Stokes-Adams syndrome

Cardiovascular

Clinicals - History

Fact Explanation
Feeling light headed/ faintness/ dizzy Is due to reduction in cardiac out put as a result of sudden changes in heart rate or rhythm Feeling light headed/ faintness/ dizzy
Is due to reduction in cardiac out put as a result of sudden changes in heart rate or rhythm
Loss of consciousness Manifests when blood flow to brain is reduced due to sudden but pronounced decrease in the cardiac output, which is caused by a sudden change in
the heart rate or rhythm. Partial or complete heart block is the usually seen in these patients but other arrhythmias such as ventricular tachycardia or ventricular fibrillation may also cause syncope. These attacks are usually transient lasting only for seconds, not related to posture and can occur several times during a day
Loss of consciousness
Manifests when blood flow to brain is reduced due to sudden but pronounced decrease in the cardiac output, which is caused by a sudden change in
the heart rate or rhythm. Partial or complete heart block is the usually seen in these patients but other arrhythmias such as ventricular tachycardia or ventricular fibrillation may also cause syncope. These attacks are usually transient lasting only for seconds, not related to posture and can occur several times during a day
Seizure When there is prolonged reduction in cerebral perfusion due to reduced cardiac out put as described above, the patient develops seizures. Absence of an aura helps to differentiate Stokes-Adams syndrome from seizures originating due to primary brain pathology Seizure
When there is prolonged reduction in cerebral perfusion due to reduced cardiac out put as described above, the patient develops seizures. Absence of an aura helps to differentiate Stokes-Adams syndrome from seizures originating due to primary brain pathology
History of congenital heart disease There are instances where the heart block is congenital in origin with or without associated structural defects History of congenital heart disease
There are instances where the heart block is congenital in origin with or without associated structural defects
History of diphtheria infection This is known to cause myocarditis and conduction defects, such as complete heart block and may present with Stokes-Adams episodes History of diphtheria infection
This is known to cause myocarditis and conduction defects, such as complete heart block and may present with Stokes-Adams episodes
History of acute rheumatic fever Acute rheumatic carditis can rarely cause acquired complete atrio-ventricular block and can present with Stokes-Adams attacks. It should therefore be excluded, particularly in pediatric patients History of acute rheumatic fever
Acute rheumatic carditis can rarely cause acquired complete atrio-ventricular block and can present with Stokes-Adams attacks. It should therefore be excluded, particularly in pediatric patients
History of cardiac surgery/ cardiac catheterisation Direct injury to conduction tissue can occur during cardiac surgery or cardiac catheterization that can lead to acquired complete heart block and Stokes-Adams syndrome History of cardiac surgery/ cardiac catheterisation
Direct injury to conduction tissue can occur during cardiac surgery or cardiac catheterization that can lead to acquired complete heart block and Stokes-Adams syndrome
History of neuro-muscular disease Heart block with Stokes-Adams syndrome can occur in neuro-muscular disorders such as acute inflammatory demyelinating polyradiculoneuropathy, myotonic dystrophy etc. History of neuro-muscular disease
Heart block with Stokes-Adams syndrome can occur in neuro-muscular disorders such as acute inflammatory demyelinating polyradiculoneuropathy, myotonic dystrophy etc.
Patient presenting with acute myocardial infarction In 2-7% of patients with acute myocardial infarction, is complicated with complete heart block Patient presenting with acute myocardial infarction
In 2-7% of patients with acute myocardial infarction, is complicated with complete heart block
History of neoplastic disease Primary or secondary neoplasms infiltrating the myocardium and the conducting system can also produce complete heart block History of neoplastic disease
Primary or secondary neoplasms infiltrating the myocardium and the conducting system can also produce complete heart block
History of sarcoidosis A cause for acquired complete heart block in young adults. Can present with rhythm abnormalities, Stokes-Adams attacks and sudden cardiac death History of sarcoidosis
A cause for acquired complete heart block in young adults. Can present with rhythm abnormalities, Stokes-Adams attacks and sudden cardiac death
History of taking medications such as digoxin and certain anti arrhythmic agents Digoxin toxicity can produce arrhythmias such as atrial fibrillation and complete heart block. Antiarrhythmic agents such as quinidine, procaineamide, lidocaine etc. can depress cardiac conduction History of taking medications such as digoxin and certain anti arrhythmic agents
Digoxin toxicity can produce arrhythmias such as atrial fibrillation and complete heart block. Antiarrhythmic agents such as quinidine, procaineamide, lidocaine etc. can depress cardiac conduction

Clinicals - Examination

Fact Explanation
Pallor followed by flushing There is initial pallor during the attack followed by facial flushing due to reactive hyperemia with the resumption of normal circulation Pallor followed by flushing
There is initial pallor during the attack followed by facial flushing due to reactive hyperemia with the resumption of normal circulation
Bradycardia Slow pulse can be detected during an attack if was caused by a bradyarrhythmia such as heart block Bradycardia
Slow pulse can be detected during an attack if was caused by a bradyarrhythmia such as heart block
Tachycardia Rapid pulse may be detected during an attack if it was caused by a tachyarrhythmia Tachycardia
Rapid pulse may be detected during an attack if it was caused by a tachyarrhythmia
Variable blood pressure Blood pressure is the manifestation of cardiac output and systemic peripheral resistance. Cardiac output is a manifestation of stroke volume and pulse rate. Stroke volume is affected by the pulse rate. e.g. during tachycardia the diastole shortens and ventricular filling is affected, so that the end diastolic volume reduces. This leads to a low stroke volume. In these patients blood pressure may vary depending on variations in the pulse rate Variable blood pressure
Blood pressure is the manifestation of cardiac output and systemic peripheral resistance. Cardiac output is a manifestation of stroke volume and pulse rate. Stroke volume is affected by the pulse rate. e.g. during tachycardia the diastole shortens and ventricular filling is affected, so that the end diastolic volume reduces. This leads to a low stroke volume. In these patients blood pressure may vary depending on variations in the pulse rate

Investigations - Diagnosis

Fact Explanation
Electrocardiography (ECG) Arrhythmias that can give rise to Stokes-Adams syndrome are divided into several groups according to their electrocardiographic manifestations.
They are as follows,
1) The transient period of asystole that occurs when there is sudden interruption to A-V impulse transmissions, before the junctional or ventricular pacemaker takes over the heart rate as seen when sinus rhythm or incomplete heart block converts to complete heart block
2) Ventricular asystole resulting from failure of the junctional pacemaker to assume heart rate when sinoatrial node impulse generation ceases (atrial stand still) as seen in patients with inferior wall myocardial
ischemia
3) Asystole in the presence of established heart block
4) Paroxysmal ventricular tachycardia or ventricular fibrillation in the presence of complete heart block. These occur when ventricular ectopic foci generate impulses that cause ventricular contraction
5) Paroxysmal ventricular tachycardia ventricular fibrillation with normal A-V conduction seen commonly after acute myocardial infarction
6) Supraventricular tachycardias and bradycardias
7) Sinus bradycardia, sinoatrial block, sinoatrial arrest in patients with pre-existing heart disease who are unable to increase stroke volume, to maintain sufficient cerebral perfusion
8) Combined forms, in which paroxysmal
tachyarrhythmias are followed by a period
of asystole due to a delay in automaticity of
pacemakers that were suppressed during
the tachycardia.
Different electrocardiographic changes may produce Stokes-Adams attacks in the same patient at different instances. In case of initial negative ECG but presence of evidence to suggest Stokes-Adams syndrome 24 hour ECG monitoring can be carried out
Electrocardiography (ECG)
Arrhythmias that can give rise to Stokes-Adams syndrome are divided into several groups according to their electrocardiographic manifestations.
They are as follows,
1) The transient period of asystole that occurs when there is sudden interruption to A-V impulse transmissions, before the junctional or ventricular pacemaker takes over the heart rate as seen when sinus rhythm or incomplete heart block converts to complete heart block
2) Ventricular asystole resulting from failure of the junctional pacemaker to assume heart rate when sinoatrial node impulse generation ceases (atrial stand still) as seen in patients with inferior wall myocardial
ischemia
3) Asystole in the presence of established heart block
4) Paroxysmal ventricular tachycardia or ventricular fibrillation in the presence of complete heart block. These occur when ventricular ectopic foci generate impulses that cause ventricular contraction
5) Paroxysmal ventricular tachycardia ventricular fibrillation with normal A-V conduction seen commonly after acute myocardial infarction
6) Supraventricular tachycardias and bradycardias
7) Sinus bradycardia, sinoatrial block, sinoatrial arrest in patients with pre-existing heart disease who are unable to increase stroke volume, to maintain sufficient cerebral perfusion
8) Combined forms, in which paroxysmal
tachyarrhythmias are followed by a period
of asystole due to a delay in automaticity of
pacemakers that were suppressed during
the tachycardia.
Different electrocardiographic changes may produce Stokes-Adams attacks in the same patient at different instances. In case of initial negative ECG but presence of evidence to suggest Stokes-Adams syndrome 24 hour ECG monitoring can be carried out
Electroencephalogram (EEG) Can be considered for patients presenting with seizures but have normal ECG, in order to rule out other causes for seizures Electroencephalogram (EEG)
Can be considered for patients presenting with seizures but have normal ECG, in order to rule out other causes for seizures

Management - Specific

Fact Explanation
Anticholinergic medication Blocks the parasympathetic activity on the heart and increases the heart rate and cardiac output. Indicated in instances such as complete heart block complicating recent inferior wall myocardial infarction, treatment and prevention of Stokes-Adams syndrome precipitated by sinus bradycardia, sinoatrial block and bradycardia-tachycardia syndrome etc. Anticholinergic medication
Blocks the parasympathetic activity on the heart and increases the heart rate and cardiac output. Indicated in instances such as complete heart block complicating recent inferior wall myocardial infarction, treatment and prevention of Stokes-Adams syndrome precipitated by sinus bradycardia, sinoatrial block and bradycardia-tachycardia syndrome etc.
Adrenergic agonist Medication Act by increasing heart rate and contractility . Can abolish some forms of atrial and ventricular extrasystoles. Also improve coronary artery blood flow. These are indicated when ventricular asystole or bradyeardia occurs in complete heart block and intracardiac pacing is unavailable Adrenergic agonist Medication
Act by increasing heart rate and contractility . Can abolish some forms of atrial and ventricular extrasystoles. Also improve coronary artery blood flow. These are indicated when ventricular asystole or bradyeardia occurs in complete heart block and intracardiac pacing is unavailable
Pacemaker Insertion of a permanent pacemaker is the treatment when the results of drug therapy is inconsistent or frequent complications arise. It is also useful in patients with congenital heart block. The general indications for implantable pacemaker include complete heart block associated with heart failure, complete
heart block with Stokes-Adams syndrome, complete heart block following acute anterior or inferior wall myocardial infarction, second degree A-V block complicating anterior wall myocardial infarction and complete heart block following cardiac surgery
Pacemaker
Insertion of a permanent pacemaker is the treatment when the results of drug therapy is inconsistent or frequent complications arise. It is also useful in patients with congenital heart block. The general indications for implantable pacemaker include complete heart block associated with heart failure, complete
heart block with Stokes-Adams syndrome, complete heart block following acute anterior or inferior wall myocardial infarction, second degree A-V block complicating anterior wall myocardial infarction and complete heart block following cardiac surgery
Cardioversion Can be considered in hemodynamically unstable patients, for rapid restoration of sinus rhythm Cardioversion
Can be considered in hemodynamically unstable patients, for rapid restoration of sinus rhythm

Concise, fact-based medical articles to refresh your knowledge

Access a wealth of content and skim through a smartly presented catalog of diseases and conditions.

  1. ABDON NJ, JOHANSSON BW, LESSEM J. Predictive use of routine 24-hour electrocardiography in suspected Adams-Stokes syndrome. Comparison with cardiac rhythm during symptoms. Br Heart J [online] 1982 Jun, 47(6):553-558 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC481181
  2. CARANO N, BO I, TCHANA B, VECCHIONE E, FANTONI S, AGNETTI A. Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature. Ital J Pediatr [online] 2012 Oct 30:61 [viewed 03 July 2014] Available from: doi:10.1186/1824-7288-38-61
  3. DíAZ-CASTRO O, ORIZAOLA P, VáZQUEZ S, GONZáLEZ-RíOS C, PARDO M, FERNáNDEZ-LOPEZ JA, ESCRICHE D. Images in cardiovascular medicine. "Stokes-adams epilepsy": sometimes we need the electroencephalogram. Circulation [online] 2005 Aug 23, 112(8):e101-2 [viewed 03 July 2014] Available from: doi:10.1161/CIRCULATIONAHA.104.503144
  4. JENSEN G, SIGURD B, MEIBOM J, SANDOE E. Adams-Stokes syndrome caused by paroxysmal third-degree atrioventricular block. Br Heart J [online] 1973 May, 35(5):516-520 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC458647
  5. NOEL C, GAGNON RM. Cardiac conduction abnormalities and Stokes-Adams attacks in myotonic dystrophy. Can Med Assoc J [online] 1978 Jun 10, 118(11):1402-4 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/657033
  6. O'ROURKE RA. Clinical Cardiology: The Stokes-Adams Syndrome--Definition and Etiology; Mechanisms and Treatment Calif Med [online] 1972 Jul, 117(1):96-99 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1518479
  7. REDWOOD D. Intravenous isoprenaline and orciprenaline as a guide to the drug treatment of Stokes-Adams attacks. Br Med J [online] 1968 Feb 17, 1(5589):419-421 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1985067
  8. SIGURD B, JENSEN G, MEIBOM J, SANDOE E. Adams-Stokes syndrome caused by sinoatrial block. Br Heart J [online] 1973 Oct, 35(10):1002-8 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/4759460
  9. SUCU M, DAVUTOGLU V, OZER O. Electrical cardioversion Ann Saudi Med [online] 2009, 29(3):201-206 [viewed 02 July 2014] Available from: doi:10.4103/0256-4947.51775
  10. VARDAS PE, SIMANTIRAKIS EN, KANOUPAKIS EM. New developments in cardiac pacemakers. Circulation [online] 2013 Jun 11, 127(23):2343-50 [viewed 02 July 2014] Available from: doi:10.1161/CIRCULATIONAHA.112.000086
  11. WANI BA, MISRA M, SHAH M, MUFTI S. Acute inflammatory demyelinating polyradiculoneuropathy presenting as complete heart block and Stoke-Adams attacks. Postgrad Med J [online] 1989 Feb, 65(760):103-4 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/2780457