Publications by authors named "Mark V Sherrid"
Background: Disopyramide is used to treat heart failure symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM) with known medium-term efficacy and safety, while long-term outcomes are unknown.
Methods And Results: A total of 92 consecutive patients with symptomatic obstructive HCM with peak left ventricular outflow tract gradients of ≥30 mm Hg at rest or with provocation who were maintained on disopyramide for ≥5 years at 2 dedicated HCM centers were included: 92 patients; mean age, 62.5 years; 54% women; treated with disopyramide for median 7.
Background: Standard-of-care (SoC) medications for the treatment of obstructive hypertrophic cardiomyopathy (oHCM) are recommended as first-line therapy despite the lack of evidence from controlled clinical trials and well known off-target side effects.
Objectives: We describe the impact of SoC therapy downtitration and withdrawal in patients already receiving aficamten in FOREST-HCM (Follow-Up, Open-Label, Research Evaluation of Sustained Treatment with Aficamten in Hypertrophic Cardiomyopathy; NCT04848506).
Methods: Patients receiving SoC therapy (beta-blocker, nondihydropyridine calcium-channel blocker, and/or disopyramide) were eligible for protocol-guided SoC downtitration and withdrawal at the discretion of the investigator and after achieving a stable dose of aficamten for ≥4 weeks.
Article Synopsis
- Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, but its signaling pathways that lead to severe forms are not well understood.
- This study aimed to uncover these pathways using proteomic profiling of heart tissue in HCM patients and controls, analyzing over 7,200 proteins.
- Results showed dysregulation in several key pathways related to heart function and disease progression, providing a comprehensive analysis of HCM at a molecular level. *
Article Synopsis
- In patients with hypertrophic cardiomyopathy (HCM), the study aimed to evaluate the relationship between apical aneurysm size and common risk factors with the occurrence of deadly arrhythmias and unexplained strokes.
- Among 108 patients studied, 19.4% experienced events like sudden cardiac death or serious arrhythmias, with a significant risk linked to larger aneurysm sizes and the presence of major HCM risk factors.
- Atrial fibrillation was common, and while stroke incidence was low without known causes, overall findings suggest that smaller aneurysms (less than 2 cm) pose a lower risk for severe events over five years.
Article Synopsis
- Latent left ventricular outflow tract obstruction (LVOTO) is a significant issue for patients with hypertrophic cardiomyopathy (HCM), making it essential to find effective ways to diagnose it.
- A study involving 252 HCM patients showed that postprandial echocardiography (after eating) revealed significantly higher LVOT gradients compared to routine assessments, indicating that eating can provoke these obstructions.
- The findings suggest that using echocardiography after meals can help identify LVOTO in many patients and lead to effective treatment options, with symptom relief observed in a large percentage of those treated.
On the Cause of Systolic Anterior Motion in Obstructive Hypertrophic Cardiomyopathy.
J Am Soc Echocardiogr
August 2024
Article Synopsis
- Mitral leaflet elongation in hypertrophic cardiomyopathy (HCM) can lead to obstruction and mitral regurgitation, complicating surgical efforts to address these issues.
- A study comparing outcomes for patients who had myectomy with and without mitral leaflet shortening found no significant difference in survival or echocardiographic results between the two groups.
- The findings support the idea that mitral leaflet shortening may be a viable surgical option for certain patients undergoing myectomy.
Article Synopsis
- Hypertension frequently co-occurs with hypertrophic cardiomyopathy (HCM) in about 50% of patients, yet its impact on clinical outcomes remains understudied in adults, complicating management due to potential negative effects of standard hypertension treatments on obstructive HCM.
- A thorough review of literature published between 2011 and 2023 identified 11 articles, revealing that adults with both conditions tend to be older and have higher obesity and diabetes rates, while the relationship between their functional class and arrhythmias was unclear.
- The study emphasizes the need for better-targeted management strategies, more research on quality of life, and an exploration of hypertension-related outcomes, such as renal disease and stroke, to enhance cardiovascular care
Article Synopsis
- This phase 2 trial assessed the safety and effectiveness of a medication called aficamten in patients suffering from nonobstructive hypertrophic cardiomyopathy (nHCM).
- 41 patients participated, and after 10 weeks of treatment, over half showed improvement in heart failure symptoms, with many reaching a better functional class.
- While some patients experienced a slight decrease in heart function (LVEF), the overall results indicated that aficamten is generally safe and effective in improving symptoms and relevant heart health markers.
Article Synopsis
- There are two main proposed causes of acute left ventricular ballooning: Takotsubo syndrome, which is linked to catecholamine toxicity and microvascular ischemia, and another related to hypertrophic cardiomyopathy with sudden outflow tract obstruction.
- Takotsubo syndrome is the more widely discussed cause, whereas the obstruction in hypertrophic cardiomyopathy leads to severe afterload mismatch and ischemia.
- Understanding these distinct mechanisms is crucial for accurate diagnosis and effective treatment, as symptoms may overlap and lead to confusion.
Article Synopsis
- Mitral valve (MV) elongation is linked to obstructive hypertrophic cardiomyopathy (HCM) and this study aimed to analyze the histopathological characteristics of MV residual leaflets in patients with this condition.
- Researchers examined 22 MV residual leaflets from patients undergoing surgery and compared their structure and composition to 11 control leaflets from healthy individuals.
- Findings showed that MV residual leaflets in HCM had disorganized structures with abnormal features such as expanded spongiosa and fragmented elastic fibers, indicating significant alterations compared to normal valves.
Article Synopsis
- The review discusses acute left ventricular (LV) ballooning, focusing on two main causes: Takotsubo Syndrome (TTS) and severe left ventricular outflow tract (LVOT) obstruction in obstructive hypertrophic cardiomyopathy (HCM).
- It highlights the clinical signs and differing mechanisms behind each condition and emphasizes the role of echocardiography and cardiac catheterization in distinguishing between them.
- The authors aim to enhance diagnostic understanding and treatment strategies by clarifying the similarities and differences between these two syndromes.
Article Synopsis
- Hypertrophic cardiomyopathy (HCM) is a serious heart condition characterized by unexplained thickening of the left ventricle, and can lead to complications like heart failure and sudden death, affecting individuals across all ages and races.
- Current studies estimate the prevalence of HCM in the general population to be about 1 in 500, or 0.2%, based on imaging techniques like echocardiography and cardiac MRI.
- HCM appears to be underdiagnosed, with around 700,000 cases in the U.S. and potentially 15 million cases globally, indicating it's more common than previously thought.
Weight loss in hypertrophic cardiomyopathy: A clinical case series.
Int J Cardiol Cardiovasc Risk Prev
June 2023
Article Synopsis
- - Obesity in patients with hypertrophic cardiomyopathy (HCM) is linked to worse heart function and more severe symptoms, but weight loss can improve the condition.
- - A case study of six HCM patients showed that losing weight through diet, lifestyle changes, or surgery resulted in decreased left ventricular (LV) mass and wall thickness, as well as symptom improvement.
- - The results suggest that weight loss may positively remodel the heart in HCM patients, but further research is needed to fully understand the benefits.
Apical Aneurysms and Mid-Left Ventricular Obstruction in Hypertrophic Cardiomyopathy.
JACC Cardiovasc Imaging
May 2023
Article Synopsis
- Apical left ventricular (LV) aneurysms in patients with hypertrophic cardiomyopathy (HCM) are common and significantly linked to mid-LV obstruction, with a striking 95% of patients showing this condition in the study.
- The study compared echocardiograms and cardiac MRIs of 108 patients with HCM-related aneurysms against 110 controls (63 normal and 47 with mid-HCM without aneurysms) to assess the frequency and characteristics of mid-LV obstruction.
- Key findings included smaller systolic areas and larger papillary muscle areas in aneurysm patients, indicating altered heart function, with more than 80% showing an absence of blood flow during mid-systole; the results
Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on >50 years experience, surgery reliably reverses disabling heart failure by permanently abolishing mechanical outflow impedance and mitral regurgitation, with normalization of LV pressures and preserved systolic function. A consortium of 10 international currently active myectomy centers report about 11,000 operations, increasing significantly in number over the most recent 15 years.
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- - Hypertrophic cardiomyopathy (HCM) is a genetic heart condition that affects quality of life (QOL) and physical functioning, but these aspects are rarely studied in diverse populations.
- - A study compared QOL and physical functioning between Black and White adults with HCM, revealing that Black participants reported worse QOL scores, while both groups had similar exercise capacities overall.
- - Findings highlight the need for further research into the health and well-being of Black individuals with HCM, especially since female gender negatively impacted QOL and functioning across both groups.
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.
View Article and Find Full Text PDFArticle Synopsis
- - Hypertrophic cardiomyopathy (HCM) is a common, often inherited heart disease affecting an estimated 750,000 Americans, but many cases go undiagnosed due to the disease's complex nature and varied symptoms.
- - Effective management strategies for HCM have been developed, leading to improved patient outcomes and lower mortality, emphasizing the need for accurate diagnosis through imaging techniques like echocardiography and cardiac magnetic resonance.
- - Comprehensive evaluations of patients are essential for correct diagnosis and understanding of HCM, including family screenings and genetic testing to identify at-risk relatives and differentiate between obstructive and nonobstructive forms of the disease.