Sudden death in a case of triple-vessel, non-atherosclerotic, diffuse ectasia and aneurysmal dilatations of the coronary arteries: An autopsy report.

Aneurysmal Coronary Artery Disease (ACAD) can occur as localized dilations of a segment of one or more coronary arteries or diffuse ectasia-type dilatations of one or more coronaries. Atherosclerosis remains the most common cause of these aneurysms, with Kawasaki Disease being implicated in the Asian population. We present a case of a 62-year-old Asian woman who dies suddenly with no prior symptoms.

Ischemic Stroke Prevention in Patients Atrial Fibrillation and a Recent Ischemic Stroke, TIA, or Intracranial Hemorrhage; a World Stroke Organisation (WSO) Scientific Statement.

Background: Secondary stroke prevention in patients with atrial fibrillation (AF) is one of the fastest growing areas in the field of cerebrovascular diseases. This Scientific statement from the World Stroke Organization Brain & Heart Task Force provides a critical analysis of the strength of current evidence this topic, highlights areas of current controversy, identifies knowledge gaps, and proposes priorities for future research.

Methods: We select topics with the highest clinical relevance and perform a systematic search to answer specific practical questions.

Influence of age and sex on the diagnostic yield of inherited cardiac conditions in sudden arrhythmic death syndrome decedents.

Aims: Sudden arrhythmic death syndrome (SADS) refers to a sudden death, which remains unexplained despite comprehensive post-mortem examination and a toxicological screen. We aimed to investigate the impact of age and sex on the overall diagnostic yield and underlying aetiology in decedents with SADS using a combined approach of familial evaluation (FE) and molecular autopsy (MA).

Methods And Results: Consecutive referrals to a single centre for FE only, MA only or both, following a SADS death were included.

A case of Sudden Cardiac Death with myocardial infarction due to iatrogenic coronary dissection in a patient with coronary vasospasm.

Coronary vasospasm and coronary artery dissection are two recognized causes of Sudden Cardiac Death [SCD] in association with Myocardial Infarction in Non-Obstructive Coronary Artery [MINOCA]. This is a case of post procedure death in a 50-year-old female during cardiac angiography, who had a family history of coronary spasm with sudden cardiac death in her mother. She went into cardiac arrest during the procedure with iatrogenic acute coronary dissection which was treated with LMS/LCx vessel stenting.

Tissue plasminogen activator associated pulmonary hemorrhage in patient with -associated arteriopathy.

Pulmonary hemorrhage after administration of tissue plasminogen activator (tPA) is a rare complication. We present a 15-year-old boy with a heterozygous pathogenic variant in who presented with right brachial artery aneurysm thrombosis treated initially with tPA, resulting in pulmonary hemorrhage. We hypothesize that the underlying pulmonary abnormalities caused by the pathogenic variant may be a risk factor for pulmonary hemorrhage after tPA administration.

Assessing hip joint-related structure and patient-reported outcomes in people with Marfan syndrome.

Objective: People with Marfan syndrome (MFS) have clinical symptoms of hip pain, but to date, there is limited knowledge about hip-related structural abnormalities in these patients. Therefore, the purpose of this cross-sectional study was to assess hip-related structural abnormalities and patient-reported outcomes (PRO) in a cohort of patients with MFS compared to healthy controls.

Methods: Nineteen individuals with MFS (17 females, 39.

Novel Insights into the Aortic Mechanical Properties of Mice Modeling Hereditary Aortic Diseases.

Objective:  Hereditary aortic diseases (hADs) increase the risk of aortic dissections and ruptures. Recently, we have established an objective approach to measure the rupture force of the murine aorta, thereby explaining the outcomes of clinical studies and assessing the added value of approved drugs in vascular Ehlers-Danlos syndrome (vEDS). Here, we applied our approach to six additional mouse hAD models.

Obesity Cardiomyopathy in Sudden Cardiac Death: A Distinct Entity? A Comparative Study.

Background: Obesity cardiomyopathy (OCM) can be associated with sudden cardiac death (SCD) but its pathologic features are not well described.

Objectives: The objective of this study was to characterize the clinical and pathological features of OCM associated with SCD.

Methods: This was a retrospective case control autopsy study.

Sudden Cardiac Death, Post-Mortem Investigation: A Proposing Panel of First Line and Second Line Genetic Tests.

Investigating the causes of Sudden cardiac death (SCD) is always difficult; in fact, genetic cardiac conditions associated with SCD could be "silent" even during autopsy investigation. In these cases, it is important to exclude other aetiology and assist to ask for genetic investigations. Herein, the purpose of this review is to collect the most-implicated genes in SCD and generate a panel with indications for first line and second line investigations.

Ethnicity and sudden cardiac death in athletes: insights from a large United Kingdom registry.

Aims: The relationship between ethnicity and causes of sudden cardiac death (SCD) in athletes is poorly understood.

Objectives: To investigate aetiology of SCD among different ethnicities in a large cohort of athletes.

Methods And Results: Between 1994 and November 2022, 7880 cases of SCD were consecutively referred from all over the United Kingdom to our national cardiac pathology centre; 848 (11%) were athletes.

Angiotensinogen as a Therapeutic Target for Cardiovascular and Metabolic Diseases.

AGT (angiotensinogen) is the unique precursor for the generation of all the peptides of the renin-angiotensin system, but it has received relatively scant attention compared to many other renin-angiotensin system components. Focus on AGT has increased recently, particularly with the evolution of drugs to target the synthesis of the protein. AGT is a noninhibitory serpin that has several conserved domains in addition to the angiotensin II sequences at the N terminus.

Incidence of quadricuspid pulmonary valves at postmortem examination.

Quadricuspid pulmonic valve is a rare congenital abnormality and because of its difficult non-invasive assessment, it is usually discovered incidentally at autopsies (reported prevalence in post-mortem specimens ranges from 1 in 400 to 1 in 2000). Unlike a bicuspid pulmonary valve, it rarely presents with clinical complications, such as valvular insufficiency or stenosis. Abnormal function is rarely reported in cases that are not associated with other congenital heart disease.

Yield of molecular autopsy in sudden cardiac death in athletes: data from a large registry in the UK.

Aims: Sudden cardiac death (SCD) may occur in apparently healthy individuals, including athletes. The aim was to investigate the diagnostic role of post-mortem genetic testing, molecular autopsy (MA), in elucidating the cause of SCD in athletes.

Methods And Results: We reviewed a database of 6860 consecutive cases of SCD referred to our specialist cardiac pathology centre.

Seasonal Variation in Sudden Cardiac Death: Insights from a Large United Kingdom Registry.

Background: Sudden cardiac death (SCD) is relatively common and may occur in apparently healthy individuals. The role of seasonal variation as a risk factor for SCD is poorly understood. The aim of this study was to investigate whether SCD exhibits a predilection for specific seasons.

Insights into malignant mitral valve degenerative disease from a sudden cardiac death cohort highlighting significant measurement differences from normal.

Aims: Mitral valve prolapse (MVP) is an accepted cause of sudden cardiac death (SCD) in most autopsy series. Diagnosis at autopsy relies upon subjective assessment with no established objective pathological criteria. This study set out to establish objective measurements to help pathologists dealing with SCD.

Sudden Cardiac Death in Young Athletes: JACC State-of-the-Art Review.

Athletes epitomize the healthiest segment of society. Despite this premise, sudden cardiac death may occur in apparently healthy athletes, attracting significant attention not only in the medical community but also in laypersons and media. The incidence of sudden cardiac death is variably reported, and epidemiological burden differs among cohorts.

Detailed study of collagen, vasculature, and innervation in the human cardiac conduction system.

Background: The cardiac conduction system (CCS) creates and propagates electrical signals generating the heartbeat. This study aimed to assess the collagen content, vasculature, and innervation in the human sinoatrial and atrioventricular CCS, and surrounding tissue.

Materials And Methods: Ten sinoatrial and 17 atrioventricular CCS samples were collected from 17 adult human autopsied hearts.

Innate immune signaling in hearts and buccal mucosa cells of patients with arrhythmogenic cardiomyopathy.

Background: Nuclear factor κB (NF-κB) signaling in cardiac myocytes causes disease in a mouse model of arrhythmogenic cardiomyopathy (ACM) by mobilizing CCR2-expressing macrophages that promote myocardial injury and arrhythmias. Buccal mucosa cells exhibit pathologic features similar to those seen in cardiac myocytes in patients with ACM.

Objectives: We sought to determine if persistent innate immune signaling via NF-κB occurs in cardiac myocytes in patients with ACM and if this is associated with myocardial infiltration of proinflammatory cells expressing CCR2.