Adams–Stokes Syndrome
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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
Carniola Carniola ( sl, Kranjska; , german: Krain; it, Carniola; hu, Krajna) is a historical region that comprised parts of present-day Slovenia. Although as a whole it does not exist anymore, Slovenes living within the former borders of the region sti ...
n 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 a ...
.


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 Breathing (or ventilation) is the process of moving air into and from the lungs to facilitate gas exchange with the internal environment, mostly to flush out carbon dioxide and bring in oxygen. All aerobic creatures need oxygen for cellular ...
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 In cardiac physiology, cardiac output (CO), also known as heart output and often denoted by the symbols Q, \dot Q, or \dot Q_ , edited by Catherine E. Williamson, Phillip Bennett is the volumetric flow rate of the heart's pumping output: t ...
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 he ...
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 v ...
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 History of writing#Inventions of writing, invention of writing systems is considered prehistory. "History" is an umbr ...
, with paleness prior to the attack and flushing after it particularly characteristic. The
ECG Electrocardiography is the process of producing an electrocardiogram (ECG or EKG), a recording of the heart's electrical activity. It is an electrogram of the heart which is a graph of voltage versus time of the electrical activity of the hear ...
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 isopropylam ...
(
Isuprel 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 ...
) and
epinephrine Adrenaline, also known as epinephrine, is a hormone and medication which is involved in regulating visceral functions (e.g., respiration). It appears as a white microcrystalline granule. Adrenaline is normally produced by the adrenal glands and ...
(adrenaline). Temporary cardiac pacing may also be used in a closely monitored setting. However, definitive treatment includes the insertion of a permanent cardiac
pacemaker An artificial cardiac pacemaker (or artificial pacemaker, so as not to be confused with the natural cardiac pacemaker) or pacemaker is a medical device that generates electrical impulses delivered by electrodes to the chambers of the heart eith ...
.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