Synonyms: Adams-Stokes, Morgagni, Morgagni-Adams-Stokes and Spens' syndrome
A classic Stokes-Adams attack is a collapse without warning, associated with loss of consciousness for a few seconds. Typically, complete (third-degree) heart block is seen on the ECG during an attack (but other ECG abnormalities such as tachy-brady syndrome have been reported).
Cardiologists, and other doctors specialising in syncope, do not use the term 'Stokes-Adams attack' as often these days. The development of investigation techniques and improvements in the understanding of the physiology of the cardiovascular system have meant that there has been a move away from clinical diagnoses to a more rigid diagnostic classification.
- The condition is usually associated with ischaemic heart disease and so tends to occur in the elderly.
- Stokes-Adams attacks have been reported in much younger age groups, including those with congenital heart block.
- There may be a familial tendency to Stokes-Adams attacks. This was first recognised by William Osler in 1903, within his own family.
- Heart block may result from:
- Myocardial infarction.
- Fibrosis (usually associated with ischaemia).
- Atrioventricular (AV) nodal disease.
- Structural or valvular heart disease.
- Electrolyte disturbance.
- Rheumatic diseases including ankylosing spondylitis, Reiter's syndrome, rheumatoid arthritis, scleroderma.
- Infiltrative processes including amyloidosis, sarcoidosis, tumours, Hodgkin's disease, multiple myeloma.
- Stokes-Adams attacks have been described as due to:
- There is collapse, usually without warning.
- Loss of consciousness is usually between about 10 and 30 seconds.
- Pallor, followed by flushing on recovery, can be reported.
- Some seizure-like activity sometimes occurs if the attack is prolonged.
- If anyone manages to check the pulse during an episode, it will be slow, usually less than 40 beats per minute.
- Recovery is fairly rapid, although the patient may be confused for a while afterwards.
- Typically, complete (third-degree) heart block is seen on the ECG during an attack but other ECG abnormalities such as tachy-brady syndrome have been reported. (A separate article ECG Identification of Conduction Disorders describes complete heart block in more detail.)
- Attacks can happen a number of times in one day.
- They are not posture-related.
See separate article Syncope, which details the assessment of a patient with a syncopal episode. Briefly, this should include:
- History of other episodes.
- Past medical history, including history of heart disease.
- Drug history: could medication be contributing?
- Blood pressure examination (supine and standing).
- Cardiovascular examination.
- 12-lead ECG: this may be normal by the time the patient is seen or may show heart block or ischaemic changes; 24-hour ECG may show changes during attacks.
- Routine haematological and biochemical investigations.
- If underlying heart disease is suspected, this should be investigated appropriately.
- If seizure activity has been witnessed, the possibility of epilepsy should be investigated.
This is the differential diagnosis of syncope (further detailed in the separate Syncope article) and includes the following:
- Epilepsy (if convulsions occur).
- Vasovagal fainting.
- Carotid sinus hypersensitivity.
- Orthostatic hypotension.
- A fast tachyarrhythmia (may also reduce cardiac output but does not usually have the same brief but dramatic effect).
- Drop attacks.
- Transient ischaemic attack.
- Syncope due to hypoperfusion, eg due to hypovolaemia.
- Reversible causes such as drug toxicity should be addressed.
- Underlying heart disease should be managed appropriately.
- A cardiac pacemaker may be required.
Driving and other activities
- If a person is susceptible to syncope with little or no warning then driving must be forbidden, at least until a diagnosis is made and a pacemaker is working well.
- Other behaviours in which sudden loss of consciousness may pose a risk also need to be addressed. These may include cycling, swimming and operating machinery.
- William Stokes (1804-1877) and Robert Adams (1791-1875) were both Irish physicians.
- Adams' description of syncope associated with bradycardia dates back to 1827 and Stokes described the same association in 1846. (Stokes is also remembered for Cheyne-Stokes breathing.)
- Thomas Spens (1764-1842), a Scottish physician, also described a similar syndrome.
Further reading & references
- Transient loss of consciousness in adults and young people, NICE Clinical Guideline (August 2010)
- Guidelines for the diagnosis and management of syncope (version 2009): The Task Force for the Diagnosis and Management of Syncope of the European Society of Cardiology (ESC), Eur Heart J. 2009 Aug 27
- Harbison J, Newton JL, Seifer C, et al; Stokes Adams attacks and cardiovascular syncope. Lancet. 2002 Jan 12;359(9301):158-60.
- Sigurd B, Sandoe E; Management of Stokes-Adams syndrome.; Cardiology. 1990;77(3):195-208.
- Wooley CF, Bliss M; William Osler: slow pulse, stokes-adams disease, and sudden death in families.; Am Heart Hosp J. 2006 Winter;4(1):60-5.
- Pearl W; Stokes-Adams attacks in congenital complete heart block.; Pediatr Cardiol. 1988;9(2):125-6.
- Alaeddini J et al, First-Degree Atrioventricular Block, Medscape, Jun 2011
- Sovari AA et al, Second-Degree Atrioventricular Block, Medscape, Sep 2011
- Levine M et al, Heart Block, Third Degree, eMedicine, Jul 2010
- ACC/AHA/NASPE Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices, American College of Cardiology/American Heart Association Task Force on Practice Guidelines (2002)
- At a Glance Guide to the Current Medical Standards of Fitness to Drive, Driver and Vehicle Licensing Agency
- Adams R; Cases of disease of the heart, accompanied with pathological observations. Dublin Hospital Reports 1827; 4:353-453
- Stokes W; Observations in some cases of permanently slow pulses. Dublin Quarterly Journal Medical Science 1846; 2:73-85
|Original Author: Dr Michelle Wright||Current Version: Dr Hayley Willacy|
|Last Checked: 18/03/2011||Document ID: 1119 Version: 23||© EMIS|
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