Sudden arrhythmic death syndrome

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Sudden arrhythmic death syndrome
Other namesSudden adult death syndrome (SADS), bed death, sudden unexpected/unexplained death syndrome (SUDS), sudden unexpected/unexplained nocturnal death syndrome (SUNDS), Sudden Death Syndrome, Sudden Death, Delayed Death
Ventricular fibrillation.png
A type of a deadly ventricular arrhythmia - Ventricular fibrillation pattern seen on an ECG
Specialty Cardiology   OOjs UI icon edit-ltr-progressive.svg
Causes
  • Ion channelopathies:
    Long QT syndrome (LQTS),
    Brugada syndrome,
    CPVT (catecholaminergic polymorphic ventricular tachycardia),
    PCCD (progressive cardiac conduction defect),
    Early repolarization syndrome,
    Mixed sodium channel disease,
    Short QT syndrome, Idiopathic ventricular fibrillation (IVF)
  • Structural heart disease:
    Arrhythmogenic right ventricular cardiomyopathy (ARVC),
    Dilated cardiomyopathy (DCM),
    Hypertrophic cardiomyopathy (HCM),
    Wolff-Parkinson-White Syndrome (WPW),
    Mitral valve prolapse (MVP)
Risk factors Family history of heart disease, [1] Asian ancestry, [2] Primary arrhythmia, atherosclerosis, [3] type 1 diabetes [4]
Frequency14.9 per 100,000 people in Japan
110.8 per 100,000 people in United States [5]

Sudden arrhythmic death syndrome (SADS) is a sudden unexpected death of adolescents and adults caused by, as the name implies, a cardiac arrest. However, the exact cause of the cardiac arrest, and thus the exact cause of death, is unknown. These deaths occur mainly during sleep or at rest. [6] One type of conduction defect known as Brugada syndrome can be responsible. [7] [8]

Contents

The syndrome is rare in most areas around the world but occurs in populations that are culturally and genetically distinct. It was first noted in 1977 among southeast Asian Hmong refugees in the United States and Canada. [9] [10] The syndrome was again noted in Singapore when a retrospective review of records showed that 230 otherwise healthy Thai foreign workers living in Singapore died suddenly of unexplained causes between 1982 and 1990. [11]

Causes

Sudden death of a young person can be caused by heart disease (including cardiomyopathy, congenital heart disease, myocarditis, genetic connective tissue disorders) or conduction disease (WPW syndrome, etc.), medication-related causes or other causes. [12] Rare diseases called ion channelopathies may play a role such as long QT syndrome (LQTS), Brugada syndrome (BrS), CPVT (catecholaminergic polymorphic ventricular tachycardia), progressive cardiac conduction defect (PCCD), early repolarization syndrome, mixed sodium channel disease, and short QT syndrome. [12] In 20% of cases, no cause of death can be found, even after extensive examination. [12]

In young people with type 1 diabetes, unexplained deaths could be due to nighttime hypoglycemia triggering abnormal heart rhythms or cardiac autonomic neuropathy, damage to nerves that control the function of the heart. [4]

Medical examiners have taken into account various factors, such as nutrition, toxicology, heart disease, metabolism, and genetics. Although there is no real known definite cause, extensive research showed victims aged 18 or older were found to have had a hypertrophic cardiomyopathy, a condition in which the heart muscle becomes oddly thickened without any obvious cause. [3] This was the most commonly identified abnormality in sudden death of young adults. Where people have died suddenly, it is most commonly found that they had had CAD (coronary artery disease) or ASCAD (atherosclerotic coronary artery disease), or any level of stress. [3] However, studies reveal that people were known to have had symptoms within the week before the terminal event such as chest pain at ~52% of patients, dyspnea at ~22%, and syncope at ~7%. About 19% are not known to have experienced symptoms. [3] Scientists have also associated this syndrome with a mutation of gene SCN5A that affects the function of the heart. A 2011 autopsy-based study found that sudden death was attributed to a cardiac condition in 79.3% of cases, and was unexplained in 20.7%. [3]

In the Philippines, sudden adult death syndrome (or in their term, bangungot) is mainly caused by the Brugada syndrome. [13]

Diagnosis

By definition, the diagnosis can only occur post-mortem after other causes are ruled out.

Prevention

A 2011 retrospective cohort study using demographic and autopsy data for a 10-year period comprising 15.2 million person-years of active surveillance suggested prevention of sudden death in young adults should focus on evaluation for causes known to be associated with SCD (e.g., primary arrhythmia) among persons less than 35 years old, and emphasise atherosclerotic coronary disease in those older. [3]

A 2003 study found that the only proven way to prevent SADS is with an implantable cardioverter-defibrillator. Oral beta-blockers such as propranolol are ineffective. [14]

Epidemiology

In 1980, a reported pattern of sudden deaths was brought to the attention of the Centers for Disease Control. The first reported sudden death occurred in 1948 when there were 81 similar deaths of Filipino men in Oahu County, Hawaii. However, it did not become relevant because there was no associated pattern. This syndrome continued to become more significant as years went on. By 1981–1982, the annual rate in the United States was high with 92/100,000 among Laotians-Hmong, 82/100,000 among other Laotian ethnic groups, and 59/100,000 among Cambodians. [2]

In a 2008 study it was found that over half of SADS deaths could be attributed to inherited heart disease: unexplained premature sudden deaths in family, long QT syndrome, Brugada syndrome, arrhythmogenic right ventricular cardiomyopathy and others. [1]

A national SADS study in England, funded by the British Heart Foundation, reported results in a 2007 journal article published in Health. [15] The study surveyed 117 coroners' jurisdictions in England. Researchers found that deaths from SADS reported by these coroners occurred "predominantly in young males". There were 500 cases a year in England, eight times more than had previously been estimated. Families are more at risk of SADS if they have a genetic cardiac disease. The study recommended that affected families should undergo "specialised cardiological evaluation". [15]

Asia

Southeast Asian immigrants, who were mostly fleeing the Vietnam War, most often had this syndrome, marking Southeast Asia as the area containing the most people with this fatal syndrome. There are other Asian populations that were affected, such as Filipinos and Chinese Filipinos, Japanese in Japan, and natives of Guam in the United States and Guam. [2] The immigrants with this syndrome were about 33 years old and seemingly healthy, and all but one of the Laotian Hmong refugees were men. [16] The condition appears to primarily affect young Hmong men from Laos (median age 33) [2] and Northeastern Thailand (where the population is mainly of Laotian descent). [17] [18]

History

Map of Thailand highlighting Loei Province Thailand Loei.png
Map of Thailand highlighting Loei Province

Laotian Hmongs were chosen for the study because they had one of the highest sudden death rates in the United States while sleeping. They were originally from Southern China and the highlands of North Vietnam, Laos, and Thailand. The location that was picked for this study was in Ban Vinai in the Loei Province, which is approximately 15 kilometers from the Lao border. This study took place between October 1982 and June 1983 as this syndrome became more of a pressing issue. Ban Vinai was the location chosen because it had 33,000 refugees in 1982, and the largest number of recorded SADS deaths. Because this syndrome was occurring most commonly in those particular men, researchers found it most beneficial and effective to study the populations they migrated from instead of studying victims and populations in the U.S. Because of local religious practices, the Hmong men in Ban Vinai did not receive autopsies. Therefore, the only results and research obtained were relating to deaths outside of the local religion or geographical area. [2]

An interview was arranged with the next of kin who lived with them, witnessed the death, or found the body. The interviews were open ended and allowed the person who was next of kin to describe what they witnessed and what preceding events they thought were relevant to the victim's death. The interviewers also collected information such as illness history, the circumstances of the death, demographic background, and history of any sleep disturbances. A genealogy was then created which included all the relatives and their vital status and/or circumstances of death. [2]

Society, culture, superstition

During the 1970s and 1980s, when an outbreak of this syndrome began, many Southeast Asians were not able to worship properly due to the Laotian Civil War. Hmong people believe that when they do not worship properly, do not perform religious rituals properly or forget to sacrifice, the ancestor spirits or the village spirits do not protect them, thus allowing evil spirits to reach them. These attacks induce a nightmare that leads to sleep paralysis, in which the victim is conscious and experiencing pressure on the chest. [16] It is also common to have a REM state that is out of sequence, where there is a mix of brain states that are normally held separate. [16] After the war, the United States government scattered the Hmong refugees across the U.S. in 53 different cities. [16] Once these nightmare visitations began, a shaman was recommended for psychic protection from the spirits of their sleep. [16] However, scattered across 53 different cities, these victims had no access to a shaman who could protect them.

Hmong people believed that rejecting the role of becoming a shaman, they are taken into the spirit world.[ clarification needed ]

The study author suggested that the Hmong who died were killed by their own beliefs in the spiritual world, otherwise known as Nocturnal pressing spirit attacks. In Indonesia it is called digeuton, which translates to "pressed on" in English. [16] In China it is called bèi guǐ yā (traditional Chinese :被鬼壓; simplified Chinese :被鬼压) which translates to "crushed by a ghost" in English. [16] The Dutch call the presence a nachtmerrie, the night-mare. [16] The "merrie" comes from the Middle Dutch mare, an incubus who "lies on people's chests, suffocating them". This phenomenon is known among the Hmong people of Laos, [19] who ascribe these deaths to a malign spirit, dab tsuam (pronounced "dah chua"), said to take the form of a jealous woman.

Bangungot is depicted in the Philippines as a mythological creature called batibat or bangungot. [20] This hag-like creature sits on the victim's face or chest so as to immobilize and suffocate him. When this occurs, the victim is usually experiencing sleep paralysis.

This phenomenon inspired the Nightmare On Elm Street series, according to interviews with creator Wes Craven.

English names

NameAcronymNotes
sudden unexpected death syndromeSUDS
sudden unexplained death syndromeSUDS
sudden unexpected nocturnal death syndromeSUNDS
sudden unexplained nocturnal death syndromeSUNDS
sudden adult death syndromeSADS(parallel in form with SIDS)
sudden arrhythmia death syndromeSADS
sudden arrhythmic death syndromeSADS
sudden arrhythmic cardiac death syndrome—
bed death—

Names in other languages

TermLanguageNotes
lai taiThai Thai : ใหลตาย; meaning "sleep and die" [17] [21]
bangungot or uromFilipinoThe term originated from the Tagalog word meaning "to rise and moan in sleep". [22] It is also the Tagalog word for nightmare.
dab tsogHmong [19] dab tsog means "ghost"
dolyeonsaKorean Korean : 돌연사; meaning literally "sudden death"
pokkuri diseaseJapanese [23]
jathoom[ citation needed ]Arabicجاثوم
albarsty (Kyrgyz : албарсты)Kyrgyz

See also

Related Research Articles

<span class="mw-page-title-main">Hmong people</span> Ethnic group in southwest China and Southeast Asia

The Hmong people are an indigenous group in Southeast Asia and East Asia. In China, the Hmong people are classified as a sub-group of the Miao people. The modern Hmong reside mainly in Southwest China and countries in Southeast Asia such as Vietnam, Laos, Thailand, and Myanmar. There is also a large diasporic community in the United States of more than 300,000. The Hmong diaspora has smaller communities in Australia and South America.

<span class="mw-page-title-main">Cardiac arrest</span> Sudden failure of heart beat

Cardiac arrest, also known as sudden cardiac arrest, is when the heart suddenly and unexpectedly stops beating. As a result, blood cannot properly circulate around the body and there is diminished blood flow to the brain and other organs. When the brain does not receive enough blood, this can cause a person to lose consciousness. Coma and persistent vegetative state may result from cardiac arrest. Cardiac arrest is also identified by a lack of central pulses and abnormal or absent breathing.

<span class="mw-page-title-main">SIDS</span> Sudden unexplained death of a child who is less than one year of age

Sudden infant death syndrome (SIDS), sometimes known as cot death, is the sudden unexplained death of a child of less than one year of age. Diagnosis requires that the death remain unexplained even after a thorough autopsy and detailed death scene investigation. SIDS usually occurs during sleep. Typically death occurs between the hours of midnight and 9:00 a.m. There is usually no noise or evidence of struggle. SIDS remains the leading cause of infant mortality in Western countries, constituting half of all post-neonatal deaths.

<span class="mw-page-title-main">Brugada syndrome</span> Heart conduction disease

Brugada syndrome (BrS) is a genetic disorder in which the electrical activity of the heart is abnormal due to channelopathy. It increases the risk of abnormal heart rhythms and sudden cardiac death. Those affected may have episodes of syncope. The abnormal heart rhythms seen in those with Brugada syndrome often occur at rest. They may be triggered by a fever.

<span class="mw-page-title-main">Ventricular fibrillation</span> Rapid quivering of the ventricles of the heart

Ventricular fibrillation is an abnormal heart rhythm in which the ventricles of the heart quiver. It is due to disorganized electrical activity. Ventricular fibrillation results in cardiac arrest with loss of consciousness and no pulse. This is followed by sudden cardiac death in the absence of treatment. Ventricular fibrillation is initially found in about 10% of people with cardiac arrest.

<span class="mw-page-title-main">Long QT syndrome</span> Medical condition

Long QT syndrome (LQTS) is a condition affecting repolarization (relaxing) of the heart after a heartbeat, giving rise to an abnormally lengthy QT interval. It results in an increased risk of an irregular heartbeat which can result in fainting, drowning, seizures, or sudden death. These episodes can be triggered by exercise or stress. Some rare forms of LQTS are associated with other symptoms and signs including deafness and periods of muscle weakness.

<span class="mw-page-title-main">Short QT syndrome</span> Medical condition

Short QT syndrome (SQT) is a very rare genetic disease of the electrical system of the heart, and is associated with an increased risk of abnormal heart rhythms and sudden cardiac death. The syndrome gets its name from a characteristic feature seen on an electrocardiogram (ECG) – a shortening of the QT interval. It is caused by mutations in genes encoding ion channels that shorten the cardiac action potential, and appears to be inherited in an autosomal dominant pattern. The condition is diagnosed using a 12-lead ECG. Short QT syndrome can be treated using an implantable cardioverter-defibrillator or medications including quinidine. Short QT syndrome was first described in 2000, and the first genetic mutation associated with the condition was identified in 2004.

<span class="mw-page-title-main">Ventricular tachycardia</span> Medical condition of the heart

Ventricular tachycardia is a cardiovascular disorder in which fast heart rate occurs in the ventricles of the heart. Although a few seconds of VT may not result in permanent problems, longer periods are dangerous; and multiple episodes over a short period of time are referred to as an electrical storm. Short periods may occur without symptoms, or present with lightheadedness, palpitations, shortness of breath, chest pain, and decreased level of consciousness. Ventricular tachycardia may lead to coma and persistent vegetative state due to lack of blood and oxygen to the brain. Ventricular tachycardia may result in ventricular fibrillation (VF) and turn into cardiac arrest. This conversion of the VT into VF is called the degeneration of the VT. It is found initially in about 7% of people in cardiac arrest.

SCN5A Protein-coding gene in the species Homo sapiens

Sodium channel protein type 5 subunit alpha, also known as NaV1.5 is an integral membrane protein and tetrodotoxin-resistant voltage-gated sodium channel subunit. NaV1.5 is found primarily in cardiac muscle, where it mediates the fast influx of Na+-ions (INa) across the cell membrane, resulting in the fast depolarization phase of the cardiac action potential. As such, it plays a major role in impulse propagation through the heart. A vast number of cardiac diseases is associated with mutations in NaV1.5 (see paragraph genetics). SCN5A is the gene that encodes the cardiac sodium channel NaV1.5.

<span class="mw-page-title-main">Ajmaline</span> Chemical compound

Ajmaline is an alkaloid that is classified as a 1-A antiarrhythmic agent. It is often used to induce arrhythmic contraction in patients suspected of having Brugada syndrome. Individuals suffering from Brugada syndrome will be more susceptible to the arrhythmogenic effects of the drug, and this can be observed on an electrocardiogram as an ST elevation.

<span class="mw-page-title-main">Catecholaminergic polymorphic ventricular tachycardia</span> Medical condition

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited genetic disorder that predisposes those affected to potentially life-threatening abnormal heart rhythms or arrhythmias. The arrhythmias seen in CPVT typically occur during exercise or at times of emotional stress, and classically take the form of bidirectional ventricular tachycardia or ventricular fibrillation. Those affected may be asymptomatic, but they may also experience blackouts or even sudden cardiac death.

<span class="mw-page-title-main">Cardiac Risk in the Young</span>

Cardiac Risk in the Young (CRY) is a humanitarian charitable organisation helping to raise awareness of young sudden cardiac death, including sudden arrhythmic death syndrome. CRY was established in May 1995 by Alison Cox MBE and is based in the United Kingdom.

<span class="mw-page-title-main">Syncope (medicine)</span> Transient loss of consciousness and postural tone

Syncope, commonly known as fainting or passing out, is a loss of consciousness and muscle strength characterized by a fast onset, short duration, and spontaneous recovery. It is caused by a decrease in blood flow to the brain, typically from low blood pressure. There are sometimes symptoms before the loss of consciousness such as lightheadedness, sweating, pale skin, blurred vision, nausea, vomiting, or feeling warm. Syncope may also be associated with a short episode of muscle twitching. Psychiatric causes can also be determined when a patient experiences fear, anxiety, or panic; particularly before a stressful event, usually medical in nature. When consciousness and muscle strength are not completely lost, it is called presyncope. It is recommended that presyncope be treated the same as syncope.

<span class="mw-page-title-main">Arrhythmia</span> Group of medical conditions characterized by irregular heartbeat

Arrhythmias, also known as cardiac arrhythmias, heart arrhythmias, or dysrhythmias, are irregularities in the heartbeat, including when it is too fast or too slow. A resting heart rate that is too fast – above 100 beats per minute in adults – is called tachycardia, and a resting heart rate that is too slow – below 60 beats per minute – is called bradycardia. Some types of arrhythmias have no symptoms. Symptoms, when present, may include palpitations or feeling a pause between heartbeats. In more serious cases, there may be lightheadedness, passing out, shortness of breath, chest pain, or decreased level of consciousness. While most cases of arrhythmia are not serious, some predispose a person to complications such as stroke or heart failure. Others may result in sudden death.

Sudden unexplained death in childhood (SUDC) is the death of a child over the age of 12 months which remains unexplained after a thorough investigation and autopsy. There has not been enough research to identify risk factors, common characteristics, or prevention strategies for SUDC.

Masonic Medical Research Institute (MMRI) is a non-profit medical research center located in Utica, New York. The Institute's research and staff are independent, but gets its name from its original funding in 1958 by the Masonic Grand Lodge of New York.

<span class="mw-page-title-main">Molecular autopsy</span>

Molecular autopsy or postmortem molecular testing is a set of molecular techniques used in forensic medicine to attempt to determine the cause of death in unexplained cases, in particular sudden unexplained deaths. About 30% of sudden cardiac deaths in young people are not explained after full conventional autopsy, and are classified as sudden unexplained deaths. The use of a panel of genetic markers for long QT syndrome, catecholaminergic polymorphic ventricular tachycardia and cardiac channel miopathies elucidated around 40 to 45% of the cases.

<span class="mw-page-title-main">Charles Antzelevitch</span> American cardiovascular research scientist

Charles Antzelevitch is an American cardiovascular research scientist in the fields of cardiac electrophysiology and cardiac arrhythmia syndromes.

Sudden unexplained nocturnal death syndrome may refer to:

<span class="mw-page-title-main">Sports cardiology</span>

Sports cardiology is an emerging subspecialty field of Cardiology. It may also be considered a subspecialty field of Sports medicine, or alternatively a hybrid subspecialty that spans cardiology and sports medicine. Emergency medicine is another medical specialty that has some overlap with Sports Cardiology. Sports cardiology is now considered to be a distinct subspecialty in Europe and the USA, with a core curriculum developed in both regions. In Europe it has traditionally been grouped under Preventive Cardiology, but the subspecialty of Sports Cardiology is now considered a distinct field. In the USA, it has developed from being a special interest area to a distinct subspecialty as well.

References

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  4. 1 2 "Dead in bed syndrome - a term used to describe the sudden unexplained deaths of young people with type 1 diabetes". Diabetes. 10 June 2022 [Originally written 15 January 2019].
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  6. ↑ Also known as SUDS. See: Reddy PR, Reinier K, Singh T, Mariani R, Gunson K, Jui J, Chugh SS. Physical activity as a trigger of sudden cardiac arrest: The Oregon Sudden Unexpected Death Study. Int J Cardiol. 2008
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  8. ↑ "Brugada syndrome". Genetics Home Reference. Retrieved 28 October 2017.
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  20. ↑ "BATIBAT | BANGUNGOT - Frightened To Death By Nightmares • THE ASWANG PROJECT". THE ASWANG PROJECT. 16 February 2016. Retrieved 17 February 2021.
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  22. ↑ Munger, Ronald G.; Booton, Elizabeth A. (1998). "Bangungut in Manila: sudden and unexplained death in sleep of adult Filipinos" (PDF). International Journal of Epidemiology. 27 (4): 677–684. doi: 10.1093/ije/27.4.677 . PMID   9758125. Archived from the original (PDF) on 30 September 2015.
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