4: Sudden Death Flashcards

Question

Define cardiomyopathies

Answer 1

- General term for diseases of myocardium - Associated with mechanical/electrical dysfunction - Primary ○ Originate from heart - Secondary ○ Myocardial involvement as component of systemic disorder § Haemochromatosis § Amyloidosis

Answer 2

``` ○ Dilated § Impairment of contractility ○ Hypertrophic § Impairment of compliance ○ Restrictive § Impairment of compliance ```

Answer 3

Progressive cardiac dilation and contractile dysfunction -> systolic dysfunction Normal wall thickness

Answer 4

``` ○ Genetic ○ Chronic Alcoholism ○ Pathologic Heart Valves ○ Supraphysiologic stress § Hyperthyroidism § Hypertension § Foetuses of insulin dependent mothers ○ Viral Myocarditis ○ Iron Overload ○ Pregnancy ○ Inflammatory diseases § Granulomatosis with polyangiitis § Sarcoidosis § Amyloidosis § Lupus ○ Idiopathic ```

Answer 5

``` ○ Four chamber dilation ○ Ventricular hypertrophy ○ Interstitial fibrosis ○ Thrombus formation ○ Reduced cardiac ejection ○ Valvular dysfunction ○ Atrial fibrillation ○ Stroke ○ Heart Failure ○ Sudden death ```

Answer 6

``` - Gross ○ Enlarged ○ Flabby ○ Mural thrombi ○ No primary cardiac dysfunction § Functional regurgitation due to ventricular dilation - Microscopic ○ No specific micro features ○ Interstitial and endocardial fibrosis ○ Hypertrophy of myocytes ```

Answer 7

Autosomal dominant disorder Replacement of right ventricle free wall by fat and fibrosis Manifests with right-sided heart failure and rhythm disturbances

Answer 8

○ Gross § Dilation of right ventricle § Replacement of right ventricle free wall by fat and fibrosis ○ Micro § Replacement of right ventricle free wall by fat and fibrosis

Answer 9

- Clinically heterogenous genetic disorder - Characterised by ○ Myocardial hypertrophy ○ Poorly compliant left ventricular myocardium - Lead to ○ Abnormal diastolic filling ○ Left ventricular outflow obstruction

Answer 10

○ Genetic § Mutations in genes that encode sarcomere proteins ○ Friedreich’s ataxia § Genetic, progressive, neurodegenerative movement disorder § Mutations in FNX gene ○ Storage diseases ○ Infants of diabetic mothers

Answer 11

``` ○ Reduced stroke volume ○ Outflow obstruction ○ Exertional dyspnoea ○ Systolic ejection murmur ○ Atrial fibrillation ○ Endocarditis ○ Mural thrombus formation ○ Cardiac failure ○ Ventricular arrhythmias ○ Sudden death ```

Answer 12

``` - Gross ○ Asymmetric septal hypertrophy ○ Without ventricular dilation - Micro ○ Myocyte disarray ○ Extreme hypertrophy ○ Exaggerated myocyte branching ○ Interstitial fibrosis ```

Answer 13

``` Primary decrease in ventricular compliance resulting in impaired ventricular diastolic filling Build-up of substances in the myocardium ○ Amyloidosis ○ Radiation induced fibrosis ○ Haemochromatosis ○ Idiopathic ```

Answer 14

- Gross ○ Normal size ventricles ○ Bi-atrial dilation due to restricted ventricular filling and pressure overloads - Histological ○ Patch or diffuse interstitial fibrosis ○ Deposition of substances

Answer 15

Group of pathologic entities in which infectious microorganisms and/or primary inflammatory process causes myocardial injury

Answer 16

``` ○ Infection § Viruses □ Coxsackie virus A and B □ Cytomegalovirus □ HIV § Bacteria □ Neisseria meningococcus □ Borrelia (Lyme Disease) □ Protozoa □ Trypanosoma cruzi (Chagas disease) ○ Immune mediated § Post-viral § Poststreptococcal § Systemic lupus erythema § Drug hypersensitivity § Transplant rection ○ Other § Sarcoidosis § Giant cell myocarditis ```

Answer 17

- Gross ○ Often mottled by either pale foci or minute haemorrhage lesions ○ Mural thrombi present ○ Small yellow pinpoint micro abscesses on epicardial surface - more common in bacterial - Micro ○ Micro abscess - bacterial colonies surrounded by acute inflammatory cells ○ Interstitial inflammatory infiltrate with degenerating or apoptotic myocytes § Viral = lymphocytic ○ Large zones of necrosis ○ Interstitial oedema

Answer 18

- Increased fluid leakage from blood vessels and injury to various cells of the CNS - Vasogenic oedema ○ Increase in extracellular fluid caused by blood-brain barrier disruptions and increased vascular permeability ○ Shift of fluid from intravascular compartment to intracellular spaces ○ Can be localised (adjacent to inflammation or neoplasms) or generalised (global ischaemic injury) - Cytotoxic oedema ○ Increase in intracellular fluid secondary to neuronal, glial or endothelial cell membrane injury ○ Generalised hypoxic/ischaemic insult ○ Metabolic derangement that prevents maintenance of the normal ionic gradients

Answer 19

- Sum of brain, CSF and intracerebral blood is constant - Eventually compensatory mechanisms will become exhausted - Enters decompensatory state - ICP rises SOL Cerebral Oedema Haemorrhage

Answer 20

- Herniation = displacement of brain tissue past rigid dural folds (falx and tentorium) or through openings in the skull because of increased intracranial pressure - Subfalcine (cingulate) ○ Unilateral or asymmetric expansion of a cerebral hemisphere displaced cingulate gyrus under falx ○ -> compression of anterior cerebral artery and branches -> secondary infarcts - Transtentorial (uncal, mesial temporal) ○ Medial aspect of temporal lobe is compressed against the free margin of the tentorium ○ Third cranial nerve compromised -> pupillary dilation and impaired ocular movements ○ Posterior cerebral artery compressed -> infarct ○ Contralateral cerebral peduncle compressed -> hemiparesis ipsilateral to side of herniation ○ Duret haemorrhages - secondary haemorrhagic lesions in midbrain and pons - Tonsillar herniation ○ Displacement of cerebellar tonsils through the foramen magnum ○ Life threatening ○ Brainstem compression and compromises vital resp and cardiac centres in the medulla What are the complications of herniation? - Cingulate ○ Compression of the Anterior Cerebral Artery and its branches leading to secondary infarcts and further cerebral oedema - Uncal ○ CNIII palsy ○ Reduced perfusion of the PCA ○ Infarct of the primary visual cortex ○ Hemiparesis ○ Duret haemorrhages - Tonsillar ○ Death

Answer 21

○ Status epilepticus ○ Accident during seizure - drowning, trauma ○ Aspiration during seizure ○ Suicide

Answer 22

``` ○ Establish cause of death - exclude other causes of sudden death ○ Identify/confirm cause of epilepsy ○ Inform bereaved family ○ Public health reasons § Accurate statistics of incidence § Setting healthcare targets/policies ○ Research § Causes of epilepsy and sudden death § Reducing epilepsy deaths ```

Answer 23

- Hospital or GP notes - ‘Verbal autopsy’- witnesses of the death or next of kin may be able to provide valuable information-Knowing the circumstances surrounding the death will contribute - Epilepsy - Type, frequency, tests, medication Hx - Other medical conditions - Circumstances at time of death Witnesses, location/position, micturition, trauma

Answer 24

- Sudden, unexpected, unwitnessed or witnessed, non-traumatic and non-drowning deaths in patients with epilepsy with or without evidence for a seizure and excluding documented status epilepticus, where post-mortem examination does not reveal a cause for death ○ Patient has epilepsy ○ Patient died unexpectedly while in reasonable state of health ○ Death occurred suddenly ○ Death occurred during normal and benign circumstances ○ Obvious medical cause of death not determined at autopsy Death was not direct restful of seizure or status epilepticus

Answer 25

``` - Epilepsy ○ Severity ○ Early onset ○ Nocturnal ○ Poor control ○ Unsupervised ○ Dravet syndrome - Personal ○ Male ○ 20-40 years ○ Developmental delay - Other ○ Hx of alcohol abuse - Drugs ○ Multiple AEDs ○ Frequent change ○ Poor compliance ```

Answer 26

- External ○ Head injuries ○ Incontinence ○ Petechial haemorrhage - asphyxia - Internal ○ Examination and sectioning of tongue - internal extravasion of blood -> tongue biting ○ Cardiovascular system - exclude cardiac cause § Syncope and seizures confused § Hypertrophic cardiomyopathy and myocardial hypertrophy through histology ○ Respiratory system § Check airway and larynx for obstruction/foreign bodies (exclude choking during seizure) § Pulmonary aspiration of stomach contents should be confirmed through histology

Answer 27

- Cardiovascular ○ Heart weight maybe increased ○ Mild interstitial fibrosis ○ Mild cardiac hypertrophy - Pulmonary ○ Increased lung weights ○ Pulmonary oedema and congestion § Acute neurogenic pulmonary oedema § Acute cardiac decompensation following seizure ○ Aspiration of gastric contents or change due to drowning - CNS ○ Exclude brain swelling with herniation, cerebral tumour and haemorrhage ○ Mild cerebral oedema has been reported ○ Low-grade cerebral tumours ○ Old TBI/contusions ○ Malformations of cortical development ○ Hippocampal sclerosis ○ Cerebellar atrophy (seizures/phenytoin) - Liver ○ Increased weight ○ Venous congestions ○ Fatty liver

Answer 28

- Alcohol and illicit drugs - Blood and urine testing for AEDs- presence, levels, to ascertain evidence of compliance/overdose - Low AED levels reported in some SUDEP cases - Should be interpreted with caution, levels may be lower - Analysis of hair samples post mortem may provide an alternative more accurate analysis of drug compliance - Microbiology: CSF sample for suspected encephalitis - Genetic testing: e.g. channelopathy causing arrhythmia & seizure

Answer 29

- Probably multifactorial - Overlapping mechanisms - Respiratory ○ Seizures cause central apnoea and oxygen desaturation § Arrhythmias ○ Asphyxiation secondary to obstruction - Cardiac ○ Arrhythmias § Asystole § Ictal tachycardia § Ictal bradycardia ○ Heart rate variability § Decreased in temporal lobe epilepsy § Increased likelihood of arrhythmias ○ Fibrosis - Genetic factors

Answer 30

Sudden unexpected death in infancy/childhood (SUDI/SUDC): death of a child which would not have been reasonable expected 24 hours previously and in whom no pre-existing medical cause of death is apparent. ○ Includes deaths for which a cause is ultimately found (‘explained SUDI/SUDC’) and those that remain unexplained following investigation (SUDI<2 years of age

Answer 31

Sudden infant death syndrome (SIDS): the sudden and unexpected death of an infant under 12 months of age, with onset of the lethal episode apparently occurring during normal sleep, which remains unexplained after thorough investigation including a complete post-mortem examination and review of the circumstances of death and the clinical history.

Answer 32

- First few hours:  Immediate response by ambulance and police. Resuscitation, scene security and consideration of needs of siblings and other family members.  Transfer to hospital if appropriate. Decision to continue/stop resus. Attendance at hospital by lead police investigator.  Support for carer/family members. Joint history, examination and immediate investigations by paediatrician and police.  Notification of coroner, CDOP, GP, social care. - First 1-2 days:  Initial planning meeting/discussion.  Joint home visit by police and paediatrician/nurse. Report to coroner  Post-mortem.  Further police investigations – review of health and social care information. - 1-6 months:  Issue of PM report  Local Case Discussion with review of circumstances and feedback of outcomes of Local Case Discussion. Report provided to Coroner and CDOP.  Coroner’s Inquest  Child Death Overview Panel (CDOPP)

Answer 33

- Previous or ongoing child safeguarding concerns e.g. on a Child Protection Plan. - Previous sibling deaths. Prompts family history, review of both deaths and - consideration of genetic testing. - Delay in seeking help without adequate explanation or persistently missing - appointments. - Inconsistent explanations: ○ Explanation changes with time or questioning. ○ The reported cause was beyond the infant’s development. ○ Explanation in relation to time of death is not supported. - Unexplained injury at death or in past: ○ Multiple bruises ○ Bruises to an immobile infant or atypical with infant’s development ○ Fingerprint bruises and linear bruises ○ Injury to frenulum ○ Petechial haemorrhages ○ Blood around mouth and nose ○ Burns, scalds, bite marks, fractures or drugs present on toxicology ○ Foreign body in upper airway - History of domestic abuse within family - Evidence of past/present drug/alcohol abuse - Evidence of parental mental health problems, including fabricated or induced illness - Neglect issues. Observations about physical condition of child and accommodation, general hygiene - and cleanliness, adequacy of food, clothing and bedding. - Previous convictions of parents and carers, particularly violence to children - Infant with learning or physical disability or significant pre-existing medical condition - Abusive head trauma apnoea, seizures, unexplained drowsiness/LOC

Answer 34

- To establish the cause of death and to address the issues related to the circumstances of death, in particular: ○ Whether the death is attributable to natural disease ○ To consider the possibility of accidental death ○ To consider the possibility of asphyxia/airway obstruction ○ To consider the possibility of inflicted injury ○ To document the presence or absence of pathological processes and to determine how the death came about.

Answer 35

- Paediatric post-mortem ○ SUDI PMs should be performed by a specialist paediatric pathologist. ○ If suspicion of abuse, joint PM with forensic pathologist should be carried out. ○ Specific examination for evidence of injury should be documented prior to PM, including full skeletal survey, reported by a radiologist with specialist expertise in this area. ○ PM should include external and internal exam, with consideration of phytography or other documentation of findings. ○ If suspicious findings encountered in a previously unsuspected case, the exam should be halted and coroner and police informed - Samples ○ Histology: lungs, heart, kidneys, liver, thymus, pancreas, spleen, lymph glands, adrenal glands, costochondral junction (including marrow), muscle, any lesions, brain, other as specified by case. ○ Bacteriology: blood, CSF, respiratory tract, spleen. ○ Virology: lung, CSF, other suspected fluid e.g. faeces ○ Biochemistry: urine, blood, bile spot on Guthrie card (metabolic testing) ○ Frozen sections for fat: heart, liver, kidney, muscle ○ Toxicology: urine. Liver, stomach content ○ Skin: fibroblast culture if metabolic disease suspected ○ Genetic testing

Answer 36

- Accidental ○ Trauma ○ Asphyxia ○ RTC ○ Drowning ○ Fires/burns ○ Poisoning ○ Electrocution ○ Drug ○ Alcohol abuse - Natural: ○ Cardiac: congenital, myocarditis, rheumatic fever, cardiomyopathies ○ Respiratory: neonatal hyaline membrane disease, asthma, pneumonia, CF ○ Neurologic: vascular malformation, tumour, hydrocephalus, seizure ○ Haematologic: sickle cell, bleeding disorder ○ Gastrointestinal: infection, intestinal obstruction, intestinal perforation, CF ○ Metabolic: DM, fatty oxidation disorders, amino-acid disorders, enzyme disorders

4: Sudden Death Flashcards

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