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[2, 3].
- There may be a familial tendency to Stokes-Adams attacks. This was first recognised by William Osler in 1903 within his own family.
With congenital heart block, it has been described as being precipitated by bradycardia or tachycardia.
- 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, reactive arthritis, rheumatoid arthritis, scleroderma.
- Infiltrative processes including amyloidosis, sarcoidosis, tumours, Hodgkin's disease, multiple myeloma.
- Stokes-Adams attacks have been described as due to:
- Chronic or paroxysmal AV block in 50-60% of patients.
- Sino-atrial (SA) block in 30-40% of patients.
- Paroxysmal supraventricular tachycardia or atrial fibrillation in up to 5% of patients.
- 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. (The separate article ECG Identification of Conduction Disorders describes a complete heart block in more detail.)
- Attacks can happen a number of times in one day.
- They are not posture-related.
See the separate Syncope article, 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: establish whether medication might 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 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 and references
Transient loss of consciousness ('blackouts') management in adults and young people; NICE Clinical Guideline (August 2010, updated September 2014).
Guidelines on Diagnosis and Management of Syncope; European Society of Cardiology (2009)
Harbison J, Newton JL, Seifer C, et al; Stokes Adams attacks and cardiovascular syncope. Lancet. 2002 Jan 12359(9301):158-60.
Carano N, Bo I, Tchana B, et al; Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature. Ital J Pediatr. 2012 Oct 3038:61. doi: 10.1186/1824-7288-38-61.
Yildirim A, Tunaoolu FS, Karaaoac AT; Neonatal congenital heart block. Indian Pediatr. 2013 May 850(5):483-8.
Wooley CF, Bliss M; William Osler: slow pulse, stokes-adams disease, and sudden death in families. Am Heart Hosp J. 2006 Winter
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)
Assessing fitness to drive: guide for medical professionals; Driver and Vehicle Licensing Agency