Stokes–Adams syndrome or Adams–Stokes syndrome is a periodic
fainting spell in which there is intermittent complete heart block or other high-grade arrhythmia that results in loss of spontaneous circulation and inadequate blood flow to the brain. Subsequently, named after two Irish physicians,
Robert Adams (1791–1875) and
William Stokes (1804–1877), the first description of the syndrome is believed to have been published in 1717 by the
Carniolan physician of
Slovene descent
Marko Gerbec. It is characterized by an abrupt decrease in cardiac output and loss of consciousness due to a transient arrhythmia; for example, bradycardia due to
complete heart block
Third-degree atrioventricular block (AV block) is a medical condition in which the electrical impulse generated in the sinoatrial node (SA node) in the atrium of the heart can not propagate to the ventricles.
Because the impulse is blocked, an ...
.
Signs and symptoms
Typically an attack occurs without warning, leading to sudden loss of consciousness. Prior to an attack, a patient may be pale with hypoperfusion. Abnormal movements may be present, typically consisting of twitching after 15–20 seconds of unconsciousness. (These movements, which are not seizures, occur because of brainstem hypoxia and not due to cortical discharge as is the case for epileptiform seizures).
Breathing typically continues normally throughout the attack, and, upon recovery, the patient becomes flushed as the heart rapidly pumps the oxygenated blood from the
pulmonary beds into the systemic circulation, which has become dilated due to hypoxia.
As with any syncopal episode that results from a cardiac dysrhythmia, the fainting does not depend on the patient's position. If it occurs during sleep, the presenting symptom may simply be feeling hot and flushed on waking.
Causes
The attacks are caused by any temporary lack of
cardiac output caused by a transient abnormal heart rhythm.
Paroxysmal supraventricular tachycardia
Paroxysmal supraventricular tachycardia (PSVT) is a type of supraventricular tachycardia, named for its intermittent episodes of abrupt onset and termination. Often people have no symptoms. Otherwise symptoms may include palpitations, increased h ...
or atrial fibrillation has been reported as the underlying cause in up to 5% of patients in one series. The resulting lack of blood flow to the
brain
A brain is an organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It is located in the head, usually close to the sensory organs for senses such as vision. It is the most complex organ in a ve ...
is responsible for the loss of consciousness and associated fainting episode.
Diagnosis
Stokes–Adams attacks may be diagnosed from the
history
History (derived ) is the systematic study and the documentation of the human activity. The time period of event before the invention of writing systems is considered prehistory. "History" is an umbrella term comprising past events as well ...
, with paleness prior to the attack and flushing after it particularly characteristic. The
ECG will show complete heart block, high grade AV block, or other malignant arrhythmia during the attacks.
Torsades de Pointes
''Torsades de pointes, torsade de pointes'' or ''torsades des pointes'' (TdP) (, , translated as "twisting of peaks") is a specific type of abnormal heart rhythm that can lead to sudden cardiac death. It is a polymorphic ventricular tachycardia t ...
can occur in a heart block setting.
Treatment
Initial treatment can be medical, involving the use of drugs like
isoprenaline
Isoprenaline, or isoproterenol (brand name: Isoprenaline Macure), is a medication used for the treatment of bradycardia (slow heart rate), heart block, and rarely for asthma. It is a non-selective β adrenoceptor agonist that is the isopropyla ...
(
Isuprel) and
epinephrine (adrenaline). Temporary cardiac pacing may also be used in a closely monitored setting. However, definitive treatment includes the insertion of a permanent cardiac
pacemaker.
[Chart 63: ''Faintness and Fainting'', page 161, ]
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.
References
External links
{{DEFAULTSORT:Adams-Stokes Syndrome
Cardiac arrhythmia
Syndromes