Sudden Death Research Articles

A Novel Missense Heterozygous Mutation in NKX2-5 Gene in a Family with Congenital Septal Defects and Cardiomyopathy: Case Series and Literature Review

AbstractSingle-gene mutations are important causes of congenital heart defects in children. Mutations in the NKX2-5 gene have been recently described in the literature as a cause of septal defects and cardiomyopathy. However, the spectrum of cardiac disease associated with NKX2-5 gene mutations is variable, ranging from asymptomatic septal defects to cardiomyopathy and sudden death. In this case report, we describe a case of 2-year-old child, along with two other family members, with a novel missense heterozygous (c.544G > T p.[Val182Phe]) mutation in NKX2-5 gene consistent with the diagnosis of autosomal dominant atrial septal defects with cardiomyopathy. This report can contribute to the understanding of genotype–phenotype correlations; it emphasizes the significant clinical relevance of NKX2-5 gene defects for congenital heart defects, sudden death, and cardiomyopathy, especially in multiple affected family members. It also suggests that individuals with NKX2-5 mutations are at risk of lethal arrhythmias and conduction disorders, that is why they should be evaluated routinely to assess the need for implantable cardioverter-defibrillator or pacemaker implantation.

Predictors of ventricular tachyarrhythmia in patients with a wearable cardioverter defibrillator: an international multicenter registry.

Wearable cardioverter defibrillator (WCD) can protect patients from sudden cardiac death due to ventricular tachyarrhythmias and serve as a bridge to decision of definite defibrillator implantation. The aim of this analysis from an international, multicenter WCD registry was to identify predictors of sustained ventricular tachycardia (VT) and/or ventricular fibrillation (VF) in this population. One thousand six hundred seventy-five patients with WCD were included in a multicenter registry from 9 European centers, with a median follow-up of 440days (IQR 120-893). The primary study end point was the occurrence of sustained VT/VF. Sustained VT was detected by WCD in 5.4% and VF in 0.9% of all patients. Of the 30.3% of patients receiving ICD implantation during follow-up, sustained VT was recorded in 9.3% and VF in 2.6%. Non-ischemic cardiomyopathy (HR 0.5, p < 0.001), and medication with angiotensin-converting enzyme inhibitors (HR 0.7, p = 0.027) and aldosterone antagonists (HR 0.7, p = 0.005) were associated with a significantly lower risk of VT/VF. Patients who received WCD due to a transient increased risk of sudden cardiac death have a comparatively lower risk of VT/VF in the presence of non-ischemic cardiomyopathy. Of note, optimal medical treatment for heart failure not only results in an improvement in left ventricular ejection fraction but also in a reduction in the risk for VT/VF.

Predictors of ventricular tachyarrhythmia in patients with a wearable cardioverter defibrillator: an international multicenter registry.

Wearable cardioverter defibrillator (WCD) can protect patients from sudden cardiac death due to ventricular tachyarrhythmias and serve as a bridge to decision of definite defibrillator implantation. The aim of this analysis from an international, multicenter WCD registry was to identify predictors of sustained ventricular tachycardia (VT) and/or ventricular fibrillation (VF) in this population. One thousand six hundred seventy-five patients with WCD were included in a multicenter registry from 9 European centers, with a median follow-up of 440days (IQR 120-893). The primary study end point was the occurrence of sustained VT/VF. Sustained VT was detected by WCD in 5.4% and VF in 0.9% of all patients. Of the 30.3% of patients receiving ICD implantation during follow-up, sustained VT was recorded in 9.3% and VF in 2.6%. Non-ischemic cardiomyopathy (HR 0.5, p < 0.001), and medication with angiotensin-converting enzyme inhibitors (HR 0.7, p = 0.027) and aldosterone antagonists (HR 0.7, p = 0.005) were associated with a significantly lower risk of VT/VF. Patients who received WCD due to a transient increased risk of sudden cardiac death have a comparatively lower risk of VT/VF in the presence of non-ischemic cardiomyopathy. Of note, optimal medical treatment for heart failure not only results in an improvement in left ventricular ejection fraction but also in a reduction in the risk for VT/VF.

Heritability of sudden cardiac death in Thoroughbred racehorses.

Sudden cardiac death (SCD) is an important cause of exercise-associated fatalities in Thoroughbred racehorses. Equine deaths share similarities with fatalities in human athletes that result from inherited cardiac disease. Whilst genetic causes have been postulated in horses, these have not been confirmed and heritability of SCD has not previously been estimated in Thoroughbred racehorses. To determine the heritability of SCD in a sample population of Thoroughbred racehorses. Retrospective case-control study. Steward and post-mortem reports of Thoroughbred racehorses in Australia between 2007 and 2020 were reviewed to identify horses with SCD. Control horses were randomly selected from races in which SCD occurred or from races on the date of the case fatality. A five-generation integrated pedigree chart was collected for each horse. Estimates of heritability were obtained using an animal model in the ASReml-R program with variance components estimated assuming SCD was normally distributed, and on the logit transformed scale. Inbreeding coefficients were calculated and the risk of producing SCD-affected progeny was calculated for stallions that sired ≥5 individuals in the case-control population. Ninety-three horses with SCD and 465 control horses were identified. Heritability on the underlying scale was 0.15 ± 0.09 (logit animal) and 0.24 ± 0.12 (normal animal). Inbreeding coefficients were not significantly different between groups. Of the 16 first generation sires that appeared ≥5 times in the case-control data set, two sires more frequently produced affected progeny (OR 7.95-10.41). Challenges in definitively confirming SCD may lead to misclassification of some cases. Some control horses may have not been exposed to environmental influences of SCD. Case numbers are low and the studied population may not represent the entire Thoroughbred genetic pool. The heritability of SCD in this population was relatively low. However, individual stallions appear more likely to produce affected progeny. Further studies are required to understand the genetic and environmental influences that contribute to disease expression.

Nexilin in cardiomyopathy: unveiling its diverse roles with special focus on endocardial fibroelastosis.

Cardiac disorders exhibit considerable heterogeneity, and understanding their genetic foundations is crucial for their diagnosis and treatment. Recent genetic analyses involving a growing number of participants have uncovered novel mutations within both coding and non-coding regions of DNA, contributing to the onset of cardiac conditions. The NEXN gene, encoding the Nexilin protein, an actin filament-binding protein, is integral to normal cardiac function. Mutations in this gene have been linked to cardiomyopathies, cardiovascular disorders, and sudden deaths. Heterozygous or homozygous variants of the NEXN gene are associated with the development of endocardial fibroelastosis (EFE), a rare cardiac condition characterized by excessive collagen and elastin deposition in the left ventricular endocardium predominantly affecting infants and young children. EFE occurs both primary and secondary to other conditions and often leads to unfavorable prognoses and outcomes. This review explores the role of NEXN genetic variants in cardiovascular disorders, particularly EFE, revealing that functional mutations are not clustered in a specific domain of Nexilin based on the cardiac disorder phenotype. Our review underscores the importance of understanding genetic mutations for the diagnosis and treatment of cardiac conditions.

Nexilin in cardiomyopathy: unveiling its diverse roles with special focus on endocardial fibroelastosis.

Cardiac disorders exhibit considerable heterogeneity, and understanding their genetic foundations is crucial for their diagnosis and treatment. Recent genetic analyses involving a growing number of participants have uncovered novel mutations within both coding and non-coding regions of DNA, contributing to the onset of cardiac conditions. The NEXN gene, encoding the Nexilin protein, an actin filament-binding protein, is integral to normal cardiac function. Mutations in this gene have been linked to cardiomyopathies, cardiovascular disorders, and sudden deaths. Heterozygous or homozygous variants of the NEXN gene are associated with the development of endocardial fibroelastosis (EFE), a rare cardiac condition characterized by excessive collagen and elastin deposition in the left ventricular endocardium predominantly affecting infants and young children. EFE occurs both primary and secondary to other conditions and often leads to unfavorable prognoses and outcomes. This review explores the role of NEXN genetic variants in cardiovascular disorders, particularly EFE, revealing that functional mutations are not clustered in a specific domain of Nexilin based on the cardiac disorder phenotype. Our review underscores the importance of understanding genetic mutations for the diagnosis and treatment of cardiac conditions.

Anxiety and depression in a patient with an implantable cardioverter defibrillator for early repolarization syndrome: A case report and clinical discussion

Early repolarization syndrome (ERS) was initially thought to be a good prognostic manifestation detectable by electrocardiogram. In recent years, with the gradual deepening of research and understanding, ERS is considered to possess malignant tendency and related to ventricular fibrillation (VF) and sudden cardiac death (SCD). Implantable cardioverter defibrillator (ICD) is currently the first choice of treatment used to reduce the risk of potentially life-threatening arrhythmias and SCD in patients at high risk for ERS. The stress of living with an implanted device and receiving ICD shock has been noted to exert a psychological toll on patients, especially those who have previously experienced VF and syncope. The current case report describes symptoms and signs of a patient with ERS, and discusses the changes of psychological condition after ICD implantation. Moreover, we explored a targeted treatment approach for anxiety and depression in an individual with an ICD for ERS, which combined medication, cognitive behavioral therapy, and physical exercise. We believe that the psychological experience of ICD recipients, which is a critical component in designing the biopsychosocial therapeutic approach for this growing patient population, is worthy of more attention.

Comprehensive assessment of the accuracy of heart rate determination with the personal wearable device

BACKGROUND: Increased resting heart rate (HR) is an independent risk factor for overall mortality, sudden cardiac death, and death from cardiovascular diseases (CVD) and a worsening factor for CVD patients’ prognosis. Moreover, HR is easy to measure and monitor independently using various devices. The accuracy of personal wearable devices used in assessing HR should be investigated. AIM: To evaluate the accuracy of HR determination with the personal wearable device (PWD). MATERIALS AND METHODS: An open, observational, single-center study was performed. Participants underwent physical examination, electrocardiography, HR recording using PWD, and electrocardiography Holter monitoring (HMECG). For each participant, the study duration was 14 days. The reliability of HR measured in everyday life was assessed using the PWD in comparison with HMECG as the “gold” standard. For statistical analysis, Microsoft Excel 2016 and STATISTICA 10.0 software were used. To assess the reliability of HR measured in everyday life using PWD in relation to HMECG, the hypothesis of the equality of average HR values measured by both methods was tested, a correlation analysis of time series of HR values was conducted, and Bland–Altman plots were used to visualize differences in HR values. RESULTS: The study involved 45 healthy individuals (22 men, 49.24±6.47 years). HR data were obtained over a 24-hour period, including the subjects' routine working day. The results demonstrated good convergence between data on HR using PWD and HMECG. The relative error in determining the HR of PWD did not exceed 3.2%. The smallest relative error was recorded at night and early morning hours (0.3÷1.1%). During the daytime it was slightly higher — from 2.0 to 3.2%. A significantly high positive correlation was obtained between HR recorded using both devices, both at night (MCorrP from 0.75 to 0.85; p 0.001) and during daytime (MCorrP from 0.77 to 0.85; p 0.001). CONCLUSION: The accuracy comparison of 24-hour HR measurements by PWD showed that they were mostly within the acceptable error range (less than 3.2%). Future studies should include HR PWD assessment in patients with various diseases.

Effect of adenosinergic manipulations on amygdala-kindled seizures in mice: Implications for sudden unexpected death in epilepsy.

Sudden unexpected death in epilepsy (SUDEP) results in more years of potential life lost than any neurological condition with the exception of stroke. It is generally agreed that SUDEP happens due to some form of respiratory, cardiac, and electrocerebral dysfunction following a seizure; however, the mechanistic cause of these perturbations is unclear. One possible explanation lies with adenosinergic signaling. Extracellular levels of the inhibitory neuromodulator adenosine rapidly rise during seizures, a countermeasure that is necessary for seizure termination. Previous evidence has suggested that excessive adenosinergic inhibition could increase the risk of SUDEP by silencing brain areas necessary for life, such as the respiratory nuclei of the brainstem. The goal of this investigation was to further clarify the role of adenosine in seizure-induced respiratory and electrocerebral dysfunction. To determine the role of adenosine in postictal physiological dysregulation, we pharmacologically manipulated adenosine signaling prior to amygdala-kindled seizures in mice while recording electroencephalogram (EEG), electromyogram, and breathing using whole body plethysmography. The adenosinergic drugs used in this study included selective and nonselective adenosine receptor antagonists and inhibitors of adenosine metabolism. We found that high doses of adenosine receptor antagonists caused some seizures to result in seizure-induced death; however, counterintuitively, animals in these conditions that did not experience seizure-induced death had little or no postictal generalized EEG suppression. Inhibitors of adenosine metabolism had no effect on postictal breathing but did worsen some postictal electrocerebral outcomes. The unexpected effect of high doses of adenosine antagonists on seizure-induced death observed in this study may be due to the increase in seizure severity, vasoconstriction, or phosphodiesterase inhibition caused by these drugs at high doses. These findings further clarify the role of adenosine in seizure-induced death and may have implications for the consumption of caffeine in epilepsy patients and the prevention of SUDEP.

Effect of adenosinergic manipulations on amygdala-kindled seizures in mice: Implications for sudden unexpected death in epilepsy.

Sudden unexpected death in epilepsy (SUDEP) results in more years of potential life lost than any neurological condition with the exception of stroke. It is generally agreed that SUDEP happens due to some form of respiratory, cardiac, and electrocerebral dysfunction following a seizure; however, the mechanistic cause of these perturbations is unclear. One possible explanation lies with adenosinergic signaling. Extracellular levels of the inhibitory neuromodulator adenosine rapidly rise during seizures, a countermeasure that is necessary for seizure termination. Previous evidence has suggested that excessive adenosinergic inhibition could increase the risk of SUDEP by silencing brain areas necessary for life, such as the respiratory nuclei of the brainstem. The goal of this investigation was to further clarify the role of adenosine in seizure-induced respiratory and electrocerebral dysfunction. To determine the role of adenosine in postictal physiological dysregulation, we pharmacologically manipulated adenosine signaling prior to amygdala-kindled seizures in mice while recording electroencephalogram (EEG), electromyogram, and breathing using whole body plethysmography. The adenosinergic drugs used in this study included selective and nonselective adenosine receptor antagonists and inhibitors of adenosine metabolism. We found that high doses of adenosine receptor antagonists caused some seizures to result in seizure-induced death; however, counterintuitively, animals in these conditions that did not experience seizure-induced death had little or no postictal generalized EEG suppression. Inhibitors of adenosine metabolism had no effect on postictal breathing but did worsen some postictal electrocerebral outcomes. The unexpected effect of high doses of adenosine antagonists on seizure-induced death observed in this study may be due to the increase in seizure severity, vasoconstriction, or phosphodiesterase inhibition caused by these drugs at high doses. These findings further clarify the role of adenosine in seizure-induced death and may have implications for the consumption of caffeine in epilepsy patients and the prevention of SUDEP.

A Simple and Systematic Approach to Qualitative Data Extraction From Social Media for Novice Health Care Researchers: Tutorial.

Social media analyses have become increasingly popular among health care researchers. Social media continues to grow its user base and, when analyzed, offers unique insight into health problems. The process of obtaining data for social media analyses varies greatly and involves ethical considerations. Data extraction is often facilitated by software tools, some of which are open source, while others are costly and therefore not accessible to all researchers. The use of software for data extraction is accompanied by additional challenges related to the uniqueness of social media data. Thus, this paper serves as a tutorial for a simple method of extracting social media data that is accessible to novice health care researchers and public health professionals who are interested in pursuing social media research. The discussed methods were used to extract data from Facebook for a study of maternal perspectives on sudden unexpected infant death.

A Calculated Risk: Evaluation of QTc Drug-Drug Interaction (DDI) Clinical Decision Support (CDS) Alerts and Performance of the Tisdale Risk Score Calculator.

A risk factor for a potentially fatal ventricular arrhythmia Torsade de Pointes is a prolongation in the heart rate-corrected QT interval (QTc) ≥ 500milliseconds (ms) or an increase of ≥60ms from a patient's baseline value, which can cause sudden cardiac death. The Tisdale risk score calculator uses clinical variables to predict which hospitalized patients are at the highest risk for QTc prolongation. To determine the rate of overridden QTc drug-drug interaction (DDI)-related clinical decision support (CDS) alerts per patient admission and the prevalence by Tisdale risk score category of these overridden alerts. Secondary outcome was to determine the rate of drug-induced QTc prolongation (diQTP) associated with overrides. Our organization's enterprise data warehouse was used to retrospectively access QTc DDI alerts presented for patients aged ≥18years who were admitted to Brigham and Women's Hospital during 2022. The QTc DDI CDS alerts were included if shown to a physician, fellow, resident, physician assistant, or nurse practitioner when entering the order in inpatient areas for patients with a length of stay of at least 2days. Variables collected for the Tisdale calculator included age, sex, whether patient was on a loop diuretic, potassium level, admission QTc value, admitting diagnosis of acute myocardial infarction, sepsis, or heart failure, and number of QTc-prolonging drugs given to the patient. A total of 2649 patients with 3033 patient admissions had 18,432 QTc DDI alerts presented that were overridden. An average of 3 unique QTc DDI alerts were presented per patient admission and the alerts were overridden an average of 6 times per patient admission. Overall, 6% of patient admissions were low risk (score ≤ 6), 64% moderate risk (score 7-10), and 30% high risk (score ≥ 11) of QTc prolongation. The most common QTc DDI alerts overridden resulting in an diQTP were quetiapine and propofol (11%) and amiodarone and haloperidol (7%). The diQTP occurred in 883 of patient admissions (29%) and was more frequent in those with higher risk score, with 46% of patient admissions with diQTP in high risk, 23% in moderate risk, and 8% in low risk. Use of the Tisdale calculator to assess patient-specific risk of QT prolongation combined with CDS may improve overall alert quality and acceptance rate, which may decrease the diQTP rate.

A Calculated Risk: Evaluation of QTc Drug-Drug Interaction (DDI) Clinical Decision Support (CDS) Alerts and Performance of the Tisdale Risk Score Calculator.

A risk factor for a potentially fatal ventricular arrhythmia Torsade de Pointes is a prolongation in the heart rate-corrected QT interval (QTc) ≥ 500milliseconds (ms) or an increase of ≥60ms from a patient's baseline value, which can cause sudden cardiac death. The Tisdale risk score calculator uses clinical variables to predict which hospitalized patients are at the highest risk for QTc prolongation. To determine the rate of overridden QTc drug-drug interaction (DDI)-related clinical decision support (CDS) alerts per patient admission and the prevalence by Tisdale risk score category of these overridden alerts. Secondary outcome was to determine the rate of drug-induced QTc prolongation (diQTP) associated with overrides. Our organization's enterprise data warehouse was used to retrospectively access QTc DDI alerts presented for patients aged ≥18years who were admitted to Brigham and Women's Hospital during 2022. The QTc DDI CDS alerts were included if shown to a physician, fellow, resident, physician assistant, or nurse practitioner when entering the order in inpatient areas for patients with a length of stay of at least 2days. Variables collected for the Tisdale calculator included age, sex, whether patient was on a loop diuretic, potassium level, admission QTc value, admitting diagnosis of acute myocardial infarction, sepsis, or heart failure, and number of QTc-prolonging drugs given to the patient. A total of 2649 patients with 3033 patient admissions had 18,432 QTc DDI alerts presented that were overridden. An average of 3 unique QTc DDI alerts were presented per patient admission and the alerts were overridden an average of 6 times per patient admission. Overall, 6% of patient admissions were low risk (score ≤ 6), 64% moderate risk (score 7-10), and 30% high risk (score ≥ 11) of QTc prolongation. The most common QTc DDI alerts overridden resulting in an diQTP were quetiapine and propofol (11%) and amiodarone and haloperidol (7%). The diQTP occurred in 883 of patient admissions (29%) and was more frequent in those with higher risk score, with 46% of patient admissions with diQTP in high risk, 23% in moderate risk, and 8% in low risk. Use of the Tisdale calculator to assess patient-specific risk of QT prolongation combined with CDS may improve overall alert quality and acceptance rate, which may decrease the diQTP rate.

2,3,5-Triphenyl Tetrazolium Chloride (TTC): A Dye to Detect Acute Myocardial Infarction in Cases of Sudden Death

2,3,5-Triphenyl Tetrazolium Chloride (TTC): A Dye to Detect Acute Myocardial Infarction in Cases of Sudden Death

2,3,5-Triphenyl Tetrazolium Chloride (TTC): A Dye to Detect Acute Myocardial Infarction in Cases of Sudden Death

2,3,5-Triphenyl Tetrazolium Chloride (TTC): A Dye to Detect Acute Myocardial Infarction in Cases of Sudden Death

Unexpected Sudden Deaths Following the Co-administration of Ceftriaxone and Lansoprazole: A Case Series

Unexpected Sudden Deaths Following the Co-administration of Ceftriaxone and Lansoprazole: A Case Series

Unexpected Sudden Deaths Following the Co-administration of Ceftriaxone and Lansoprazole: A Case Series

Unexpected Sudden Deaths Following the Co-administration of Ceftriaxone and Lansoprazole: A Case Series

Exposures to potentially psychologically traumatic events among Royal Canadian Mounted Police.

Royal Canadian Mounted Police (RCMP) are among the public safety sectors reporting the highest exposures to diverse potentially psychologically traumatic events (PPTEs), which contribute to the risk of developing mental health disorders. Current increases in prevalence of mental health disorders among RCMP are substantial and warrant updated prevalence estimates of PPTE exposures. The objective of the present study was to provide updated estimates of lifetime PPTE exposures among RCMP and assess for associations with mental disorders and sociodemographic variables. Participants (n = 1,348) completed a survey assessing self-reported sociodemographic information, PPTE exposures as measured by the Life Events Checklist for the Diagnostic and Statistical Manual of Mental Disorders (5th ed.) Extended, and mental health disorder symptoms. Participants reported exposure to more PPTE types (∼13) than previous samples of RCMP (∼11) and public safety personnel (PSP) (∼11). Prevalence for all the PPTE types was statistically significantly higher for the current sample than previously reported samples of PSP and RCMP, and higher than the Canadian general population. Participants reported highest exposures through any modality (i.e., directly or indirectly) to sudden violent death (98.3%), physical assault (97.9%), serious transport accident (97.0%), sudden accidental death (96.7%), and assault with a weapon (93.7%). Most PPTEs were statistically significantly associated with screening positive for posttraumatic stress disorder, major depressive disorder, generalized anxiety disorder, social anxiety disorder, and panic disorder, but not alcohol use disorder. Increased PPTE exposures reported by current participants compared to a previous sample of RCMP, coupled with the observed associations between PPTE exposures and mental health disorders, highlights the need for further resources and support for RCMP to mitigate and manage the impact of PPTEs. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Cardiac channelopathies in the context of hereditary arrhythmia syndromes

Genetic arrhythmia disorders are rare diseases; however, they are acommon cause of sudden cardiac death in children, adolescents, and young adults. In principle, adistinction can be made between channelopathies and cardiomyopathies in the context of genetic diseases. This paper focuses on the channelopathies long and short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT). Early diagnosis of these diseases is essential, as drug therapy, behavioral measures, and if necessary, implantation of acardioverter defibrillator can significantly improve the prognosis and quality of life of patients. This paper highlights the pathophysiological and genetic basis of these channelopathies, describes their clinical manifestations, and comments on the principles of diagnosis, risk stratification and therapy.

Assessing Cardiovascular Health in Paediatric Hypertrophic Cardiomyopathy Using the CANHEART Health Index

BackgroundPaediatric hypertrophic cardiomyopathy (HCM) is associated with sudden cardiac death, often resulting in restriction of strenuous physical activity. High levels of inactivity and a rise in youth obesity may increase risk of poor cardiovascular (CV) health in these patients. We aim to compare the CV disease risk profile of paediatric patients with HCM to Canadian youth. MethodsPatients with HCM (10-19 years) were recruited from 10 paediatric cardiac centres in Canada. The CV disease risk profile was determined using the Healthy Hearts School Program questionnaire and Cardiovascular Health in Ambulatory Care Research Team (CANHEART) health index. Poisson regressions were used to assess associations between the CV disease risk profile of paediatric patients with HCM and the overall CANHEART health score. ResultsA total of 56 subjects were enrolled (71% male, median age: 15.5 years [interquartile range: 13.8-16.8], median body mass index: 23.3 [interquartile range: 19.5-27.7]). Results of the CANHEART health index revealed that 89% of participants with HCM never tried smoking, 52% had a healthy body mass index, 39% consumed ≥5 fruits/vegetables per day, and 50% engaged in ≥60 minutes of walking or 20 minutes of running/jogging per day. When CANHEART intermediate and poor health index scores were combined, similar proportions were obtained for each group (HCM 82.1% vs Canadian youth 83.4%; P = 0.724). ConclusionsMany paediatric patients with HCM in Canada have intermediate or poor CV health as determined by the CANHEART health score, with high rates of obesity and low physical activity levels driving these trends. Interventions should be developed to promote positive CV health behaviours among patients with HCM.