflet plication was performed in 73, ReLex in 151 and both in 32. Hospital mortality for patients undergoing myectomy was 0.7%. At 8 years, cumulative survival was 95% for both myectomy plus leaflet shortening and myectomy alone groups, with no difference in survival between the two. There was no difference in survival between anterior leaflet plication and ReLex groups. Echocardiography 2.5 years after surgery showed a decrease in resting and provoked gradients, mitral regurgitation and left atrial volume and no difference in key variables between ancillary leaflet shortening and myectomy alone patients. CONCLUSIONS:These results affirm that mitral shortening may be an appropriate surgical judgment for selected patients.
PMID: 38518836
ISSN: 1552-6259
CID: 5640912

Efficacy and Safety of aficamten in Symptomatic Non-Obstructive Hypertrophic Cardiomyopathy: Results From the REDWOOD-HCM Trial, Cohort 4

Masri, Ahmad; Sherrid, Mark V; Abraham, Theodore P; Choudhury, Lubna; Garcia-Pavia, Pablo; Kramer, Christopher M; Barriales-Villa, Roberto; Owens, Anjali T; Rader, Florian; Nagueh, Sherif F; Olivotto, Iacopo; Saberi, Sara; Tower-Rader, Albree; Wong, Timothy C; Coats, Caroline J; Watkins, Hugh; Fifer, Michael A; Solomon, Scott D; Heitner, Stephen B; Jacoby, Daniel L; Kupfer, Stuart; Malik, Fady I; Meng, Lisa; Sohn, Regina L; Wohltman, Amy; Maron, Martin S; ,
BACKGROUND:This open-label phase 2 trial evaluated the safety and efficacy of aficamten in patients with non-obstructive hypertrophic cardiomyopathy (nHCM). METHODS:Patients with symptomatic nHCM (left ventricular outflow tract obstruction gradient ≤30 mmHg, left ventricular ejection fraction [LVEF] ≥60%, N-terminal pro-B-type natriuretic peptide [NT-proBNP] >300 pg/mL) received aficamten 5-20 mg once daily (doses adjusted according to echocardiographic LVEF) for 10 weeks. RESULTS:41 patients were enrolled (mean ± SD age 56 ± 16 years; 59% female). At Week 10, 22 (55%) patients experienced an improvement of ≥1 New York Heart Association class; 11 (29%) became asymptomatic. Clinically relevant improvements in Kansas City Cardiomyopathy Questionnaire Clinical Summary Scores occurred in 22 (55%) patients. Symptom relief was paralleled by reductions in NT-proBNP (56%; P < 0.001) and high-sensitivity cardiac troponin I (22%; P < 0.005). Modest reductions in LVEF (mean ± SD) of -5.4% ± 10 to 64.6% ± 9.1 were observed. Three (8%) patients had asymptomatic reduction in LVEF <50% (range: 41-48%), all returning to normal after 2 weeks of washout. One patient with prior history of aborted sudden cardiac death experienced a fatal arrhythmia during the study. CONCLUSIONS:Aficamten administration for symptomatic nHCM was generally safe and associated with improvements in heart failure symptoms and cardiac biomarkers. TRIAL REGISTRATION/ Identifier: NCT04219826 LAY SUMMARY: Non-obstructive hypertrophic cardiomyopathy (nHCM) is a disease in which the heart muscle becomes abnormally thickened. There are no proven medical therapies. Aficamten is a new cardiac myosin inhibitor designed to target the underlying cause of HCM. REDWOOD-HCM Cohort 4 was the first study exploring the efficacy and safety of aficamten in people with symptoms from nHCM. Most patients reported improved health and functional status. There was also significant decrease in blood levels of biomarkers indicating excessive pressure within the heart and damage to heart muscle cells. These results support a larger placebo-controlled study of aficamten for people with nHCM (ACACIA-HCM).
PMID: 38493832
ISSN: 1532-8414
CID: 5639922

Clinical course of adults with co-occurring hypertrophic cardiomyopathy and hypertension: A scoping review

Arabadjian, Milla; Montgomery, Sophie; Pleasure, Mitchell; Nicolas, Barnaby; Collins, Maxine; Reuter, Maria; Massera, Daniele; Shimbo, Daichi; Sherrid, Mark V
INTRODUCTION/UNASSIGNED:Hypertension affects approximately 50 % of patients with hypertrophic cardiomyopathy (HCM) but clinical course in adults with co-occurring HCM and hypertension is underexplored. Management may be challenging as routine anti-hypertensive medications may worsen obstructive HCM, the most common HCM phenotype. In this scoping review, we sought to synthesize the available literature related to clinical course and outcomes in adults with both conditions and to highlight knowledge gaps to inform future research directions. METHODS/UNASSIGNED:We searched 5 electronic databases (PubMed, CINAHL, Scopus, Embase, Web of Science) to identify peer-reviewed articles, 2011-2023. We followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses-Scoping Review (PRISMA-ScR) guideline. RESULTS/UNASSIGNED:Eleven articles met eligibility. Adults with both conditions were older and had higher rates of obesity and diabetes than adults with HCM alone. Results related to functional class and arrhythmia were equivocal in cross-sectional studies. Only 1 article investigated changes in medical therapy among adults with both conditions. Hypertension was a predictor of worse functional class, but was not associated with all-cause mortality, heart failure-related mortality, or sudden-death. No data was found that related to common hypertension-related outcomes, including renal disease progression, nor patient-reported outcomes, including quality of life. CONCLUSIONS/UNASSIGNED:Our results highlight areas for future research to improve understanding of co-occurring HCM and hypertension. These include a need for tailored approaches to medical management to optimize outcomes, evaluation of symptom burden and quality of life, and investigation of hypertension-related outcomes, like renal disease and ischemic stroke, to inform cardiovascular risk mitigation strategies.
PMID: 38510995
ISSN: 2666-6022
CID: 5640672

Obstructive Hypertrophic Cardiomyopathy and Takotsubo Syndrome: How to Deal With Left Ventricular Ballooning?

Citro, Rodolfo; Bellino, Michele; Merli, Elisa; Di Vece, Davide; Sherrid, Mark V
Currently, there are 2 proposed causes of acute left ventricular ballooning. The first is the most cited hypothesis that ballooning is caused by direct catecholamine toxicity on cardiomyocytes or by microvascular ischemia. We refer to this pathogenesis as Takotsubo syndrome. More recently, a second cause has emerged: that in some patients with underlying hypertrophic cardiomyopathy, left ventricular ballooning is caused by the sudden onset of latent left ventricular outflow tract obstruction. When it becomes severe and unrelenting, severe afterload mismatch and acute supply-demand ischemia appear and result in ballooning. In the context of 2 causes, presentations might overlap and cause confusion. Knowing the pathophysiology of each mechanism and how to determine a correct diagnosis might guide treatment.
PMID: 37889174
ISSN: 2047-9980
CID: 5609592

Genotype Analysis as a Clinical Tool for Families in Hypertrophic Cardiomyopathy

Sherrid, Mark V.; Massera, Daniele
ISSN: 2772-963x
CID: 5621482

What Predicts the Success of Alcohol Septal Ablation?: The Myocardium Counts, After All [Comment]

Massera, Daniele; Sherrid, Mark V
PMID: 37582171
ISSN: 1941-7632
CID: 5595582

Clinical course and outcomes in adults with co-occurring hypertrophic cardiomyopathy and hypertension: a scoping review protocol

Arabadjian, Milla; Nicolas, Barnaby; Montgomery, Sophie; Pleasure, Mitchell; Collins, Maxine; Reuter, Maria; Massera, Daniele; Shimbo, Daichi; Sherrid, Mark
INTRODUCTION:Hypertension affects 40%-60% of adults with hypertrophic cardiomyopathy (HCM), the most common inherited cardiac condition. It can be a diagnostic confounder for HCM, contributing to delayed diagnosis. Clinically, treatment of co-occurring hypertension and HCM poses challenges as first-line and second-line antihypertensive medications are often contraindicated in HCM. The clinical course in adults with hypertension and HCM is also not well understood, and studies examining patient outcomes in this population are equivocal. In this paper, we aim to outline the protocol of a scoping review, a type of literature review, to systematically synthesise existing knowledge on adults with co-occurring HCM and hypertension, highlighting knowledge and evidence gaps, and identifying future research directions to optimise outcomes in this population. METHODS AND ANALYSIS:This review is guided by Arksey and O'Malley's conceptual framework on conducting scoping reviews. We will search five electronic databases (PubMed, CINAHL, Scopus, Embase and Web of Science) and reference lists of publications to identify eligible articles focusing on medical therapy, clinical course or outcomes in adults with HCM and hypertension, between 2011 and 2023. Our search strategy and presentation of results will be guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses-Scoping Review guideline. First, two independent reviewers will screen articles, by title and abstract, followed by a full-text screen to identify eligible articles. Relevant data will be extracted and synthesised. ETHICS AND DISSEMINATION:Ethical approval is not required for this review as it is a secondary data collection of published articles and does not involve human subject participation. We will present results of this review at relevant professional conferences and patient-centred educational events. Results will be published in a peer-reviewed journal. TRIAL REGISTRATION NUMBER:
PMID: 37463810
ISSN: 2044-6055
CID: 5535662

How common is hypertrophic cardiomyopathy… really?: Disease prevalence revisited 27 years after CARDIA

Massera, Daniele; Sherrid, Mark V; Maron, Martin S; Rowin, Ethan J; Maron, Barry J
Hypertrophic cardiomyopathy (HCM) is a heterogeneous albeit treatable cardiac disease of variable severity, with the potential for heart failure, atrial fibrillation and arrhythmic sudden death, characterized by otherwise unexplained left ventricular (LV) hypertrophy and affecting all ages and races. Over the last 30 years, several studies have estimated the prevalence of HCM in the general population, employing echocardiography and cardiac magnetic resonance imaging (CMR), as well electronic health records and billing databases for clinical diagnosis. The estimated prevalence in the general population based on the disease phenotype of LV hypertrophy by imaging is 1:500 (0.2%). This prevalence was initially proposed in 1995 in the population-based CARDIA study employing echocardiography, and more recently confirmed by automated CMR analysis in the large UK Biobank cohort. The 1:500 prevalence appears most relevant to clinical assessment and management of HCM. These available data suggest that HCM is not a rare condition but likely underdiagnosed clinically and by extrapolation potentially affects about 700,000 Americans and possibly 15 million people worldwide.
PMID: 37028711
ISSN: 1874-1754
CID: 5463952