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Unmasking Obstruction in Hypertrophic Cardiomyopathy With Postprandial Resting and Treadmill Stress Echocardiography
Massera, Daniele; Long, Clarine; Xia, Yuhe; James, Les; Adlestein, Elizabeth; Alvarez, Isabel C; Wu, Woon Y; Reuter, Maria C; Arabadjian, Milla; Grossi, Eugene A; Saric, Muhamed; Sherrid, Mark V
BACKGROUND:Latent left ventricular outflow tract obstruction (LVOTO) is an important cause of symptoms in patients with hypertrophic cardiomyopathy (HCM) but can be challenging to provoke. OBJECTIVES AND METHODS/OBJECTIVE:To examine the value of postprandial resting and stress echocardiography and utilization of invasive or enhanced drug therapies (surgical myectomy, alcohol septal ablation, disopyramide, and mavacamten) in patients with postprandial LVOTO. Consecutive HCM patients without LVOTO underwent routine and postprandial echocardiography at rest, with provocation (Valsalva and standing) and after symptom-limited treadmill stress. RESULTS:Among 252 patients (mean age, 58 years, 39% women), postprandial LVOT gradients were higher compared with routine echocardiography at rest (median, 9.0 [0-38.0] vs 0 [0-14.0] mm Hg; P < .0001) and with provocation (18.5 [0-70.3] vs 1.5 [0-41.0] mm Hg; P < .0001). Postprandial exercise stress echocardiogram (PPXSE) gradients were higher in a subset of 44 patients who underwent both postprandial and fasting stress echocardiography (47.0 [5.3-81.0] vs 17.5 [0-46.0] mm Hg; P < .0001). In total, 49 (19.5%) patients achieved the ≥50 mm Hg threshold under routine conditions (rest/provocation); 90 (35.7%) additional patients achieved postprandial gradients ≥50 mm Hg (rest/provocation/exercise), 38 (15.1%) with PPXSE alone. A total of 71 patients were treated with 91 invasive or enhanced drug therapies, 32 (45.1%) of whom had gradients ≥50 mm Hg only after eating (rest/provocation) and 8 (11.3%) only with PPXSE, with symptom relief in the majority. CONCLUSIONS:Postprandial echocardiography was useful at unmasking LVOTO in more than one-third of patients who did not have high gradients otherwise. Eating before echocardiography is a powerful provocative tool in the evaluation of patients with HCM.
PMID: 38950755
ISSN: 1097-6795
CID: 5685002
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
Safety and Efficacy of Metabolic Modulation With Ninerafaxstat in Patients With Nonobstructive Hypertrophic Cardiomyopathy
Maron, Martin S; Mahmod, Masliza; Abd Samat, Azlan Helmy; Choudhury, Lubna; Massera, Daniele; Phelan, Dermot M J; Cresci, Sharon; Martinez, Matthew W; Masri, Ahmad; Abraham, Theodore P; Adler, Eric; Wever-Pinzon, Omar; Nagueh, Sherif F; Lewis, Gregory D; Chamberlin, Paul; Patel, Jai; Yavari, Arash; Dehbi, Hakim-Moulay; Sarwar, Rizwan; Raman, Betty; ValkoviÄ, Ladislav; Neubauer, Stefan; Udelson, James E; Watkins, Hugh
BACKGROUND:In nonobstructive hypertrophic cardiomyopathy (nHCM), there are no approved medical therapies. Impaired myocardial energetics is a potential cause of symptoms and exercise limitation. Ninerafaxstat, a novel cardiac mitotrope, enhances cardiac energetics. OBJECTIVES/OBJECTIVE:This study sought to evaluate the safety and efficacy of ninerafaxstat in nHCM. METHODS:Patients with hypertrophic cardiomyopathy and left ventricular outflow tract gradient <30 mm Hg, ejection fraction ≥50%, and peak oxygen consumption <80% predicted were randomized to ninerafaxstat 200 mg twice daily or placebo (1:1) for 12 weeks. The primary endpoint was safety and tolerability, with efficacy outcomes also assessed as secondary endpoints. RESULTS:(P = 0.90). The Kansas City Cardiomyopathy Questionnaire Clinical Summary Score was directionally, though not significantly, improved with ninerafaxstat vs placebo (LS mean 3.2; 95% CI: -2.9 to 9.2; P = 0.30); however, it was statistically significant when analyzed post hoc in the 35 patients with baseline Kansas City Cardiomyopathy Questionnaire Clinical Summary Score ≤80 (LS mean 9.4; 95% CI: 0.3-18.5; P = 0.04). CONCLUSIONS:In symptomatic nHCM, novel drug therapy targeting myocardial energetics was safe and well tolerated and associated with better exercise performance and health status among those most symptomatically limited. The findings support assessing ninerafaxstat in a phase 3 study.
PMID: 38599256
ISSN: 1558-3597
CID: 5653952
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: 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: 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: 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: 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.
SCOPUS:85184564037
ISSN: 2666-6022
CID: 5700022
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.
PMCID:10945972
PMID: 38510995
ISSN: 2666-6022
CID: 5640672
Traditional and novel risk factors for incident aortic stenosis in community-dwelling older adults
Massera, Daniele; Bartz, Traci M; Biggs, Mary L; Sotoodehnia, Nona; Reiner, Alexander P; Semba, Richard D; Gottdiener, John S; Psaty, Bruce M; Owens, David S; Kizer, Jorge R
OBJECTIVES/OBJECTIVE:Calcific aortic stenosis (AS) is the most common valvular disease in older adults, yet its risk factors remain insufficiently studied in this population. Such studies are necessary to enhance understanding of mechanisms, disease management and therapeutics. METHODS:The Cardiovascular Health Study is a population-based investigation of older adults that completed adjudication of incident AS over long-term follow-up. We evaluated traditional cardiovascular risk factors or disease, as well as novel risk factors from lipid, inflammatory and mineral metabolism pathways, in relation to incident moderate or severe AS (including AS procedures) and clinically significant AS (severe AS, including procedures). RESULTS:) showed a U-shaped relationship. Findings were similar for clinically significant AS, although CHD and sCD14 fell short of significance, but interleukin-(IL) 6 showed a positive association. CONCLUSION/CONCLUSIONS:. These factors may hold clues to biology, preventive efforts and potential therapeutics for those at highest risk.
PMID: 37463733
ISSN: 1468-201x
CID: 5535652
Genotype Analysis as a Clinical Tool for Families in Hypertrophic Cardiomyopathy
Sherrid, Mark V.; Massera, Daniele
SCOPUS:85180257056
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:https://osf.io/cy8qb/?view_only=98197f4850584e51807ff9b62533a706.
PMCID:10357759
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