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syndrome
Author: David Benditt, MD
Section Editor: Peter Kowey, MD, FACC, FAHA, FHRS
Deputy Editor: Susan B Yeon, MD, JD, FACC
Contributor Disclosures
All topics are updated as new evidence becomes available and our peer review process is
complete.
Literature review current through: Jul 2021. | This topic last updated: Aug 19, 2020.
INTRODUCTION
The carotid baroreceptors, located bilaterally in the neck near the bifurcation of the
internal and external carotid arteries, play an important physiologic role in heart
rate and blood pressure control. Carotid sinus hypersensitivity (CSH) consists of
the observation that stimulation of the carotid artery baroreceptors results in a
greater than expected fall in heart rate and drop in blood pressure. In such cases,
carotid baroreceptor stimulation (eg, mechanical forces such as may occur with
turning of the neck or looking upward) results in vagal activation and/or
sympathetic inhibition. CSH is not a clinical diagnosis per se but is a potential
cause for symptoms in some patients, at which time the condition is termed
carotid sinus syndrome (CSS).
CSH and CSS will be reviewed here. Other types of reflex syncope, including
vasovagal syncope, as well as general discussions of the pathogenesis, etiology,
and evaluation of syncope, are discussed separately. (See "Reflex syncope in
adults and adolescents: Clinical presentation and diagnostic evaluation" and
"Syncope in adults: Epidemiology, pathogenesis, and etiologies" and "Syncope in
adults: Clinical manifestations and initial diagnostic evaluation".)
DEFINITION
However, others believe that the scientific basis for the stated criteria is unclear
and that they criteria are too sensitive and nonspecific. Some have proposed that
cerebral hypoperfusion for >5 seconds with the patient in an upright position be
utilized [3]. An even more stringent requirement, advanced especially in Europe, is
that CSH should result in reproduction of symptoms with the patient in the upright
posture. Our experts agree that a five-second pause induced by carotid sinus
massage is indicative of CSH and in a symptomatic individual is diagnostic of CSS
in the absence of a reasonable alternative diagnosis. In any case, regardless of the
chosen definition, it is paramount to observe changes in HR and BP. (See 'Test
results' below.)
In contrast to CSH, there is general consensus in the definition of carotid sinus
syndrome (CSS), which reflects that a patient's symptoms (eg, lightheadedness,
syncope, etc) directly result from CSH.
PREVALENCE
PATHOPHYSIOLOGY
● Innervation of the sinoatrial and atrioventricular (AV) nodes via the vagus nerve
and the parasympathetic ganglia.
The site of the abnormality that causes the hypersensitive response in patients
with CSH has not been definitively established. Arguments have been made for
several possibilities, and several factors may be operative in any patient:
CLINICAL FEATURES
CSH is a clinical observation of heart rate (HR) pauses and drop in systolic blood
pressure (BP) but is not inherently a clinical condition requiring treatment. CSS
reflects the spontaneous clinical manifestation of CSH when changes in HR
and/or BP cause symptoms, most commonly syncope, lightheadedness, or
otherwise unexplained falls in older patients.
Carotid sinus hypersensitivity — CSH, most commonly identified as a pause
duration of >3 seconds or drop in BP >50 mmHg during carotid sinus massage,
may or may not be associated with symptoms [1]. However, as with the debate
over the definition of CSH, there are several reasons these criteria are inadequately
sensitive and specific, most notably:
● Both bradycardia and vascular dilation may work synergistically and contribute
to symptomatic hypotension.
One examination of diagnostic criteria suggests that when carotid sinus massage
is used as part of the overall diagnostic evaluation of syncope of unknown cause,
an induced pause or cerebral hypotension duration of >5 seconds is a more
reasonable diagnostic standard in the absence of an alternative cause for
symptoms [3].
● Lightheadedness/presyncope
● Syncope
● Otherwise unexplained falls in older patients
Risk factors such as older age, male gender, concomitant atherosclerotic disease,
prior neck surgery, or neck irradiation should increase suspicion. CSS is diagnosed
when, by history, spontaneous syncope symptoms can be reasonably attributed to
mechanical manipulation of the carotid sinuses. Unfortunately, obtaining a clear
history of carotid sinus stimulation is challenging and uncommon. Symptoms
including syncope may be reproduced by carotid sinus massage, but while such
demonstration can be of greatest value in establishing CSS as the diagnosis, it is
often not possible. Reproduction of symptoms may be better achieved if carotid
sinus massage is undertaken with the patient in an upright position. The latter is
most safely accomplished with the patient gently secured on a tilt table so the
patient can be prevented from falling. (See 'Diagnostic testing' below.)
DIAGNOSTIC TESTING
Apart from careful exclusion of other possible causes of symptoms, the only
relatively specific test for CSH/CSS is carotid sinus massage usually best
performed in combination with upright posture. Both the American and European
syncope guidelines recommend including carotid sinus massage after the initial
evaluation in patients over age 40 with syncope of unknown etiology (but in whom
other findings are suggestive of a reflex mechanism) [1,2].
● The patient is placed in the supine position with the neck extended (ie, raising
the chin away from the chest) to maximize access to the carotid artery. The
carotid sinus is usually located inferior to the angle of the mandible at the level
of the thyroid cartilage near the arterial impulse (figure 1). If the test is non-
diagnostic in terms of reproducing symptoms, then it should be repeated with
the patient in a more upright posture.
● Symptoms associated with CSH are rarely induced unless carotid sinus
massage is carried out with the patient upright. If the response is
nondiagnostic in the supine position, the procedure should be repeated with
the patient seated upright or positioned head-up on a tilt table (but gently
secured so as not to fall).
● Positive for CSH following induction of either a pause >3 seconds (with some
experts preferring >5 seconds) OR a drop of systolic BP >50 mmHg [1-3].
DIAGNOSIS
CSH is identified based on a heart rate (HR) pause duration of >3 seconds (some
prefer >5 seconds, as discussed above) or drop in blood pressure (BP) >50 mmHg
during carotid sinus massage.
The finding of CSH in older adult patients should not be construed as diagnostic
for the presenting symptoms (ie, lightheadedness, syncope, falls) unless other
causes of the symptoms have been eliminated or the history clearly relates
symptoms to neck movement or mechanical distortion.
DIFFERENTIAL DIAGNOSIS
Permanent pacing for symptomatic patients — For patients with CSS (ie, a history
of suspected reflex syncope and a positive cardioinhibitory response following
carotid sinus massage), we suggest implanting a permanent cardiac pacemaker in
the following situations [2]:
In CSS, virtually all studies seem to favor the value of pacing despite the fact that
both cardioinhibitory and vasodepressor mechanisms may be operating. This
latter observation differs from other reflex conditions such as the vasovagal faint
in which a predominant vasodepressor component may undermine the value of
pacing therapy. Why these two forms of reflex symptoms are so different is
unknown.
Two small randomized trials found that pacing reduced the rate of syncope
recurrence in patients with CSS [19,20]. Dual-chamber pacing is beneficial in
patients with CSH who have a cardioinhibitory response, but not in those with a
pure vasodilatory response.
Pacing may prevent nonmechanical falls as well as syncope. This was illustrated
in the SAFE PACE trial, in which 175 patients seen in an emergency facility
because of a nonmechanical fall without loss of consciousness were randomly
assigned to a dual-chamber pacemaker or no therapy [21], but not confirmed in
SAFE PACE 2 [22].
● Patients with untreated CSS (ie, known or suspected [by history] relation of
symptoms to CSH in the absence of alternative cause) – Not fit to drive.
UpToDate offers two types of patient education materials, "The Basics" and
"Beyond the Basics." The Basics patient education pieces are written in plain
language, at the 5th to 6th grade reading level, and they answer the four or five key
questions a patient might have about a given condition. These articles are best for
patients who want a general overview and who prefer short, easy-to-read
materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th
grade reading level and are best for patients who want in-depth information and
are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We
encourage you to print or e-mail these topics to your patients. (You can also locate
patient education articles on a variety of subjects by searching on "patient info"
and the keyword(s) of interest.)
● Basics topics (see "Patient education: Vagal maneuvers and their responses
(The Basics)")
● Dual-chamber pacing is often helpful in patients with CSS who are found to
have a slow heart rate (HR) or prolonged pause in their heart rhythm as a
cause of their symptoms. Since the slow HR is only part of the problem, pacing
may not be a perfect solution in all individuals. However, it is valuable in most
CSS patients. Medications may need to be added to diminish the vasodilatory
component for some other patients.
• Patients with recurrent syncope in the setting of a history of spontaneous
carotid sinus stimulation, who also have ventricular asystole of more than
three to five seconds in response to carotid sinus massage, are considered
to have been definitively diagnosed with carotid sinus syncope. We
recommend that such patients be treated with a permanent pacemaker
(Grade 2C). (See 'Management' above.)
ACKNOWLEDGMENT
The editorial staff at UpToDate would like to acknowledge Brian Olshansky, MD,
who contributed to an earlier version of this topic review.
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