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Sinus node dysfunction (SND), also known as sick sinus syndrome (SSS), is a group of abnormal heart rhythms (
arrhythmia Arrhythmias, also known as cardiac arrhythmias, are irregularities in the cardiac cycle, heartbeat, including when it is too fast or too slow. Essentially, this is anything but normal sinus rhythm. A resting heart rate that is too fast – ab ...
s) usually caused by a malfunction of the sinus node, the heart's primary pacemaker. Tachycardia-bradycardia syndrome is a variant of sick sinus syndrome in which the arrhythmia alternates between fast and slow heart rates.


Signs and symptoms

Often sinus node dysfunction produces no symptoms, especially early in the disease course. Signs and symptoms usually appear in more advanced disease and more than 50% of patients will present with syncope or transient near-fainting spells as well as bradycardias that are accompanied by rapid heart rhythms, referred to as tachycardia-bradycardia syndrome Other presenting signs or symptoms can include confusion, fatigue,
palpitations Palpitations occur when a person becomes aware of their heartbeat. The heartbeat may feel hard, fast, or uneven in their chest. Symptoms include a very fast or irregular heartbeat. Palpitations are a sensory symptom. They are often described as ...
,
chest pain Chest pain is pain or discomfort in the chest, typically the front of the chest. It may be described as sharp, dull, pressure, heaviness or squeezing. Associated symptoms may include pain in the shoulder, arm, upper abdomen, or jaw, along with n ...
,
shortness of breath Shortness of breath (SOB), known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that con ...
, headache, and nausea. Patients can also present with symptoms of
congestive heart failure Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome caused by an impairment in the heart's ability to fill with and pump blood. Although symptoms vary based on which side of the heart is affected, HF typically pr ...
,
stroke Stroke is a medical condition in which poor cerebral circulation, blood flow to a part of the brain causes cell death. There are two main types of stroke: brain ischemia, ischemic, due to lack of blood flow, and intracranial hemorrhage, hemor ...
or transient ischemic attacks due to the abnormal rhythm.


Complications

The most common complication of sinus node dysfunction is the development of tachycardia-bradycardia syndrome with abnormal atrial rhythms such as
atrial tachycardia Atrial tachycardia is a type of heart rhythm problem in which the heart's electrical impulse comes from an ectopic pacemaker (that is, an abnormally located cardiac pacemaker) in the upper chambers ( atria) of the heart, rather than from the sin ...
,
atrial fibrillation Atrial fibrillation (AF, AFib or A-fib) is an Heart arrhythmia, abnormal heart rhythm (arrhythmia) characterized by fibrillation, rapid and irregular beating of the Atrium (heart), atrial chambers of the heart. It often begins as short periods ...
, and flutter. These rhythms increases the risk of clot formation in the atrium, embolization, and
stroke Stroke is a medical condition in which poor cerebral circulation, blood flow to a part of the brain causes cell death. There are two main types of stroke: brain ischemia, ischemic, due to lack of blood flow, and intracranial hemorrhage, hemor ...
. Developing sinus arrest, sinus node exit block,
sinus bradycardia Sinus bradycardia is a sinus rhythm with a reduced rate of electrical discharge from the sinoatrial node, resulting in a bradycardia, a heart rate that is lower than the normal range (60–100 beats per minute for adult humans). Signs and sympt ...
, atrioventricular block, and other types of abnormal rhythms are also common complications. Sinus node dysfunction has a close association with the presence of
atrial fibrillation Atrial fibrillation (AF, AFib or A-fib) is an Heart arrhythmia, abnormal heart rhythm (arrhythmia) characterized by fibrillation, rapid and irregular beating of the Atrium (heart), atrial chambers of the heart. It often begins as short periods ...
due to their shared etiology of remodeling.


Causes

Sinus node dysfunction can be caused by intrinsic and extrinsic factors that affect the normal functioning of the sinus node. Intrinsic causes can include degeneration, dysfunction, or remodeling of the sinus node while extrinsic causes can create or worsen underlying atrial arrhythmias. Intrinsic causes tend to be responsible for permanent sinus node dysfunction while extrinsic causes are more commonly temporary.


Intrinsic causes

Age-related degenerative fibrosis of the sinus node is often identified as the most common intrinsic cause. Other intrinsic causes include inherited ion channel dysfunctions, remodeling diseases such as
heart failure Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome caused by an impairment in the heart's ability to Cardiac cycle, fill with and pump blood. Although symptoms vary based on which side of the heart is affected, HF ...
and
atrial fibrillation Atrial fibrillation (AF, AFib or A-fib) is an Heart arrhythmia, abnormal heart rhythm (arrhythmia) characterized by fibrillation, rapid and irregular beating of the Atrium (heart), atrial chambers of the heart. It often begins as short periods ...
, infiltrative diseases such as
sarcoidosis Sarcoidosis (; also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of White blood cell, inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph n ...
,
amyloidosis Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. There are several non-specific and vague signs and symptoms associated with amyloidosis. These include fatigue, peripheral edema, weigh ...
,
hemochromatosis Iron overload is the abnormal and increased accumulation of total iron in the body, leading to organ damage. The primary mechanism of organ damage is oxidative stress, as elevated intracellular iron levels increase free radical formation via the ...
, and connective tissue diseases, inflammatory etiology such as
rheumatic fever Rheumatic fever (RF) is an inflammation#Disorders, inflammatory disease that can involve the heart, joints, skin, and brain. The disease typically develops two to four weeks after a Streptococcal pharyngitis, streptococcal throat infection. Si ...
,
Chagas disease Chagas disease, also known as American trypanosomiasis, is a tropical parasitic disease caused by ''Trypanosoma cruzi''. It is spread mostly by insects in the subfamily Triatominae, known as "kissing bugs". The symptoms change throughout the ...
, and
Lyme disease Lyme disease, also known as Lyme borreliosis, is a tick-borne disease caused by species of ''Borrelia'' bacteria, Disease vector, transmitted by blood-feeding ticks in the genus ''Ixodes''. It is the most common disease spread by ticks in th ...
, as well as atherosclerotic and ischemic changes to the sinus node artery. Inherited sinus node dysfunction has been associated with mutations of the gene responsible for the formation of the alpha subunit of the sodium channel ( SCN5A).


Extrinsic causes

Common cardiac pharmacology such as beta-blockers,
calcium channel blocker Calcium channel blockers (CCB), calcium channel antagonists or calcium antagonists are a group of medications that disrupt the movement of calcium () through calcium channels. Calcium channel blockers are used as antihypertensive drugs, i.e., as ...
s,
digoxin Digoxin (better known as digitalis), sold under the brand name Lanoxin among others, is a medication used to treat various heart disease, heart conditions. Most frequently it is used for atrial fibrillation, atrial flutter, and heart failure. ...
, sympatholytic medication, and other antiarrhythmics can alter sinus node function to create an arrhythmia such as sick sinus syndrome. Electrolyte abnormalities such as
hyperkalemia Hyperkalemia is an elevated level of potassium (K+) in the blood. Normal potassium levels are between 3.5 and 5.0 mmol/L (3.5 and 5.0 mEq/L) with levels above 5.5mmol/L defined as hyperkalemia. Typically hyperkalemia does not cause symptoms. Oc ...
,
hypokalemia Hypokalemia is a low level of potassium (K+) in the blood serum. Mild low potassium does not typically cause symptoms. Symptoms may include feeling tired, leg cramps, weakness, and constipation. Low potassium also increases the risk of an a ...
, and
hypocalcemia Hypocalcemia is a medical condition characterized by low calcium levels in the blood serum. The normal range of blood calcium is typically between 2.1–2.6  mmol/L (8.8–10.7 mg/dL, 4.3–5.2 mEq/L), while levels less than 2.1  ...
can also alter normal sinus node functioning.
Hypothyroidism Hypothyroidism is an endocrine disease in which the thyroid gland does not produce enough thyroid hormones. It can cause a number of symptoms, such as cold intolerance, poor ability to tolerate cold, fatigue, extreme fatigue, muscle aches, co ...
, hypoxia,
hypothermia Hypothermia is defined as a body core temperature below in humans. Symptoms depend on the temperature. In mild hypothermia, there is shivering and mental confusion. In moderate hypothermia, shivering stops and confusion increases. In severe ...
, and various toxins have also been associated with sinus node dysfunctions.


Diagnosis


Electrocardiogram

The primary 12-lead electrocardiogram (ECG) finding in sinus node dysfunction is inappropriate
sinus bradycardia Sinus bradycardia is a sinus rhythm with a reduced rate of electrical discharge from the sinoatrial node, resulting in a bradycardia, a heart rate that is lower than the normal range (60–100 beats per minute for adult humans). Signs and sympt ...
. Sinus node dysfunction can also present with sudden sinus arrest with or without junctional escape, sinoatrial block, prolonged asystolic period followed by tachycardias, or tachycardia-bradycardia syndrome presenting as various atrial arrhythmias such as
atrial fibrillation Atrial fibrillation (AF, AFib or A-fib) is an Heart arrhythmia, abnormal heart rhythm (arrhythmia) characterized by fibrillation, rapid and irregular beating of the Atrium (heart), atrial chambers of the heart. It often begins as short periods ...
, flutter,
tachycardia Tachycardia, also called tachyarrhythmia, is a heart rate that exceeds the normal resting rate. In general, a resting heart rate over 100 beats per minute is accepted as tachycardia in adults. Heart rates above the resting rate may be normal ...
, or
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, feeling li ...
.


Clinical diagnosis

Diagnosing sinus node dysfunction requires clinical symptoms as well as ECG abnormalities. If ECG findings cannot be identified, prolonged cardiac monitoring should be pursued either with a Holter monitor in an outpatient setting or telemetry while inpatient, due to the transient nature of abnormal ECG findings. If Holter or telemetry monitoring fails to identify ECG changes and suspicion of sinus node dysfunction remains high due to severe symptoms or episodes of syncope, implantable loop recorders should be considered for extended monitoring up to 24 months. Exercise stress tests can be utilized to identify intrinsic causes of sinus node dysfunction. Tilt table tests can be used to discriminate bradycardia caused by dysfunction of the
autonomic nervous system The autonomic nervous system (ANS), sometimes called the visceral nervous system and formerly the vegetative nervous system, is a division of the nervous system that operates viscera, internal organs, smooth muscle and glands. The autonomic nervo ...
.


Treatment

The primary reason for considering treatment is the presence of symptoms. Pacemaker implantation is the primary treatment modality of symptomatic sinus node dysfunction. The goal of this treatment modality is to relieve symptoms associated with sinus node dysfunction and improve quality of life. Dual chamber pacemakers are preferred due to the possibility of developing atrioventricular block as well as long term cost-effectiveness relative to single-chamber atrial pacemakers. In tachycardia-bradycardia syndrome, medication-based management can treat atrial tachyarrhythmias. However, these medications may exacerbate underlying bradyarrhythmia. Therefore, a dual-chamber pacemaker capable of managing atrial tachyarrhythmias as well as bradyarrhythmias is implanted before drug therapy is begun.


Epidemiology

Overall incidence of sinus node dysfunction increases with age with 1 in 1000 in adults over 45 years old and 1 in 600 cardiac patients over 65 years old. Sinus node dysfunction is the primary indication for approximately 30%-50% of all pacemaker implantation in the United States. Sinus node dysfunction is a relatively uncommon syndrome in the young and middle-aged population.


References


External links

{{Heart diseases Cardiac arrhythmia Syndromes affecting the heart