Patients with hypertrophic cardiomyopathy (HCM) may have delayed septal activation and left ventricular (LV) mechanical dyssynchrony, and may improve after alcohol septal ablation (ASA). This study used phase analysis of gated SPECT myocardial perfusion imaging (MPI) to evaluate septal activation and LV dyssynchrony in HCM patients pre- and post-ASA. Phase analysis was applied to 28 controls, and 32 HCM patients having rest MPI pre- and post-ASA to assess septal-lateral mechanical activation delay (SLD) and consequent LV dyssynchrony. In addition, phase analysis was applied to another group of 30 patients having serial MPI to measure variability of the LV dyssynchrony parameters on serial studies. ASA significantly reduced SLD and improved LV synchrony in the HCM patients with SLD < 0° due to earlier activation of the lateral wall relative to the septum. Based on the measured variability, 12 HCM patients had significant (Z < -1.65, P < 0.05) and 4 had moderate (Z < -1.00, P < 0.15) improvement in LV synchrony post-ASA. SLD < 0° predicted improvement in LV synchrony after ASA with a sensitivity of 81% and a specificity of 88%. SLD and LV dyssynchrony were frequent in HCM patients. HCM patients, whose septal activation became later than lateral activation, had significant reduction in septal activation delay and improvement in LV synchrony after ASA.
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http://dx.doi.org/10.1007/s10554-011-9942-y | DOI Listing |
Cardiol Rev
October 2024
Department of Cardiology, Royal Devon University Healthcare National Health Service Foundation Trust, Exeter, United Kingdom.
Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disorder characterized by structural and functional abnormalities. Current management strategies, such as medications and septal reduction therapies, have significant limitations and risks. Recently, cardiac myosin inhibitors (CMIs) like mavacamten and aficamten have shown promise as noninvasive treatment options.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
December 2024
3rd Department of Cardiology, "Sotiria" Chest Diseases Hospital, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece.
Hypertrophic cardiomyopathy (HCM) is a complex and heterogeneous cardiac disorder, often complicated by cardiogenic shock, a life-threatening condition marked by severe cardiac output failure. Managing cardiogenic shock in HCM patients presents unique challenges due to the distinct pathophysiology of the disease, which includes dynamic left ventricular outflow tract obstruction, diastolic dysfunction, and myocardial ischemia. This review discusses current and emerging therapeutic strategies tailored to address the complexities of HCM-associated cardiogenic shock and other diseases with similar pathophysiology that provoke left ventricular outflow tract obstruction.
View Article and Find Full Text PDFMedeni Med J
December 2024
Dr Siyami Ersek Thoracic and Cardiovascular Surgery Training Hospital, Clinic of Cardiology, Istanbul, Turkey.
Objective: Despite significant advances in understanding hypertrophic cardiomyopathy (HCM) in recent years, there is a need to improve risk stratification for patients at high risk of adverse outcomes. The relationship between inflammation and disease severity in HCM patients is known. Recently, a new inflammation parameter called the pan-immune inflammation value (PIV) has been introduced.
View Article and Find Full Text PDFAm J Cardiol
December 2024
Northwestern University, Feinberg School of Medicine, Chicago IL 60611; The Hypertrophic Cardiomyopathy Program at the Bluhm Cardiovascular Institute, Chicago IL 60611; Bluhm Cardiovascular Institute, Northwestern Memorial Hospital, Chicago IL 60611.
Background: Obstructive hypertrophic cardiomyopathy (HCM) is associated with significant morbidity due to left ventricular outflow tract (LVOT) obstruction. While alcohol septal ablation (ASA) is an established interventional treatment, mavacamten, a novel cardiac myosin inhibitor, has emerged as a non-invasive pharmacological alternative. Understanding the comparative efficacy of these two treatments is important for optimizing patient care.
View Article and Find Full Text PDFASAIO J
October 2024
From the Division of Cardiovascular Critical Care, Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
Patients with restrictive cardiomyopathy (RCM) and hypertrophic cardiomyopathy (HCM) were previously considered poor candidates for mechanical circulatory support due to technical limitations related to restrictive ventricular physiology and small ventricular size, limiting the ability to provide adequate flows and decompress the heart. Literature examining use of extracorporeal membrane oxygenation (ECMO) in this population consists of a single case series reporting no survivors. We report our experience providing ECMO in children with RCM or HCM at a large pediatric quaternary cardiac center.
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