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Diagnosis and Evaluation of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review

Maron, Barry J; Desai, Milind Y; Nishimura, Rick A; Spirito, Paolo; Rakowski, Harry; Towbin, Jeffrey A; Rowin, Ethan J; Maron, Martin S; Sherrid, Mark V
Hypertrophic cardiomyopathy (HCM) is a relatively common often inherited global heart disease, with complex phenotypic and genetic expression and natural history, affecting both genders and many races and cultures. Prevalence is 1:200-1:500, largely based on the disease phenotype with imaging, inferring that 750,000 Americans may be affected by HCM. However, cross-sectional data show that only a fraction are clinically diagnosed, suggesting under-recognition, with most clinicians exposed to small segments of the broad disease spectrum. Highly effective HCM management strategies have emerged, altering clinical course and substantially lowering mortality and morbidity rates. These advances underscore the importance of reliable HCM diagnosis with echocardiography and cardiac magnetic resonance. Family screening with noninvasive imaging will identify relatives with the HCM phenotype, while genetic analysis recognizes preclinical sarcomere gene carriers without left ventricular hypertrophy, but with the potential to transmit disease. Comprehensive initial patient evaluations are important for reliable diagnosis, accurate portrayal of HCM and family history, risk stratification, and distinguishing obstructive versus nonobstructive forms.
PMID: 35086660
ISSN: 1558-3597
CID: 5147572

Management of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review

Maron, Barry J; Desai, Milind Y; Nishimura, Rick A; Spirito, Paolo; Rakowski, Harry; Towbin, Jeffrey A; Dearani, Joseph A; Rowin, Ethan J; Maron, Martin S; Sherrid, Mark V
Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited primary cardiac disease, has now transformed into a contemporary highly treatable condition with effective options that alter natural history along specific personalized adverse pathways at all ages. HCM patients with disease-related complications benefit from: matured risk stratification in which major markers reliably select patients for prophylactic defibrillators and prevention of arrhythmic sudden death; low risk to high benefit surgical myectomy (with percutaneous alcohol ablation a selective alternative) that reverses progressive heart failure caused by outflow obstruction; anticoagulation prophylaxis that prevents atrial fibrillation-related embolic stroke and ablation techniques that decrease the frequency of paroxysmal episodes; and occasionally, heart transplant for end-stage nonobstructive patients. Those innovations have substantially improved outcomes by significantly reducing morbidity and HCM-related mortality to 0.5%/y. Palliative pharmacological strategies with currently available negative inotropic drugs can control symptoms over the short-term in some patients, but generally do not alter long-term clinical course. Notably, a substantial proportion of HCM patients (largely those identified without outflow obstruction) experience a stable/benign course without major interventions. The expert panel has critically appraised all available data and presented management insights and recommendations with concise principles for clinical decision-making.
PMID: 35086661
ISSN: 1558-3597
CID: 5153022

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

COVID-19 in Adults With Hypertrophic Cardiomyopathy

Arabadjian, Milla E; Reuter, Maria C; Stepanovic, Alexandra; Sherrid, Mark V; Massera, Daniele
PMID: 34859067
ISSN: 2297-055x
CID: 5069242

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

Arabadjian, Milla; Serrato, Stephanie; Sherrid, Mark V
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