Benign or

Harbinger of Sudden Death?

R. Oehm, MD, FACC,
10/26/06 with POTTS 10/20/08
 Definition: a transient loss of consciousness &
postural tone with a rapid return to baseline.
 Pre syncope included in evaluation
 Common: lifetime risk ~ 33%; 1-4% of ED visits & 1-
6% of hospital admissions
 Risk of death & serious morbidity varies with
etiology & “malignancy”
 Primary reference: JACC.2006;47:473-484
 Neurocardiogenic = neuromediated =
vasodepressor = vasovagal syncope, is the biggest
single cause; together with other non cardiac causes
results in ~ a 3% annual mortality
 Cardiac causes of syncope result in ~ a 30% annual
mortality!
 Neurocardiogenic (NCS)
 Situational: micturition, cough, defecation,
laughter, breath holding (children), swallowing,
 Orthostatic
 Carotid sinus sensitivity
 Neurologic
 Conversion reactions & panic
 Medications ( cardiac & non cardiac)
Young People, including Medical Students
[JAMA.2006;296:1211-1212]
Observing surgery or procedures involving pain to a
patient
Blood or bloody procedures
Something shocking or new
Can almost always be eventually overcome
 Inability of postural reflexes to compensate for
hemodynamic requirements when upright
 Medications
 Aging, autonomic dysfunction
 Deconditioning, influenza
 Dehydration
 Blood loss: AAA, GI bleed, ectopic pregnancy,
trauma, etc.
 Postural Tachycardia Syndrome, a type of autonomic
disturbance with similarities to NCS [Circulation.2008;117:2814-2817]
 Characterized by orthostatic intolerance relieved by
recumbence, palpitations, fatigue, lightheadedness, exercise
intolerance, nausea, diminished concentration,
tremulousness, syncope, & near syncope. HR may increase
by 30 bpm
 They may not have orthostatic hypotension
 They may be misdiagnosed as having severe anxiety or panic
disorder
 Functional impairment may be similar to COPD or CHF
 Adequate fluids & reconditioning may help
 Multiple medications have been tried, but most have little or
no benefit
 Some drugs, mainly diuretics & vasodilators, worsen
symptoms
 Pulmonary embolism
 Subarachnoid hemorrhage
 Occult blood loss:
 ruptured AAA,
 ectopic pregnancy,
 trauma,
 GI bleed

 ED Lecture, Rose, 3/2/06

 Fear Adrenergic
Fright Outflow
Stress Increased cardiac
pain contractility

Vagotonia
Vasodilation
Bradycardia
syncope
 Cause: thought to be stimulation of cardiac
mechanoreceptors by catecholamines, resulting in reflex
sympathetic withdrawal & parasympathetic-vagal
stimulation with bradycardia & vasodilation
 Treatment: 1) hydration + salt, 2) vigorous leg muscle
contraction ie with leg crossing [JACC.2006;48:1652-1657], 3) meds:
beta blockers largely unsuccessful, even with
tilt/isoproterenol + cases [circulation.2006;113:1164-1170], other meds
also not really helpful either [NEJM.2005;352:1004-1010]
 Tachyarrhythmias: include WPW, Brugada, LQTS,
Catecholaminergic Polymorphic VT,
 Bradyarrhythmias & Blocks
 Cardiomyopathies: (HCM, Ischemic CM, Dilated CM,
ARVD/C. etc.)
 Outlet Obstruction: ( AS, PS, Pulmonary Embolism,
HCM, etc.)
 The most important role of evaluation is to determine the
risk of death!
 Careful history: include that of bystanders
 Careful physical exam, including orthostatic BP & pulse
 ECG: for morphology & rhythm disturbances
 In many cases no further workup may be needed
 Echocardiogram: to look for structural abnormalities
 Suspected blood loss: Hemoglobin, stool for occult blood,
abdominal ultrasound, pregnancy test, etc
 Exercise testing: for ischemia, exercise induced
tachyarrhythmias
 Non invasive ECG monitoring, holter, event monitoring, etc
 Other: tilt table, EPS (electrophysiolgic study)
 Observations of onlookers
 In NCS, premonitory symptoms common, also post episode
fatigue or weakness
 Tonic/clonic seizures may be in both cardiac & non cardiac
causes
 Absence of prodrome c/w arrhythmia or central
neurogenerative disorder
 Focal neurological signs, auras, premonitions, & post ictal
confusion suggest neurological etiology
 TIA’s (transient ischemic attacks) rarely result in syncope, except with
basilar A. or bilateral carotid disease
 History of myocardial infarction or repaired congenital heart disease
greatly increases likelihood of serious V. arrhythmias
 Head trauma
 Carotid sinus sensitivity (ie with head turning or tight collar)
 New drug given, ie antihypertensive, antiarrhythmic, phenothiazine,
tricyclic, ephedra, etc. Both arrhythmias & orthostasis to be considered
 Orthostatic BP & pulse, reclining & upright
 Carotid Bruits
 Murmurs, reclining & upright, evidence of valvular heart
disease
 LV dysfunction, S3 gallop, JVD, rales, peripheral edema, etc.
 Pulmonary hypertension by loud S2P, RV heave, etc
 Neurological exam
 Bradyarrhythmias & blocks, long pauses, “sick sinus
syndrome”
 Tachyarrhythmias, also VPB’s, NSVT
 Delta Wave & WPW
 Ion Channel: LQTS (types 1,2, & 3)
 Ion channel: Brugada Syndrome (patterns 1,2,&3)
 ARVD/C (epsilon wave or “knuckle”)
 With a classic history for NCS, particularly in a
younger person, without any significant risk factors,
no further workup may be needed
 With more risk factors, suspicious history, and older
age, then further workup may be indicated
 Excellent for identifying underlying structural heart
disease
 Valvular heart disease, esp. AS, PS
 Hypertrophic CM, outlet gradient
 Pulmonary embolism by RVE, pulmonary
hypertension
 ARVD/C suggested (may need MRI)
 Poor LV systolic function: CAD, Cardiomyopathies
 Supervised by someone able to identify > 6 parameters,
esp. pulse & BP
 Failure of SBP to rise or actual drop in BP in < age 40
suggests HCM or severe 3 vessel or L. main CAD; in elderly
also consider autononomic dysfunction
 Catecholaminergic polymorphic VT
 Any inducible VT may indicate increased risk, particularly
with structural disease
 Holter monitoring: 24-48 hrs, may allow discovery
of relatively frequent rhythm abnormalities
 Event recording: allows monitoring for 7-60 days,
patient activated, but lower quality & many findings
may missed
 Implantable loop recorder: subcutaneous, up to 14
months, may yield diagnostic information in > 90%
 Formerly frequently done, but seldom done now
 Now serious questions about sensitivity, specificity,
diagnostic yield, & day-to-day reproducibility exist;
many false + studies occur
 With a good history for NCS & normal workup,
neither tilt table nor further workup is needed,
unless episodes “malignant”
 In the absence of underlying heart disease, syncope
is not associated with excess mortality
 However, syncope that occurs with little or no
warning, & results in significant injury or property
damage, may be considered as “malignant”; in
these cases further workup may be warranted
 Exercise induced syncope needs workup
 In a patient with normal evaluation of syncope, the yield of
EPS is only ~ 3%
 Not recommended unless underlying heart disease or
“malignancy”
 Consider for CAD with ischemia & syncope (not necessarily
acute STEMI), also consider for non ischemic CM; but for
both with LVEF < 35% could go directly to AICD
 ? For HCM & ARVD/C; for any suspected VT/VF as cause of
syncope, could go directly to AICD
 Evaluation for ACS, including STEMI & Non STEMI,
is routine in our ED’s, but may be missed in other
settings
 Any ischemia may result in VT/VF, & syncope, if not
sudden death
 Bezold-Jarisch reflex: inferior MI
 Acute STEMI may not necessarily require
arrhythmia evaluation beyond monitoring, if well
reversed by PCI or thrombolytic
 However ischemia may still persist & many
recommend pursuing EPS anyway, especially for
late ventricular arrhythmias
 Risk of death is mainly related to LV function, &
LVEF < 35% may be best be treated with AICD
 Syncope is related to increased mortality due to episodes of
VT
 Differential Diagnosis includes: bradycardia, tachycardia,
orthostatic hypotension, pulmonary embolism, & even NCS
(abnormal Vasodilatory response)
 1 yr. risk of sudden death ~ 45% regardless of cause
 Consider for AICD, with/without first doing EPS
 A relatively frequent (1/500) genetically determined
myocardial disease with variable prognosis
 Risk of sudden death 0.6-1.0%/year
 Most common cause of sudden death in young athletes
 Syncope may be due to self terminating episodes of VT, but
also SV arrhythmias, severe outflow obstruction,
bradyarrhythmias, BP drop with exercise, & NCS
 Other risk factors for sudden death may include: family
history of sudden death, marked hypertrophy ( ~ > 3 cm), &
episodes of NSVT on holter
 EPS plays a minimal role
 High risk patients are better served with AICD
 Certain genetic mutations have higher risk, but this has not
been useful or practical knowledge so far
 ARVD/C characterized by VT with LBBB pattern, episodes of
syncope, & risk of sudden death, especially with exercise
 Enlarged RV with myocyte replacement by fat or fibrosis;
may also effect LV
 May be familial, & more common in the Veneto region of
Northern Italy, where it was the most common cause of
sudden death, age < 35
 Diagnosis by history, ECG (epsilon wave, possible T inversion
beyond V2), skin changes (palmar keratoses),
echocardiography, & MRI to confirm
 Phase 1, or the early phase, may be silent or be with sporadic
ventricular ectopy, subtle ECG changes, morphologic
changes & sudden death
 A “hot phase” may be marked by ventricular arrhythmias,
recurrent syncope, & inflammation.
 Phase 2, may include sustained VT, diffuse RV & LV changes,
& more sudden death
 Phase 3 & 4 more advanced
 AICD may be indicated for all these
 Inherited cardiac ion channel abnormality
 Defect in potassium channel, LQT1 & LQT2, & sodium channel, LQT3,
(also now 5 other Long QT “channelopathies” identified)
 Lifetime risk of syncope, aborted, or actual sudden death by QTc:
▪ < 440 ms: 5%
▪ 460-500 ms: 20%
▪ > 500 ms: 500 ms: 50%
 Syncope likely due to torsade des pointes (polymorphic VT) that
terminates spontaneously
 Treatment with: AICD, beta blockers, avoiding strenuous exercise,
avoiding meds that prolong QT
 Danger of sudden death with VT/VF
 International LQT Registry, ages 10- 20 years, 2772
participants [JAMA.2006;296:1249-1254]
 81 pts. with aborted cardiac arrest & 45 pts. With sudden
death; 9/81 pts. Initially surviving had later sudden death!
 Predictors: syncope, sex (M’s > F’s), QTc > 530 msec (but incr.
risk >440 M., > 450 F)
 Treatment: beta blockers reduced risk in this study (but may
not help LQT3)
 Reduced Risk after age 50 (not this study)
 Only recently described (since 2000)
 QT < 300 ms (sometimes 320 or 330)
 Risk of Syncope & Sudden Death!
 Risk of SV Arrhythmias ( ie PAF)
 Involves overactive K+ channel
 Drugs to lengthen QT may help, Quinidine best so
far
 AICD best, esp. if VT/VF inducible by EPS
 Inherited Cardiac Ion (Sodium) Channel inherited disorder
with susceptibility to polymorphic VT, syncope, & sudden
death
 Distinctive ECG pattern of RBBB with ST elevation in V1 & V2
(types 1, 2, & 3)
 Pattern may be intermittent or brought out by type 1a or 1c
antiarrhythmics (procainamide, flecainide, ajmaline)
 With syncope, 2 year risk of death of ~ 30%
 Sudden death @ rest, night, fever, activity
 AICD recommended
 An inherited cause of syncope & sudden death in children &
adolescents
1) No structural abnormality identified
2) Adrenergic or catecholamine activation by emotion or
physical stress
3) Normal resting ECG
4) Bidirectional VT, alternating 180 degree QRS axis on a beat
to beat basis
5) Can be brought out by exercise testing
6) Treatment: beta blockers & ICD’s
 Despite some serious causes of syncope in pediatric patients,
it is more often benign
 NCS is common & breath holding is common in very young
patients
 With no ECG abnormalities, + family history, or exertional
symptoms, further workup may not be necessary.
 Resting bradycardia may indicate drug ingestion, anorexia
nervosa, & central nervous system trauma
 Marked sinus pauses or marked bradycardia may result in
syncope
 3rd degree AVB may require pacemaker
 With ECG abnormalities, + family history, & syncope
with exertion further workup is needed
 It is important to exclude: HCM, ARVD/C,
catecholaminergic polymorphic VT, LQTS, Brugada
syndrome, or any exertional induced
tachyarrhythmia
 Congenital heart disease with/without repair,
with/without cyanosis, may result in syncope &
sudden death. EPS may be helpful to sort out
 Anomalous coronary arteries are a rare cause of
exertional syncope & sudden death
 Extensive screening of athletes prior to any sports
participation may greatly reduce the incidence of sudden
death by 89% as in N. Italy [JAMA.2006;1593-1601]
 Prospective athletes aged, 12-35 years, in the Veneto region
of Italy were screened
 An excellent history & physical exam were first done, then a
12 lead ECG on all
 Further studies were done if indicated
 Vigorous sports were prohibited in those who failed
examinations
 There is ~ a 30% annual incidence of falls in the elderly & ~
30% may be due to syncope
 There is overlap of falls, orthostatic hypotension, &
dizzyness
 Also consider age related changes, including gait disorders,
leg weakness, orthostatic & autonomic changes, drugs,
dehydration with inadequate thirst mechanism, carotid sinus
sensitivity, Parkinson’s disease, etc
 Syncope is unusual in neurologic disease
 Even cardiac causes can result in upward gaze deviation,
myoclonal jerks, & brief automatisms
 Severe bilateral carotid disease, basilar artery disease, &
neurodegenerative disease may sometimes be a cause
 Subarachnoid hemorrhage may proceed from syncope to
coma
 Seizure disorders are common
 EEG, CT & MRI may be helpful
 Most causes of syncope are benign, with NCS most
common, especially in young people
 Beware of possible “malignant” or serious cardiac
causes, especially in the elderly or in younger
people, during or immediately after exertion!