Background: Since the initial clinical description of hypertrophic cardiomyopathy (HCM) over 60 years ago, sudden cardiac death (SCD) has been the most visible and feared complication of HCM.
Objectives: This study sought to characterize the temporal, geographic, and age-related trends of reported SCD rates in adult HCM patients.
Methods: Electronic databases were systematically searched up to November 2021 for studies reporting on SCD event rates in HCM patients. Patients with SCD equivalents (appropriate implantable cardioverter-defibrillator [ICD] shocks and nonfatal cardiac arrests) were not included. A random-effects model was used to pool study estimates calculating the overall incidence rates (IR) for each time-era, geographic region, and age group. We analyzed 2 periods (before vs after 2000, following clinical implementation of ICD in HCM). Following 2000, 5-year intervals were used to demonstrate the temporal change in SCD rates.
Results: A total of 98 studies (N = 70,510 patients and 431,407 patient-years) met our inclusion criteria. The overall rate of HCM SCD was 0.43%/y (95% CI: 0.37-0.50%/y; I = 75%; SCD events: 1,938; person-years of follow-up: 408,715), with young patients (<18 years of age) demonstrating a >2-fold-risk for sudden death vs adult patients 18-60 years of age (IR: 1.09%; 95% CI: 0.69%-1.73% vs IR: 0.43%; 95% CI: 0.37%-0.50%) (P value for subgroup differences <0.01). Contemporary SCD rates from 2015 to present were 0.32%/y and significantly lower compared with 2000 or earlier (IR: 0.32%; 95% CI: 0.20%-0.52% vs IR: 0.73%; 95% CI: 0.53%-1.02%, respectively). Reported SCD rates for HCM were lowest in North America (IR: 0.28%; 95% CI: 0.18%-0.43%,) and highest in Asia (IR: 0.67%; 95% CI: 0.54%-0.84%).
Conclusions: Contemporary HCM-related SCD rates are low (0.32%/y) representing a 2-fold decrease compared with prior treatment eras. Young HCM patients are at the highest risk. The maturation of SCD risk stratification strategies and the application of primary prevention ICD to HCM are likely responsible for the notable decline over time in SCD events. In addition, worldwide geographic disparities in SCD rates were evident, underscoring the need to increase access to SCD prevention treatment for all HCM patients.
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http://dx.doi.org/10.1016/j.jacep.2022.07.012 | DOI Listing |
Heart Rhythm
January 2025
Department of Cardiovascular Medicine, Section of Cardiovascular Imaging, Cleveland Clinic Foundation, Cleveland, OH. Electronic address:
Background: Better risk stratification is needed to evaluate patients with non-ischemic cardiomyopathy (NICM) for prophylactic implantable cardioverter-defibrillators (ICD). Growing evidence suggests cardiac magnetic resonance imaging (CMR) may be useful in this regard.
Objective: We aimed to determine if late-gadolinium enhancement (LGE) seen on CMR (dichotomized as none/minimal <2% vs significant ≥2%) predicts appropriate ICD therapies (primary endpoint) and/or all-cause mortality/transplant/left-ventricular assist device (LVAD) implantation (secondary endpoint) in NICM patients.
Forensic Sci Med Pathol
January 2025
Department of Forensic Pathology, School of Forensic Medicine, China Medical University, Shenyang, 110122, P. R. China.
Forensic diagnosis of sudden cardiac death (SCD) is an extremely important part of routine forensic practice. The present study aimed to develop and validate nomograms for predicting the probability of SCD with special regards to ischemic heart disease-induced SCD (IHD-induced SCD) based on multiple autopsy variables. A total of 3322 cases, were enrolled and randomly assigned into a training cohort (n = 2325) and a validation cohort (n = 997), respectively.
View Article and Find Full Text PDFMayo Clin Proc
January 2025
Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN; Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN; Division of Heart Rhythm Services, Department of Cardiovascular Medicine, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, MN. Electronic address:
Objective: To test whether an artificial intelligence (AI) deep neural network (DNN)-derived analysis of the 12-lead electrocardiogram (ECG) can distinguish patients with long QT syndrome (LQTS) from those with acquired QT prolongation.
Methods: The study cohort included all patients with genetically confirmed LQTS evaluated in the Windland Smith Rice Genetic Heart Rhythm Clinic and controls from Mayo Clinic's ECG data vault comprising more than 2.5 million patients.
Epilepsia
January 2025
Division of Pediatric Neurology and Developmental Medicine, Department of Pediatrics, Duke University, Durham, North Carolina, USA.
Objective: This study was undertaken to test the following hypotheses in the Atp1a3 mouse (which carries the most common human ATP1A3 (the major subunit of the neuronal Na/K-adenosine triphosphatase [ATPase]) mutation, D801N): sudden unexpected death in epilepsy (SUDEP) occurs during seizures and is due to terminal apneas in some and due to lethal cardiac arrhythmias in others; and Atp1a3 mice have central cardiorespiratory dysregulation and abnormal respiratory drive.
Methods: Comparison was made of littermate wild-type and Atp1a3 groups using (1) simultaneous in vivo video-telemetry recordings of electroencephalogram, electrocardiogram, and breathing; (2) whole-body plethysmography; and (3) hypoglossal nerve recordings.
Results: In Atp1a3 mice, (1) SUDEP consistently occurred during seizures that were more severe than preterminal seizures; (2) seizure clustering occurred in periods preceding SUDEP; (3) slowing of breathing rate (BR) and heart rate was observed preictally before preterminal and terminal seizures; and (4) the sequence during terminal seizures was as follows: bradypnea with bradycardia/cardiac arrhythmias, then terminal apnea, followed by terminal cardiac arrhythmias.
Turk Kardiyol Dern Ars
January 2025
Department of Cardiology, Isfahan University of Medical Sciences, Isfahan, Iran.
Hypereosinophilic syndrome (HES) is traditionally described as chronic peripheral eosinophilia with involvement of various organs and systems, including the heart and nervous system. In this report, we describe cardiac involvement and border zone stroke in a patient with idiopathic HES. A 37-year-old woman presented with sudden right-sided weakness and slurred speech, which began four days before admission, accompanied by palpitations, retrosternal exertional chest discomfort, dry cough, and progressive shortness of breath over approximately two months.
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