Adams-Stokes syndrome overview: Difference between revisions
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== Overview == | ==Overview== | ||
Adams-Stokes syndrome is a sudden collapse into [[unconsciousness]] due to a disorder of [[heart rhythm]] causing heart blocks in which there is a slow or absent pulse resulting in decreased blood flow to the brain causing [[syncope]] (fainting) with or without [[convulsions]]. High grade arrhythmias often lead to abrupt decrease in cardiac output and transient loss of consciousness. | Adams-Stokes syndrome is a sudden collapse into [[unconsciousness]] due to a disorder of [[heart rhythm]] causing heart blocks in which there is a slow or absent pulse resulting in decreased blood flow to the brain causing [[syncope]] (fainting) with or without [[convulsions]]. High grade arrhythmias often lead to abrupt decrease in cardiac output and transient loss of consciousness. | ||
==Historical Perspective== | ==Historical Perspective== | ||
* It is named after two Irish physicians, [[Robert Adams (physician)|Robert Adams]] (1791–1875)<ref>R. Adams. Cases of Diseases of the Heart, Accompanied with Pathological Observations. Dublin Hospital Reports, 1827, 4: 353-453. </ref> and [[William Stokes]] (1804–1877).<ref>W. Stokes. Observations on some cases of permanently slow pulse. Dublin Quarterly Journal of Medical Science, 1846, 2: 73-85.</ref> | |||
* It was first described by [[Marcus Gerbezius]] and referenced by Morgagni later in 1761 with his two cases. | *It is named after two Irish physicians, [[Robert Adams (physician)|Robert Adams]] (1791–1875)<ref>R. Adams. Cases of Diseases of the Heart, Accompanied with Pathological Observations. Dublin Hospital Reports, 1827, 4: 353-453. </ref> and [[William Stokes]] (1804–1877).<ref>W. Stokes. Observations on some cases of permanently slow pulse. Dublin Quarterly Journal of Medical Science, 1846, 2: 73-85.</ref> | ||
*It was first described by [[Marcus Gerbezius]] and referenced by Morgagni later in 1761 with his two cases. | |||
==Classification== | ==Classification== | ||
| Line 15: | Line 16: | ||
==Pathophysiology== | ==Pathophysiology== | ||
* The attacks are caused by loss of [[cardiac output]] due to cardiac [[asystole]], [[heart block]], or [[ventricular fibrillation]]. The resulting lack of blood flow to the [[brain]] is responsible for the [[syncope]]. | |||
*The attacks are caused by loss of [[cardiac output]] due to cardiac [[asystole]], [[heart block]], or [[ventricular fibrillation]]. The resulting lack of blood flow to the [[brain]] is responsible for the [[syncope]]. | |||
==Causes== | ==Causes== | ||
* Causes of Adams-Stokes syndrome include [[asystole]], [[heart block]], or [[ventricular fibrillation]]. | |||
* Paroxysmal supraventricular tachycardia or atrial fibrillation has been reported as the underlying cause in up to 5% of patients in one series. | *Causes of Adams-Stokes syndrome include [[asystole]], [[heart block]], or [[ventricular fibrillation]]. | ||
*Paroxysmal supraventricular tachycardia or atrial fibrillation has been reported as the underlying cause in up to 5% of patients in one series. | |||
==[[Adam Stokes Syndrome differential diagnosis|Differentiating Any Disease from other Diseases]]== | ==[[Adam Stokes Syndrome differential diagnosis|Differentiating Any Disease from other Diseases]]== | ||
Adam Stokes Syndrome should be differentiated from other causes of [[syncope]]. These include: | Adam Stokes Syndrome should be differentiated from other causes of [[syncope]]. These include: | ||
* [[Vasovagal Fainting]] | |||
* [[Orthostatic hypotension]] | *[[Vasovagal Fainting]] | ||
* [[Carotid Sinus Hypersensitivity]] | *[[Orthostatic hypotension]] | ||
* [[Syncope]] due to hypo-perfusion | *[[Carotid Sinus Hypersensitivity]] | ||
* [[Epilepsy]] <ref name="pmid14122675">{{cite journal| author=IRA GH, FLOYD WL, ORGAIN ES| title=SYNCOPE WITH COMPLETE HEART BLOCK. DIFFERENTIATION OF REAL AND SIMULATED ADAMS-STOKES SEIZURES BY RADIOTELEMETRY. | journal=JAMA | year= 1964 | volume= 188 | issue= | pages= 707-10 | pmid=14122675 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14122675 }} </ref> | *[[Syncope]] due to hypo-perfusion | ||
*[[Epilepsy]] <ref name="pmid14122675">{{cite journal| author=IRA GH, FLOYD WL, ORGAIN ES| title=SYNCOPE WITH COMPLETE HEART BLOCK. DIFFERENTIATION OF REAL AND SIMULATED ADAMS-STOKES SEIZURES BY RADIOTELEMETRY. | journal=JAMA | year= 1964 | volume= 188 | issue= | pages= 707-10 | pmid=14122675 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14122675 }} </ref> | |||
==Screening== | ==Screening== | ||
*There is insufficient evidence to recommend routine [[screening]] for Adam Stokes Syndrome. | *There is insufficient evidence to recommend routine [[screening]] for Adam Stokes Syndrome. | ||
==[[Adam Stokes Syndrome epidemiology and demographics|Epidemiology and Demographics]]== | |||
*The syndrome was first described and published in 1717 by [[Marco Gerbec]]. | |||
* The syndrome was first described and published in 1717 by [[Marco Gerbec]]. | |||
==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
* If undiagnosed (or untreated), Stokes-Adams attacks have a 50% mortality within a year of the first episode. | |||
* The prognosis following treatment is very good.<ref name="pmid32595044">{{cite journal| author=| title=Correction to Lancet Infectious Diseases 2020; published online April 29. https://doi.org/10.1016/ S1473-3099(20)30064-5. | journal=Lancet Infect Dis | year= 2020 | volume= 20 | issue= 7 | pages= e148 | pmid=32595044 | doi=10.1016/S1473-3099(20)30370-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32595044 }} </ref> | *If undiagnosed (or untreated), Stokes-Adams attacks have a 50% mortality within a year of the first episode. | ||
* However, if attacks occur after a myocardial infarction, the prognosis is bad.<ref name="pmid32595044">{{cite journal| author=| title=Correction to Lancet Infectious Diseases 2020; published online April 29. https://doi.org/10.1016/ S1473-3099(20)30064-5. | journal=Lancet Infect Dis | year= 2020 | volume= 20 | issue= 7 | pages= e148 | pmid=32595044 | doi=10.1016/S1473-3099(20)30370-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32595044 }} </ref> | *The prognosis following treatment is very good.<ref name="pmid32595044">{{cite journal| author=| title=Correction to Lancet Infectious Diseases 2020; published online April 29. https://doi.org/10.1016/ S1473-3099(20)30064-5. | journal=Lancet Infect Dis | year= 2020 | volume= 20 | issue= 7 | pages= e148 | pmid=32595044 | doi=10.1016/S1473-3099(20)30370-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32595044 }} </ref> | ||
*However, if attacks occur after a myocardial infarction, the prognosis is bad.<ref name="pmid32595044">{{cite journal| author=| title=Correction to Lancet Infectious Diseases 2020; published online April 29. https://doi.org/10.1016/ S1473-3099(20)30064-5. | journal=Lancet Infect Dis | year= 2020 | volume= 20 | issue= 7 | pages= e148 | pmid=32595044 | doi=10.1016/S1473-3099(20)30370-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32595044 }} </ref> | |||
==Diagnosis== | ==Diagnosis== | ||
===History and Symptoms=== | ===History and Symptoms=== | ||
* Stokes-Adams attacks may be diagnosed from the [[Medical history|history]], with paleness prior to the attack and flushing after recovery is particularly characteristic. | |||
* Loss of consciousness. | *Stokes-Adams attacks may be diagnosed from the [[Medical history|history]], with paleness prior to the attack and flushing after recovery is particularly characteristic. | ||
* If the attack is prolonged, it may lead to seizure-like-activity. | *Loss of consciousness. | ||
*If the attack is prolonged, it may lead to seizure-like-activity. | |||
===Physical Examination=== | ===Physical Examination=== | ||
* Prior to an attack, the patient may become pale, their heart rhythm experiences a temporary pause, and collapse may follow. | |||
* Normal periods of unconsciousness last approximately thirty seconds; if seizures are present, they will consist of twitching after 15–20 seconds. | *Prior to an attack, the patient may become pale, their heart rhythm experiences a temporary pause, and collapse may follow. | ||
*[[Respiratory system|Breathing]] continues normally throughout the attack, and so on recovery the patient becomes flushed as the heart rapidly pumps the oxygenated blood from the [[pulmonary circulation|pulmonary beds]] into a systemic circulation which has become dilated due to hypoxia.<ref name="Parkland">{{cite book|last = Katz | first = Jason| authorlink = | coauthors = Patel, Chetan| title = Parkland Manual of Inpatient Medicine| publisher = FA Davis| date = 2006| location = Dallas, TX| pages = 903|}}</ref> | *Normal periods of unconsciousness last approximately thirty seconds; if seizures are present, they will consist of twitching after 15–20 seconds. | ||
* As with any syncopal episode that results from a cardiac dysrhythmia, the faints do not depend on the patient's position. | *[[Respiratory system|Breathing]] continues normally throughout the attack, and so on recovery the patient becomes flushed as the heart rapidly pumps the oxygenated blood from the [[pulmonary circulation|pulmonary beds]] into a systemic circulation which has become dilated due to hypoxia.<ref name="Parkland">{{cite book|last = Katz | first = Jason| authorlink = | coauthors = Patel, Chetan| title = Parkland Manual of Inpatient Medicine| publisher = FA Davis| date = 2006| location = Dallas, TX| pages = 903|}}</ref> | ||
* If they occur during sleep, the presenting symptom may simply be feeling hot and flushed on waking.<ref name="Parkland"/> | *As with any syncopal episode that results from a cardiac dysrhythmia, the faints do not depend on the patient's position. | ||
*If they occur during sleep, the presenting symptom may simply be feeling hot and flushed on waking.<ref name="Parkland" /> | |||
===Electrocradiogram=== | ===Electrocradiogram=== | ||
== | *The [[Electrocardiogram|ECG]] will show asystole or [[ventricular fibrillation]] during the attacks. | ||
* | *Typically, complete or third heart block is seen on the [[ECG]] during an attack but other ECG abnormalities such as tachy-brady syndrome have also been reported.<ref name="pmid11809277">{{cite journal| author=Harbison J, Newton JL, Seifer C, Kenny RA| title=Stokes Adams attacks and cardiovascular syncope. | journal=Lancet | year= 2002 | volume= 359 | issue= 9301 | pages= 158-60 | pmid=11809277 | doi=10.1016/s0140-6736(02)07376-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11809277 }} </ref> | ||
*[[Torsades de pointes]] can also been seen.<ref name="pmid28850992">{{cite journal| author=Ernst A, Schlattmann P, Waldfahrer F, Westhofen M| title= | journal=Laryngorhinootologie | year= 2017 | volume= 96 | issue= 8 | pages= 519-521 | pmid=28850992 | doi=10.1055/s-0043-113690 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28850992 }} </ref> | |||
== | ==Echocardiography or Ultrasound== | ||
*Echocardiography may be helpful in the diagnosis of underlying heart disease. | |||
* | |||
== | ==CT scan== | ||
== Other Diagnostic Studies == | *There are no CT scan findings associated with placental aromatase deficiency. | ||
* There are no other diagnostic studies associated with placental aromatase deficiency. | |||
==MRI== | |||
*There are no MRI findings associated with placental aromatase deficiency. | |||
==Other Imaging Findings== | |||
*There are no other imaging findings associated with placental aromatase deficiency. | |||
==Other Diagnostic Studies== | |||
*There are no other diagnostic studies associated with placental aromatase deficiency. | |||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
*Initial treatment can be medical, involving the use of drugs like [[isoproterenol]] ([[Isuprel]])and [[epinephrine]] ([[Adrenaline]]). | *Initial treatment can be medical, involving the use of drugs like [[isoproterenol]] ([[Isuprel]])and [[epinephrine]] ([[Adrenaline]]). | ||
* Most patients who develop tachycardia or bradycardia syndromes are managed with supplementary anti-arrhythmic treatment. | *Most patients who develop tachycardia or bradycardia syndromes are managed with supplementary anti-arrhythmic treatment. | ||
*Long-term anticoagulation is required in some patients. <ref name="pmid2272057">{{cite journal| author=Sigurd B, Sandøe E| title=Management of Stokes-Adams syndrome. | journal=Cardiology | year= 1990 | volume= 77 | issue= 3 | pages= 195-208 | pmid=2272057 | doi=10.1159/000174601 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2272057 }} </ref> | *Long-term anticoagulation is required in some patients. <ref name="pmid2272057">{{cite journal| author=Sigurd B, Sandøe E| title=Management of Stokes-Adams syndrome. | journal=Cardiology | year= 1990 | volume= 77 | issue= 3 | pages= 195-208 | pmid=2272057 | doi=10.1159/000174601 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2272057 }} </ref> | ||
===Surgery=== | ===Surgery=== | ||
*Definitive treatment is [[surgery|surgical]], involving the insertion of a [[artificial pacemaker|pacemaker]] – most likely one with sequential pacing such as a DDI mode as opposed to the older VVI mechanisms. <ref name="Parkland">{{cite book|last = Katz | first = Jason| authorlink = | coauthors = Patel, Chetan| title = Parkland Manual of Inpatient Medicine| publisher = FA Davis| date = 2006| location = Dallas, TX| pages = 903|}}</ref> | *Definitive treatment is [[surgery|surgical]], involving the insertion of a [[artificial pacemaker|pacemaker]] – most likely one with sequential pacing such as a DDI mode as opposed to the older VVI mechanisms. <ref name="Parkland">{{cite book|last = Katz | first = Jason| authorlink = | coauthors = Patel, Chetan| title = Parkland Manual of Inpatient Medicine| publisher = FA Davis| date = 2006| location = Dallas, TX| pages = 903|}}</ref> | ||
== References == | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
{{WH}} | {{WH}} | ||
Revision as of 11:13, 24 June 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Adams-Stokes syndrome is a sudden collapse into unconsciousness due to a disorder of heart rhythm causing heart blocks in which there is a slow or absent pulse resulting in decreased blood flow to the brain causing syncope (fainting) with or without convulsions. High grade arrhythmias often lead to abrupt decrease in cardiac output and transient loss of consciousness.
Historical Perspective
- It is named after two Irish physicians, Robert Adams (1791–1875)[1] and William Stokes (1804–1877).[2]
- It was first described by Marcus Gerbezius and referenced by Morgagni later in 1761 with his two cases.
Classification
- There is no established system for the classification of placental aromatase deficiency.
Pathophysiology
- The attacks are caused by loss of cardiac output due to cardiac asystole, heart block, or ventricular fibrillation. The resulting lack of blood flow to the brain is responsible for the syncope.
Causes
- Causes of Adams-Stokes syndrome include asystole, heart block, or ventricular fibrillation.
- Paroxysmal supraventricular tachycardia or atrial fibrillation has been reported as the underlying cause in up to 5% of patients in one series.
Differentiating Any Disease from other Diseases
Adam Stokes Syndrome should be differentiated from other causes of syncope. These include:
- Vasovagal Fainting
- Orthostatic hypotension
- Carotid Sinus Hypersensitivity
- Syncope due to hypo-perfusion
- Epilepsy [3]
Screening
- There is insufficient evidence to recommend routine screening for Adam Stokes Syndrome.
Epidemiology and Demographics
- The syndrome was first described and published in 1717 by Marco Gerbec.
Natural History, Complications and Prognosis
- If undiagnosed (or untreated), Stokes-Adams attacks have a 50% mortality within a year of the first episode.
- The prognosis following treatment is very good.[4]
- However, if attacks occur after a myocardial infarction, the prognosis is bad.[4]
Diagnosis
History and Symptoms
- Stokes-Adams attacks may be diagnosed from the history, with paleness prior to the attack and flushing after recovery is particularly characteristic.
- Loss of consciousness.
- If the attack is prolonged, it may lead to seizure-like-activity.
Physical Examination
- Prior to an attack, the patient may become pale, their heart rhythm experiences a temporary pause, and collapse may follow.
- Normal periods of unconsciousness last approximately thirty seconds; if seizures are present, they will consist of twitching after 15–20 seconds.
- Breathing continues normally throughout the attack, and so on recovery the patient becomes flushed as the heart rapidly pumps the oxygenated blood from the pulmonary beds into a systemic circulation which has become dilated due to hypoxia.[5]
- As with any syncopal episode that results from a cardiac dysrhythmia, the faints do not depend on the patient's position.
- If they occur during sleep, the presenting symptom may simply be feeling hot and flushed on waking.[5]
Electrocradiogram
- The ECG will show asystole or ventricular fibrillation during the attacks.
- Typically, complete or third heart block is seen on the ECG during an attack but other ECG abnormalities such as tachy-brady syndrome have also been reported.[6]
- Torsades de pointes can also been seen.[7]
Echocardiography or Ultrasound
- Echocardiography may be helpful in the diagnosis of underlying heart disease.
CT scan
- There are no CT scan findings associated with placental aromatase deficiency.
MRI
- There are no MRI findings associated with placental aromatase deficiency.
Other Imaging Findings
- There are no other imaging findings associated with placental aromatase deficiency.
Other Diagnostic Studies
- There are no other diagnostic studies associated with placental aromatase deficiency.
Treatment
Medical Therapy
- Initial treatment can be medical, involving the use of drugs like isoproterenol (Isuprel)and epinephrine (Adrenaline).
- Most patients who develop tachycardia or bradycardia syndromes are managed with supplementary anti-arrhythmic treatment.
- Long-term anticoagulation is required in some patients. [8]
Surgery
- Definitive treatment is surgical, involving the insertion of a pacemaker – most likely one with sequential pacing such as a DDI mode as opposed to the older VVI mechanisms. [5]
References
- ↑ R. Adams. Cases of Diseases of the Heart, Accompanied with Pathological Observations. Dublin Hospital Reports, 1827, 4: 353-453.
- ↑ W. Stokes. Observations on some cases of permanently slow pulse. Dublin Quarterly Journal of Medical Science, 1846, 2: 73-85.
- ↑ IRA GH, FLOYD WL, ORGAIN ES (1964). "SYNCOPE WITH COMPLETE HEART BLOCK. DIFFERENTIATION OF REAL AND SIMULATED ADAMS-STOKES SEIZURES BY RADIOTELEMETRY". JAMA. 188: 707–10. PMID 14122675.
- ↑ 4.0 4.1 "Correction to Lancet Infectious Diseases 2020; published online April 29. https://doi.org/10.1016/ S1473-3099(20)30064-5". Lancet Infect Dis. 20 (7): e148. 2020. doi:10.1016/S1473-3099(20)30370-4. PMID 32595044 Check
|pmid=value (help). External link in|title=(help) - ↑ 5.0 5.1 5.2 Katz, Jason (2006). Parkland Manual of Inpatient Medicine. Dallas, TX: FA Davis. p. 903. Unknown parameter
|coauthors=ignored (help) - ↑ Harbison J, Newton JL, Seifer C, Kenny RA (2002). "Stokes Adams attacks and cardiovascular syncope". Lancet. 359 (9301): 158–60. doi:10.1016/s0140-6736(02)07376-2. PMID 11809277.
- ↑ Ernst A, Schlattmann P, Waldfahrer F, Westhofen M (2017). Laryngorhinootologie. 96 (8): 519–521. doi:10.1055/s-0043-113690. PMID 28850992 https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28850992. Missing or empty
|title=(help) - ↑ Sigurd B, Sandøe E (1990). "Management of Stokes-Adams syndrome". Cardiology. 77 (3): 195–208. doi:10.1159/000174601. PMID 2272057.