Introduction
Background
Sinus node dysfunction (SND) first appeared in the literature as a clinical entity in 1968 under the name of sick sinus syndrome.1 Today SND refers to abnormalities in sinus node impulse formation and propagation, and includes sinus bradycardia, sinus pause/arrest, chronotropic incompetence, and sinoatrial exit block.2 SND is frequently associated with conduction system disease in the heart and various supraventricular tachyarrhythmias, such as atrial fibrillation and atrial flutter. When associated with supraventricular tachyarrhythmias, SND is often termed tachy-brady syndrome.2
Although SND may occur at any age, it is primarily a disease of the elderly and, presumably, related to the senescence of the sinus node, which is often accompanied with the senescence of the atrium and the conduction system in the heart. When SND occurs earlier in life, it is often secondary to other cardiac disease processes.3 The natural history of SND may be highly variable, although it tends to be progressive in nature. The only effective treatment for patients with chronic symptomatic SND is pacemaker therapy. Asymptomatic patients do not require therapy.
Pathophysiology
SND involves abnormalities in sinus node impulse formation and propagation, which are often accompanied with similar abnormalities in the atrium and the conduction system in the heart. Together, these abnormalities may result in inappropriately slow ventricular rates and long pauses at rest or during various stresses. When SND is mild, patients are usually asymptomatic. As SND becomes more severe, patients may develop symptoms due to organ hypoperfusion and pulse irregularity. Among these symptoms are fatigue, dizziness, confusion, fall, syncope, angina, heart failure symptoms and palpitations.
Frequency
United States
The exact incidence of sinus node dysfunction is unknown. The syndrome occurs in approximately one in 600 cardiac patients older than 65 years.4
International
Due to its relationship with advanced age, SND is more prevalent in countries where citizens have a longer life expectancy.
Mortality/Morbidity
Symptoms of sinus node dysfunction almost invariably progress over time. The most dramatic symptom in patients with SND is syncope. About 50% of patients with SND develop tachy-brady syndrome over a lifetime8 ; such patients have higher risk of stroke and death. The survival of patients with SND appears to depend primarily on the severity of underlying cardiac disease and is not significantly changed by pacemaker therapy.5,6,7 However, incidence of sudden death owing directly to SND is extremely low.5
Race
No racial preponderance exists.
Sex
Men and women are affected in equal numbers.
Age
Sinus node dysfunction may occur at any age but is primarily a disease of the elderly, with the average age being about 68 years-old9 . SND in young patients is often related to underlying heart diseases.
Clinical
History
With mild SND, patients are usually asymptomatic. As SND progresses, patients often develop symptoms due to pulse irregularity and organ hypoperfusion. The severity of organ hypoperfusion symptoms depends on the severity of SND and on the functional reserve state of an organ.
- Cerebral symptoms - Irritability, labile mood swings, forgetfulness, dizziness, slurred speech, blanking periods, falls, and syncope
- Cardiac symptoms - Palpitations, angina, congestive heart failure symptoms, and sudden cardiac death (rare)
- Other symptoms
- Vague gastrointestinal symptoms and oligurea
- Tachy-brady syndrome may include palpitations due to tachycardia and stroke/transient ischemia attack (TIA) symptoms
Physical
- Inappropriately slow heart rate.
- Carotid sinus massage: may reveal sinus pause of more than 3 seconds ± hypotension in patients with carotid sinus hypersensitivity.
Causes
Although the exact etiology is usually not identified, most cases are believed to be attributable to a combination of various intrinsic and extrinsic factors. The most common intrinsic causes are cardiac age-related sinus node changes and coronary artery disease. The most common extrinsic causes are medications and autonomic hyperactivity.
Intrinsic SND
- Age-related changes: the most common cause of SND is the replacement of sinus node tissue with fibrotic tissue. These fibrotic changes also occur in the atrium and the conduction system of the heart, and are believed to contribute to the association among SND, tachy-brady syndrome, conductive system disease and an inappropriately slow escape rhythm. Recently, age-related down-regulation of calcium channel expression in the sinus node has also been suggested as a potential cause of SND.10
- Coronary artery disease: coronary artery disease is believed to be a common contributory cause of SND, probably through atherosclerotic changes in the sinus node artery.
- Familial sick sinus syndromes and SND in children: Several molecular defects in human hearts (defects in the sodium channel, calcium channel and hyperpolarization-activated cyclic nucleotide-gated cation (HCN) channel, ankyrin-B and connexin 40) have been associated with familial sick sinus syndromes.3 SND is seen in children with congenital and acquired heart disease, particularly after corrective surgery. The cause of SND in these children is likely related to the underlying structural heart disease and surgical trauma to the sinus node and/or sinus node artery.
- Tachy-brady syndrome: tachycardia-mediated remodeling of the sinus node is present in patients with atrial fibrillation/flutter and it may contribute to SND in these patients. In patients with tachy-brady syndrome, atrial fibrillation ablation can reverse sinus node dysfunction as evidenced by a reduction in sinus node recovery time, increase in mean and maximal heart rates and lack of symptoms related to sinus bradycardia or pause.11
- Other structural heart diseases are uncommon causes of SND. These include, but are not limited to various cardiomyopathies, myocarditis, pericarditis, infiltrative heart diseases (amyloidosis, hemochromatosis, neoplasm), collagen vascular diseases (systemic lupus, scleroderma), neuromuscular diseases (myotonic dystrophy, Friedreich ataxia).
Extrinsic SND
- Medications: Beta-blockers, calcium channel blockers, digoxin, and various anti-arrhythmic drugs suppress sinus node function.
- Autonomic dysfunction: SND can be secondary to autonomic nervous system dysfunction in patients with neurocardiogenic syncope, and carotid sinus hypersensitivity. Conditions associated with marked hypervagotonia, as in well-trained athletes, can also result in SND. Recent evidence, however, suggests that there may be some intrinsic factor as well in well-trained athletes who develop SND.12 .
- Endocrine-metabolic diseases (hypothyroidism and hypothermia) and electrolyte imbalances (hypokalemia and hypocalcemia) can contribute to SND.
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Overview: Sinus Node Dysfunction |
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| References |
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References
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Further Reading
Keywords
sick sinus syndrome, sinus node dysfunction, SND, sinoatrial disease, sinoatrial dysfunction, sluggish sinus syndrome, sinus nodal dysfunction, Valsalva maneuver, carotid sinus massage, idiopathic degenerative disease, coronary artery disease, autonomic hyperactivity, intrinsic sinus nodal dysfunction, infiltrative diseases, cardiomyopathy, hypertension, collagen vascular diseases, congenital heart disease, heart transplant, musculoskeletal disorders, myocarditis, pericarditis, beta-blockers, nondihydropyridine calcium channel blockers, cardiac glycosides, sympatholytic antihypertensives, membrane-active antiarrhythmics, autonomic dysfunction, vasovagal syncope, carotid sinus syndrome, extrinsic sinus nodal dysfunction, electrolyte imbalance, hypothyroidism, hyperthyroidism, hypothermia, sepsis
Overview: Sinus Node Dysfunction