BACKGROUND: Sudden deaths (SD) in young people including competitive athletes, albeit uncommon, are usually attributable to genetic, congenital or acquired cardiovascular conditions. However, it is under-appreciated that mitral valve prolapse (MVP), a relatively common valvular heart disease, is associated with SD in this youthful population.
METHODS: Forty-three MVP-related SDs are identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported.
RESULTS: Events occurred in both genders, but females were unexpectedly common (49%); median age was 22 ± 8 years, and 29 (67%) were engaged in competitive sports, including 17 with preparticipation examination. Of the 43 MVP cases, 21 died suddenly during or just after vigorous exercise including 6 during organized sports. Sixteen (37%) had been evaluated by a cardiologist resulting in confirmed MVP diagnosis in 11.. Pathologic findings characteristic of MVP included: bileaflet myxomatous involvement in all cases; and areas of interstitial or replacement myocardial fibrosis in 79%, most evident in posterolateral left ventricular wall.
CONCLUSIONS: Arrhythmogenic myxomatous degeneration (MVP) is an under-recognized cause of SD in young people including competitive athletes, disproportionally affecting females and requiring requires a high index of clinical suspicion. Frequency of left ventricular fibrosis in these young people with MVP suggests a mechanism for ventricular tachyarrhythmias and SD, relevant to future risk stratification.

Sudden cardiac death represents a significant diagnostic challenge for forensic pathologists, particularly in inherited arrhythmia syndromes or cardiomyopathies resulting from genetic defects. Molecular autopsies can reveal the underlying molecular etiology in such cases. In this study, we investigated a family with a history of sudden cardiac death to elucidate the molecular basis responsible for sudden cardiac death. The proband underwent a comprehensive forensic examination. Family members received thorough clinical evaluations, including electrocardiogram, Holter monitoring, echocardiography, and cardiac magnetic imaging. Whole exome sequencing and genetic analysis were performed on the deceased and her parents. In addition, Western blotting and patch-clamp recordings were employed to evaluate the expression and function of the mutant protein in vitro. Forensic examination diagnosed arrhythmogenic right ventricular cardiomyopathy (ARVC) as the cause of sudden death. Genetic analysis identified a novel missense mutation in SCN5A (p.V1323L), which was assessed as likely pathogenic by the ACMG guideline. Another family member carrying the mutation manifested long QT syndrome and mild cardiac fibrosis. The cellular electrophysiological study demonstrated that the mutation resulted in an enhanced late sodium current, suggesting it was a gain-of-function mutation. This study characterizes a novel SCN5A mutation that putatively causes long QT syndrome and may contribute to the development of ARVC. Our work expands the pathogenic spectrum of SCN5A variants and underscores the importance of molecular autopsy in sudden death cases, especially in those with suspected genetic disorders.

Background Cardiovascular diseases, especially ischemic heart disease, are the most frequent cause of sudden and unexpected death that constitute a significant portion of the autopsies conducted in our country. Though these deaths may be natural as well as unnatural, they carry medico-legal importance because they occur in a person who has been apparently healthy before the supervening of death, and the cause of death is difficult to ascertain. An infarction can be missed by gross and histological examination within the first few hours of sudden death. 2,3,5-triphenyl-tetrazolium chloride (TTC) is a sensitive histochemical method for diagnosing myocardial infarction within four hours of sudden death. The use of such dyes, hence, can possibly aid in ascertaining the cause of death in such cases wherein there are no known preceding factors. Aim The aim of this article was to study the occurrence of myocardial ischemia by histochemical staining method - 2,3,5-triphenyl-tetrazolium chloride (TTC).  Methods This study involved patients who underwent postmortem examination in the Department of Forensic Medicine and Toxicology, Sri Ramachandra Medical College and Research Institute, Chennai. Results Of 62 cases, 31 cases were found to be positive for TTC staining, and those heart slices were subjected to histopathological examination. The maximum number of cases (77.4%) showed the age of infarction within zero to four hours, which was detected early by TTC staining compared to microscopic changes in the heart. Only seven cases were positive for myocardial infarction by histopathological examination, proving that it is difficult to detect acute infarction if the age of infarction is less than four hours. Conclusion This suggests that for all sudden death cases, 2,3,5-triphenyl tetrazolium chloride could be used as a better tool for the identification of early infarcts.

OBJECTIVE: To explore how emergency nurses experienced caring for brought-in-dead persons and their relatives, and what hindered or facilitated this care in an emergency setting.
METHODS: A qualitative study using Interpretive Description.
METHODS: Data were collected as individual interviews with 13 nurses at seven Danish emergency departments from February to June 2023.
RESULTS: Our analysis revealed the overarching theme \'Navigating the complexities of providing holistic care in a constrained environment\', covering five sub-themes: (1) An important yet not recognized nursing task; (2) Pending care needs of the living and the dead; (3) No physical or mental room for the brought-in-dead persons; (4) Utilizing personal experiences in the absence of formal education and training and (5) Navigating professionalism and empathy.
CONCLUSIONS: Emergency departments posed unique challenges in providing care to brought-in-dead persons and their relatives.
UNASSIGNED: The unrecognized nature of caring for brought-in-dead persons and their relatives suggests a universal undervaluation of this care in emergency departments.
CONCLUSIONS: Care for brought-in-dead persons and their relatives is neither recognized nor evidence-based. This study initiates a discussion of the circumstances for delivering care for persons brought-in-dead and has an impact on nurses and nursing leaders employed in emergency departments.
UNASSIGNED: The Consolidated Criteria for Reporting Qualitative Research (COREQ).
UNASSIGNED: None.

OBJECTIVE: The indication for implantable cardioverter defibrillator (ICD) for sudden cardiac death (SCD) prevention relies mostly on left ventricular ejection fraction (LVEF) ≤ 35%. The use of a wearable cardioverter defibrillator (WCD) in the case of dynamic alterations of LVEF may help avoid an improper early ICD implant when a favourable evolution in the post-acute phase is observed and may help reduce costs.
METHODS: This parallel cohort retrospective study included patients with heart failure with reduced ejection fraction (HFrEF) at high risk of arrhythmias recruited in the acute phase and divided into an early ICD cohort and a WCD cohort for primary prevention during the waiting period established by European Society of Cardiology guidelines.
RESULTS: A total of 41 consecutive patients were enrolled: 26 in the WCD group and 15 in the early ICD group. Age, LVEF at baseline, causes of HFrEF and drug therapy in the two cohorts were similar. During the waiting period after the inclusion, three patients (11.5%) in the WCD cohort and four (26.7%) in the early ICD cohort developed relevant ventricular arrhythmias (P = 0.22); none of them had subsequent LVEF recovery. At the end of the waiting period, 13 patients (50%) in the WCD group and 7 (46.7%) in the early ICD group experienced LVEF recovery (P = 0.84). The average cost per patient at the end of the waiting period was €23 934 in the early ICD cohort versus €19 167 in the WCD cohort (-19.9%). This cost savings from WCD use appears even higher when projected over a 10 year period (-41.2%).
CONCLUSIONS: WCD may represent a cost-effective strategy to more accurately select candidates for the primary prevention ICD implant among high-risk patients with HFrEF. ICD use provides effective protection from SCD and reduces costs compared with an extensive early ICD implant.

BACKGROUND: Sudden non-cardiac death (SNCD) is a clinical entity comprising deaths lacking previous clinically significant symptoms, and in which the mechanisms of death do not involve the heart. Infection is a major cause of SNCD, particularly in children, and viruses are frequently involved in the disease process. Nevertheless, SNCD of viral infectious causes remains poorly characterized. Thus, a systematic review of the literature describing the association between viral infection and the development of SNCD was performed.
METHODS: PRISMA statement guidelines were followed in this systematic review. A literature search was conducted across MEDLINE, Scopus and Web of Science databases. Studies considered eligible were autopsy series or cohort studies of sudden death cases, in which evidence of viral disease as a cause of death was demonstrated, along with identification of causative agents.
RESULTS: Twelve studies published between 1996 and 2020 were included in this review. Selected studies were categorized into three groups according to the study population: infants and young children (up to four years of age); presumed sudden infant death syndrome patients; and older individuals (five years of age and older). SNCD with viral implication represents a minority of sudden death cases in all age groups, with infants and young children having a higher prevalence across studies. Respiratory infection was the main cause of viral SNCD, with influenza virus and respiratory syncytial virus being the most commonly identified agents in older individuals, and infants and young children respectively. Disseminated infection, gastrointestinal infection, and meningitis were other identified causes of SNCD in children.
CONCLUSIONS: No studies have directly assessed the frequency and causes of viral SNCD. Infants and young children show a considerable, but variable, prevalence of this clinical entity. Wider implementation of post-mortem virological molecular testing may help uncover previously unknown cases. More research into viral SNCD is needed, especially in the adult population.

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OBJECTIVE: Borzoi reportedly experience sudden death. The objective of this study was to report ECG intervals, amplitudes, and frequency of ECG abnormalities in clinically healthy Borzoi.
METHODS: 98 clinically healthy Borzoi were prospectively recruited and underwent echocardiogram, ECG, and cardiac troponin I testing between October 2020 and December 2022. Standard ECG measurements were obtained. Early repolarization notches and slurs were recorded.
RESULTS: Of 82 Borzoi with a structurally normal echocardiogram, ventricular arrhythmias were documented in 8 (10%) dogs, all of which had normal cardiac troponin I concentrations. Median P wave duration was 55 milliseconds (range, 45 to 70 milliseconds). Median PR interval was 125 milliseconds (range, 80 to 175 milliseconds). Thirty-one (38%) Borzoi had first-degree atrioventricular block (PR interval > 130 milliseconds). Median QRS duration was 65 milliseconds (range, 48 to 90 milliseconds). Median QT interval was 235 milliseconds (range, 185 to 275 milliseconds). Twenty-nine (35%) and 15 (18%) of 82 Borzoi had QT intervals > 240 or > 250 milliseconds, respectively. Sixty-seven of 82 (82%) Borzoi had early repolarization notches or slurs. Seventeen of 82 (21%) Borzoi had an abnormality of the ST segment, most commonly convexity/doming. Convexity of the ST segment was intermittent (n = 9) or persistent (4).
CONCLUSIONS: Ventricular arrhythmias, early repolarization, prolonged QT intervals, and ST segment abnormalities are not infrequent in clinically healthy Borzoi. P, PR, and QRS durations are commonly prolonged compared to general canine reference intervals.
CONCLUSIONS: Future study into heritable channelopathies in Borzoi is warranted given the frequency of ventricular arrhythmias, repolarization abnormalities, and sudden death in the breed. Breed-specific ECG reference intervals are needed.

OBJECTIVE: Anticoagulation can prevent stroke and prolong lives in patients with atrial fibrillation (AF). However, anticoagulated patients with AF remain at risk of death. The aim of this study was to investigate the causes of death and factors associated with all-cause and cardiovascular death in the XANTUS population.
RESULTS: Causes of death occurring within a year after rivaroxaban initiation in patients in the XANTUS programme studies were adjudicated by a central adjudication committee and classified following international guidance. Baseline characteristics associated with all-cause or cardiovascular death were identified. Of 11 040 patients, 187 (1.7%) died. Almost half of these deaths were due to cardiovascular causes other than bleeding (n = 82, 43.9%), particularly heart failure (n = 38, 20.3%) and sudden or unwitnessed death (n = 24, 12.8%). Fatal stroke (n = 8, 4.3%), which was classified as a type of cardiovascular death, and fatal bleeding (n = 17, 9.1%) were less common causes of death. Independent factors associated with all-cause or cardiovascular death included age, AF type, body mass index, left ventricular ejection fraction, hospitalization at baseline, rivaroxaban dose, and anaemia.
CONCLUSIONS: The overall risk of death due to stroke or bleeding was low in XANTUS. Anticoagulated patients with AF remain at risk of death due to heart failure and sudden death. Potential interventions to reduce cardiovascular deaths in anticoagulated patients with AF require further investigation, e.g. early rhythm control therapy and AF ablation.
BACKGROUND: NCT01606995, NCT01750788, NCT01800006.

BACKGROUND: Early-onset atrial fibrillation (AF) has already been observed in approximately 2% of patients with genetically proven long QT syndrome (LQTS). This frequency is higher than population-based estimates of early-onset AF. However, the concomitant expression of AF in LQTS is likely underestimated. The purpose of this study was to examine the clinical presentation, genetic background, and outcomes of a cohort of patients with LQTS and early-onset AF referred to a single tertiary center.
METHODS: Twenty-seven patients diagnosed with congenital LQTS were included in the study based on the documentation of early-onset (age ≤50 years) clinical or subclinical AF episodes in all available medical records, including standard electrocardiograms, wearable monitor or cardiac implantable electronic devices.
RESULTS: Seventeen patients experienced clinical AF during the follow-up period. Subclinical AF was detected in 10 patients through insertable or wearable cardiac monitors. In our series, the mean heart rate during AF episodes was found to be relatively low despite the patients\' young age and the low or minimal effective doses of beta-blockers used for QTc interval control. All patients exhibiting LQTS and early-onset AF were genotype positive, carrying mutations in the KCNQ1 (66%), KCNH2, KCNE1, and SCN5A genes. Notably, most of these patients carried the same p.(R231C) mutation in the KCNQ1 gene (59%) and were from the same families, suggesting concurrent expression of familial AF and LQTS.
CONCLUSIONS: LQTS patients are prone to developing clinical and subclinical AF, even at a younger age. The occurrence of early-onset AF in the LQTS population could be more frequent than previously assumed. AF should be considered as a potential dysrhythmia related to LQTS. Our study emphasizes the importance of carefully researching clinical and/or subclinical episodes of AF through strict heart rhythm monitoring in the LQTS population.