Adams–Stokes syndrome

Stokes-Adams syndrome
Other names	Adams–Stokes syndrome,Gerbezius–Morgagni–Adams–Stokes syndromeandGerbec–Morgagni–Adams–Stokes syndrome
Specialty	Cardiology

Adams–Stokes syndrome,Stokes–Adams syndromeorGerbec–Morgagni–Adams–Stokes syndromeis a periodicfainting spellin which there is intermittent completeheart blockor other high-gradearrhythmiathat results in loss of spontaneous circulation and inadequate blood flow to the brain. Subsequently, named after two Irish physicians,Robert Adams(1791–1875)andWilliam Stokes(1804–1877),the first description of the syndrome is believed to have been published in 1717 by theCarniolanphysician ofSlovenedescentMarko Gerbec.It is characterized by an abrupt decrease in cardiac output and loss of consciousness due to a transient arrhythmia; for example,bradycardiadue tocomplete heart block.

Signs and symptoms

Typically an attack occurs without warning, leading to suddenloss 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).Breathingtypically continues normally throughout the attack, and, upon recovery, the patient becomes flushed as the heart rapidly pumps the oxygenated blood from thepulmonary bedsinto 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 ofcardiac outputcaused by a transient abnormal heart rhythm.Paroxysmal supraventricular tachycardiaor 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 thebrainis responsible for the loss of consciousness and associated fainting episode.

Diagnosis

Stokes–Adams attacks may be diagnosed from thehistory,with paleness prior to the attack and flushing after it particularly characteristic. TheECGwill show complete heart block, high grade AV block, or other malignant arrhythmia during the attacks.Torsades de Pointescan occur in a heart block setting.

Treatment

Initial treatment can be medical, involving the use of drugs likeisoprenaline(Europe) or isoproterenol (US/Canada) (Isuprel) andepinephrine(adrenaline). Temporary cardiac pacing may also be used in a closely monitored setting. However, definitive treatment includes the insertion of a permanent cardiacpacemaker.