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Goldman: Cecil Medicine, 23rd ed.

Copyright © 2007 Saunders, An Imprint of Elsevier
Chapter 64 – VENTRICULAR ARRHYTHMIAS
Bruce B. Lerman
PREMATURE VENTRICULAR COMPLEXES
Definition and Electrocardiographic Features
Premature ventricular complexes (PVCs) are ubiquitous arrhythmias that are recognized on the
surface electrocardiogram (ECG) by their wide (generally >120 msec) and bizarre QRS
morphology, which occurs independently of atrial activation (P waves). Late-cycle PVCs may
follow a P wave that occurs on time and are identified by a shorter than normal PR interval.
PVCs may be due to enhanced automaticity, triggered activity, or re-entry.
Most PVCs are followed by a “compensatory pause” because the PVC fails to conduct
retrogradely to the atria and cannot affect or reset the electrical activity of the sinus node. The
interval between the first sinus beat and the PVC plus the interval between the PVC and the
next sinus beat equals two normal sinus intervals (Fig. 64-1). Occasionally, PVCs may be
interpolated between two sinus beats (i.e., produce no pause), and rarely, PVCs may penetrate
and reset the sinus node.
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FIGURE 64-1 Multiform premature ventricular complexes (PVCs). A, PVC followed by a compensatory pause. B, A
recording from the same individual shows an interpolated PVC of a different morphology. Tracings are from lead I. N = normal
sinus beat; V = premature ventricular beat. Intervals are given in milliseconds.
PVCs may be isolated or occur in groups. Two consecutive PVCs are termed a couplet. Three
or more consecutive PVCs at a rate of 100 beats per minute or higher are termed ventricular
tachycardia (VT). Single PVCs may occur sporadically or as bigeminy (every other beat is a
PVC), trigeminy (every third beat is a PVC), or higher-order periodicities. A patient may
manifest PVCs with two or more different morphologies, in which case the ectopy is termed
multiform. Single PVCs, regardless of whether they occur sporadically or in a periodic pattern,
are sometimes referred to as “simple” ventricular ectopy, whereas multiform PVCs, closely
coupled PVCs (so-called R-on-T phenomenon), ventricular couplets, and nonsustained VT are
referred to as “complex” ventricular ectopy. Fusion beats result from simultaneous activation of
the ventricle by a normally conducted supraventricular beat and a concurrent PVC and have a
morphology with some similarities to the supraventricular and ventricular beats.
Epidemiology
Ventricular ectopy is exceedingly rare in infants but increases in frequency with age. PVCs
occur in patients with and without structural heart disease. Holter monitoring (Chapter 61)
reveals at least one PVC in 40 to 75% and complex ventricular ectopy in 5 to 10% of normal
adults. PVCs occur with greater frequency and complexity in patients with structural heart
disease, especially ischemic and valvar heart disease and idiopathic cardiomyopathy. PVCs
may also occur in the setting of drug toxicity (e.g., digitalis intoxication) or electrolyte
disturbances (e.g., hypokalemia).
Prognosis
In general, the frequency and complexity of PVCs are not associated with an adverse outcome
in patients without structural heart disease. Rare exceptions do exist, however. In very
occasional patients without structural heart disease, single but repetitive PVCs originating from
the Purkinje network or right ventricular outflow tract can initiate ventricular fibrillation (VF). In
some patients with frequent PVCs originating from the right ventricular outflow tract (10 to 40%
of all ventricular beats), a secondary cardiomyopathy may develop that can be reversed by
ablating the arrhythmogenic focus.
Among patients with a previous myocardial infarction (MI; Chapter 72), frequent (>10 PVCs per
hour) and complex ventricular ectopy is associated with an increased risk for death. This risk is
concentrated in patients with depressed left ventricular function, however. Likewise, in patients
with valvar heart disease, sudden death is rare when ventricular function is normal (e.g.,
uncomplicated mitral valve prolapse), but risk increases when complex ventricular ectopy is
observed in patients with depressed left ventricular function. R-on-T PVCs may be more likely
than later coupled PVCs to result in VF or polymorphic VT. This relationship is weak, however,
and has limited prognostic utility.
Sustained re-entrant ventricular arrhythmias probably result from the interaction of a critically
timed triggering event (PVC) with an appropriate substrate (myocardial scarring resulting in
mechanical and electrical ventricular dysfunction). Because of the high prevalence of ventricular
ectopy in patients with structural heart disease, however, the predictive value for future events
is low. Even among patients for whom PVCs indicate a poor prognosis, antiarrhythmic drug
therapy aimed specifically at suppressing PVCs does not provide benefit. Suppression of PVCs
with encainide, flecainide, or moricizine results in a significant increase in mortality in patients
with frequent PVCs after MI.
Treatment
Because there is no evidence that treatment directed at suppressing PVCs improves overall
mortality, the primary indication for treatment is to relieve symptoms. Although most PVCs
are asymptomatic, in some patients they may result in troubling palpitations. Frequent PVCs
can also cause a pounding sensation in the neck secondary to cannon a waves from
atrioventricular dissociation. Because PVCs result in reduced stroke volume, patients with
frequent PVCs may occasionally have fatigue, exertional intolerance, dyspnea, and
lightheadedness.
Most patients with symptomatic PVCs in the absence of structural heart disease can be
managed with a β-blocker such as atenolol (25 to 100 mg/day) or metoprolol (50 to 200
mg/day). Class I or class III antiarrhythmic drugs may be considered, but the potential for
proarrhythmia and organ toxicity must be weighed. An alternative to antiarrhythmic drug
therapy for highly symptomatic patients, particularly those without structural heart disease
whose PVCs originate from the right ventricular outflow tract, is radiofrequency catheter
ablation of the arrhythmogenic focus (Chapter 65).
PARASYSTOLE
Ventricular parasystole results when an automatic focus arises from the ventricles and fires
independently of supraventricular impulses conducted through the atrioventricular node.
Classically, a surrounding region of depressed conductivity protects the focus by creating a
complete entrance block that prevents supraventricular beats from resetting the focus.
Independence of the parasystolic focus from the underlying rhythm is shown by variable
coupling intervals between the ectopic beats and the preceding sinus beats and a fixed
minimum time interval between PVCs, with any longer interectopic intervals being integral
multiples of this minimum interval (reflecting an exit block from the parasystolic focus) (Fig. 64-
2).
FIGURE 64-2 Parasystole: sinus rhythm with a competing ventricular parasystolic focus. N = normal sinus beat; N* =
timing of normal sinus discharge (occurring during the ventricular refractory period); V = ventricular parasystolic beat; V* =
timing of ventricular parasystolic discharge (occurring during the ventricular refractory period). Intervals are given in
milliseconds.
The entrance block surrounding the parasystolic focus can be partial rather than complete, so
conducted supraventricular beats may influence depolarization of the parasystolic focus and
either delay or accelerate its next discharge. Clinically, parasystole may be manifested as
sporadic PVCs or as bigeminy or trigeminy.
ACCELERATED IDIOVENTRICULAR RHYTHM
Definition and Electrocardiographic Features
An accelerated idioventricular rhythm (AIVR) refers to an ectopic ventricular rhythm

characterized by three or more consecutive PVCs occurring at a rate faster than the normal
ventricular escape rate of 30 to 40 beats per minute but slower than VT. No single rate
differentiates “fast” AIVR from “slow” VT, however. In general, the upper rate limit for AIVR is
100 to 120 beats per minute. Because AIVR has different prognostic and therapeutic
implications than VT does, it is important to recognize AIVR's gradual onset, acceleration
(“warm-up”), and deceleration before termination (consistent with an automatic mechanism) as
compared with the paroxysmal initiation and abrupt termination characteristic of re-entrant VT.
AIVR is generally brief (<1 minute) and is suppressed when the sinus rate exceeds its rate.
Clinical Manifestations
AIVR occurs most often in patients with acute MI, particularly after reperfusion, and usually
resolves spontaneously. AIVR is also observed in patients with rheumatic heart disease, dilated
cardiomyopathy, acute myocarditis, hypertensive heart disease, digitalis toxicity, and cocaine
intoxication and in those without structural heart disease. It is generally benign, and because
most runs of AIVR are brief and asymptomatic, it requires no specific treatment. If patients with
left ventricular dysfunction do not tolerate AIVR because of the loss of atrioventricular
synchrony, increasing the atrial rate with intravenous (IV) atropine (1 mg) or by pacing
suppresses AIVR.
VENTRICULAR TACHYCARDIA
Definition
VT, which originates below the bundle of His at a rate greater than 100 beats per minute, is a
wide-complex rhythm that may be monomorphic (uniform) or polymorphic with beat-to-beat
changes in the QRS configuration (Fig. 64-3). Sustained VT persists for 30 seconds or longer or
requires termination because of hemodynamic instability. Sustained polymorphic VT is
generally unstable and often degenerates into VF. Sustained monomorphic VT may be stable
for long periods or, with faster rates or myocardial ischemia, may degenerate into polymorphic
VT or VF. Torsades de pointes (TdP), a particular form of polymorphic VT, has a characteristic
morphology (“twisting around a point”) and is associated with prolongation of the QT interval on
the surface ECG.
FIGURE 64-3 Ventricular tachyarrhythmias. A, Rhythm strip showing monomorphic ventricular tachycardia. B, Example of
polymorphic ventricular tachycardia. C, Example of ventricular fibrillation. All tracings are from lead V1.
Electrocardiographic Features
It is important to distinguish monomorphic VT from supraventricular tachycardia with aberrant

conduction because both are manifested as wide-complex tachycardias (Table 64-1). Features
on the surface ECG permit differentiation of VT from supraventricular tachycardia with an
overall accuracy that approaches 90%. Atrioventricular dissociation, which can be identified on
the surface ECG in 25% of VTs, strongly suggests a ventricular origin. The presence of a one-
to-one atrioventricular relationship does not imply a supraventricular origin, however, because
some patients with VT have one-to-one retrograde conduction from the ventricles to the atria
during tachycardia. Capture and fusion beats also suggest VT but are generally seen only
during slow VT. A capture beat represents activation of the ventricles by a supraventricular
impulse that conducts via the His-Purkinje system. This beat prematurely “captures” both
ventricles during VT and results in a single narrow QRS interposed between wide tachycardia
complexes. Other features of the surface ECG that permit differentiation of VT from
supraventricular tachycardia include QRS width, axis, and morphology. Though helpful in most
situations, these morphologic criteria are not 100% specific. An irregular wide-complex rhythm
with essentially a single QRS morphology raises the possibility of atrial fibrillation with
ventricular pre-excitation (Chapter 63), particularly in patients without structural heart disease.
TABLE 64-1 -- ELECTROCARDIOGRAPHIC CHARACTERISTICS OF VENTRICULAR

TACHYCARDIA
ATRIOVENTRICULAR RELATIONSHIP
Atrioventricular dissociation
Sinus capture beats
Fusion beats
QRS WIDTH
Left bundle branch block >160 msec
Right bundle branch block >140 msec
QRS AXIS
Extreme left axis (− 90 to − 180 degrees)

Right axis deviation in the presence of a left bundle branch block (+90 to +180
degrees)
QRS MORPHOLOGY
Right bundle branch block

Morphology in V1
Monophasic R wave
Biphasic (qR or RS)
Triphasic with R >
R′
Morphology in V6
R/S ratio <1
Left bundle branch block

Morphology in V1
Broad R wave (>30 msec)
Onset of R wave to nadir of S wave >60
msec
Notched downstroke in lead V1
Morphology in V6
QR or QS complex
Onset of R wave to nadir of S wave >100 msec in any precordial lead

Absence of RS wave in any precordial lead
Positive or negative precordial concordance
Other diagnostic measures include the response of the tachycardia to vagal maneuvers and
adenosine. Most VT is insensitive to vagal maneuvers, such as carotid sinus massage and the
Valsalva maneuver, and to adenosine, whereas most forms of supraventricular tachycardia
terminate or persist with a transient high-grade atrioventricular block in response to these
measures. Idiopathic right ventricular outflow tract tachycardia in patients with normal hearts
may also terminate with vagal maneuvers and adenosine, however.
Clinical Manifestations and Acute Treatment
Patients with monomorphic VT may initially be seen in sudden cardiac death (SCD) or with
symptoms of impaired consciousness, such as syncope or near-syncope. Associated
symptoms may include chest pain, dyspnea, and palpitations. Occasionally, patients with VT
and slow rates may be asymptomatic, and hemodynamic stability is unreliable in distinguishing
VT from supraventricular tachycardia.
Physical examination during VT may reveal signs of cardiogenic shock, including

pulselessness, apnea, cool extremities, and cyanosis, as well as hypotension or signs of heart
failure, such as pulmonary rales or jugular venous distention. Characteristic features during VT
include cannon a waves and variable intensity of S1, both of which result from atrioventricular
dissociation.
Acute therapy for VT depends on the degree of hemodynamic instability that accompanies the
arrhythmia. For stable VT in patients with left ventricular dysfunction, pharmacologic therapy
should be initiated with either IV amiodarone (150 mg administered over a period of 10 minutes,
followed by 1 mg/min over the next 6 hours, then 0.5 mg/min over an 18-hour period) or
lidocaine (bolus dose of 0.5 to 0.75 mg/kg, followed by additional boluses of 0.5 to 0.75 mg/kg
at 5- to 10-minute intervals, up to a maximal dose of 3 mg/kg and maintained with an infusion of
1 to 4 mg/min). If these drugs are ineffective, IV procainamide (maximal dose of 17 mg/kg) at
an infusion rate of 20 to 30 mg/min can be considered (Chapter 61). If pharmacologic therapy is
unsuccessful for hemodynamically stable VT, synchronized cardioversion with a direct current
shock may be required, beginning at 50 to 100 J and increasing to 360 J if necessary.
For patients with severe signs or symptoms during VT, such as chest pain or myocardial
ischemia, heart failure or shortness of breath, decreased level of consciousness, or
hypotension (systolic blood pressure <90 mm Hg), immediate synchronous cardioversion is
indicated, with subsequent IV antiarrhythmic therapy to maintain sinus rhythm. After
resuscitation from VT, the patient must be evaluated for a possible primary cause, such as
electrolyte imbalance, acid-base disturbance, hypoxemia, drug toxicity, and myocardial
ischemia.
Diagnosis and Treatment
Invasive electrophysiologic testing (Chapter 65) can guide pharmacologic or device-based

therapy (Chapter 65) in patients with sustained monomorphic VT and previous MI. In these
patients, a sustained ventricular tachyarrhythmia can be induced with programmed
stimulation in approximately 90%. When compared with antiarrhythmic therapy, an
implantable cardioverter-defibrillator (ICD) improves outcome in patients who are
hypotensive with their VT.
Ischemic Heart Disease
VT during acute MI (Chapter 72) may be monomorphic or polymorphic. Sustained ventricular

arrhythmias within the first 48 hours of an acute MI do not convey an increased risk for future
spontaneous arrhythmias. Coronary revascularization or antianginal medical therapy may be
sufficient to control arrhythmias resulting from acute ischemia. Antiarrhythmic medications, such
as lidocaine, are likewise useful to control recurrent sustained arrhythmias in this setting.
Polymorphic VT and VF can also result from coronary vasospasm, which may occur in diseased
or normal coronary arteries.
Sustained monomorphic VT occurs most frequently in patients with previous MI and depressed
left ventricular function. VT arises from the border zone of the MI, where viable myocytes
scattered within areas of fibrosis form a chronic substrate for re-entry. VT can occur days to
decades after the MI, with an incidence that declines with time. The strongest risk factors for
sustained ventricular arrhythmias late after MI are depressed left ventricular function and
increased frequency and complexity of ventricular ectopy. Patients with a left ventricular
ejection fraction less than 30% after MI have a nearly threefold increased risk for mortality or
arrhythmic events.
Patients with VT beyond the first 48 hours after MI have a high recurrence rate, with an annual
risk that approaches 30% in the absence of treatment. In general, coronary revascularization
does not reduce the risk for recurrent VT arising from a chronic substrate after a remote MI, and
therefore definitive antiarrhythmic therapy is required. Randomized trials have shown the
superiority of ICDs over antiarrhythmic medications in patients with sustained monomorphic VT
(Chapter 65). Catheter ablation may have an adjunctive role in controlling frequent ventricular
arrhythmias in selected patients.
Cardiomyopathy
Monomorphic VT and polymorphic VT occur in patients with nonischemic dilated

cardiomyopathy (Chapter 59). The signal-averaged ECG and invasive programmed stimulation
(Chapter 65) have limited sensitivity and specificity for predicting future risk in these patients.
VF, polymorphic VT, and to a lesser extent, monomorphic VT occur in patients with
hypertrophic cardiomyopathy as a result of myocyte disarray, fibrosis, or ischemia. ICDs are
effective for primary and secondary prevention in patients with hypertrophic cardiomyopathy.
ICDs implanted in high-risk patients, including those with syncope, nonsustained VT, a family
history of sudden death, or left ventricular wall thickness 30 mm or greater, have an appropriate
yearly discharge rate of 5% (patients with documented VF have an 11% annual discharge rate),
with most shocks occurring during sedentary periods. Other potential risk factors for SCD in
these patients are a blunted exercise blood pressure response and the presence of myocardial
bridging of the left anterior descending artery in children.
Arrhythmogenic Right Ventricular Dysplasia
Arrhythmogenic right ventricular dysplasia (ARVD) is a cardiomyopathy characterized by loss of

myocytes in the right ventricle with replacement by fibroadipose tissue. ARVD causes SCD in
adolescents and young adults, most commonly while the patient is sedentary, with a peak
occurrence between 30 and 40 years of age. Patients have a propensity for re-entry ventricular
arrhythmias involving diseased portions of the right ventricle, although dysplasia confined
primarily to the left ventricle may occur.
The condition may be sporadic or familial. In most families, inheritance shows an autosomal
dominant pattern with incomplete penetrance. ARVD has been linked to mutations in at least
four desmosomal genes: plakophilin-2 (PKP2), which is observed in 25% of unrelated
probands; plakoglobin, which is responsible for Naxos disease (ARVD, palmoplantar
keratoderma, and wooly hair); desmoplakin; and desmoglein-2. Desmosomes are protein
complexes that mediate mechanical coupling between cardiac cells; mutations in desmosomal
proteins cause cell death with subsequent fibrofatty replacement. The surface ECG of patients
with ARVD may show a terminal notch on the QRS complex (e wave) in lead V1, T wave
inversions in the anterior precordial leads, and an S wave duration of 55 msec or longer in V1 to
V3. A right bundle branch block is present in approximately 25% of patients. Echocardiography
(Chapter 53) and right ventriculography show abnormalities of the right ventricle, including
enlargement, wall motion abnormalities, decreased systolic function, and aneurysmal dilation.
Magnetic resonance imaging (Chapter 55) reveals fatty replacement of the right ventricle,
thinning of the right ventricular wall, and wall motion abnormalities. Endomyocardial biopsy may
show characteristic fatty replacement and fibrosis, particularly in the so-called triangle of
dysplasia—the right ventricular inflow tract, apex, and infundibulum. During invasive
electrophysiologic testing, multiple morphologies of monomorphic VT may be inducible, usually
with a left bundle branch block configuration.
Treatment of ARVD is an ICD, sometimes with the addition of an antiarrhythmic medication

such as metoprolol (50 to 200 mg/day) or sotalol (160 to 320 mg/day). Catheter ablation,
though not always curative, may ameliorate frequent ventricular arrhythmias.
Idiopathic Ventricular Tachycardia/Ventricular Fibrillation
Idiopathic monomorphic VT may be manifested as a sustained arrhythmia facilitated by

exercise or as repetitive nonsustained VT that occurs at rest. The most common form of
idiopathic monomorphic VT typically arises from the right ventricular outflow tract and has a left
bundle branch block morphology and inferior axis. A somatic mutation in the inhibitory G protein
(Gα2) is responsible for the right ventricular outflow tract tachycardia in some patients. Right
ventricular outflow tract tachycardia is due to cyclic adenosine monophosphate–mediated,
triggered activity resulting from intracellular calcium overload. Right ventricular outflow tract
tachycardia can be cured by radio frequency catheter ablation (Chapter 65). In 10% of patients,
tachycardia may originate from the left ventricular outflow tract. Identifying features of this form
of VT are its responsiveness to adenosine and its sensitivity to vagal maneuvers and IV
verapamil (10 mg). For patients reluctant to proceed with ablative therapy, consideration can be
given to treatment with β-blockers (e.g., metoprolol, 50 to 200 mg/day, or atenolol, 25 to 100
mg/day) or class IC antiarrhythmic medications (e.g., flecainide, 100 to 300 mg/day), provided
that there is no evidence of ischemic heart disease.
A less common form of monomorphic VT that also occurs in patients without structural heart
disease is fascicular re-entrant tachycardia. This arrhythmia usually originates in the left
ventricular region of the left posterior fascicle and has a right bundle branch block, left superior
axis morphology. The tachycardia terminates in response to verapamil (10 mg) but not
adenosine, vagal maneuvers, or β-blockers, and it is readily treated by radio frequency catheter
Chapter 64VENTRICULAR ARRHYTHMIAS from Goldman: Cecil Medicine on ... Página 10 de 26
ablation.
Idiopathic VF is initiated or triggered by extrasystoles that originate from the Purkinje system or
right ventricular outflow tract myocardium. The coupling interval of the extrasystole with the
preceding sinus beat may be short, intermediate, or long. Preliminary findings suggest that
successful mapping and ablation of the initiating extrasystole can eliminate the VF. Until these
data can be confirmed in a large number of patients, however, this approach should be
considered palliative and definitive therapy should include an ICD.
Long QT Syndrome
Congenital Long QT Syndrome
The congenital long QT syndromes (LQTS) are related to cardiac ion channel defects that affect
cardiac repolarization and result in prolongation of the QT interval and TdP. In the United
States, the estimated prevalence of LQTS is 1 in 7000. Traditionally, congenital LQTS was
classified into two forms: the Romano-Ward syndrome (more common; autosomal dominant
and associated with normal hearing) and the Jervell and Lange-Nielsen syndrome (autosomal
recessive and associated with congenital sensorineural deafness).
Molecular genetics has identified at least eight genes that account for genotypic designations
(representing 50% of patients with LQTS). LQT1 accounts for approximately 45% of genotyped
patients. The responsible gene, KvLQT1 (also known as KCNQ1), is found on chromosome 11
and encodes a potassium channel pore-forming α subunit. Coexpression of KvLQT1 with
KCNE1, which encodes the non–pore-forming minK β subunit, forms IKs, the slowly activating
component of the delayed rectifier potassium current. LQT2 (45% of genotyped patients) is due
to mutations in the HERG (or KCNH2) gene located on chromosome 7. HERG encodes a
potassium channel α subunit, and four HERG subunits assemble with MiRP1 (minK-related
protein; β subunit), which is encoded by KCNE2, to form IKr, the rapidly activating component of
the delayed rectifier potassium current. Failure of these potassium channels to activate
normally (“loss of function”) prolongs the action potential duration and provokes early
afterdepolarizations. LQT3 (5%) is due to a mutation in the gene SCN5A, which encodes the
cardiac sodium channel and is located on chromosome 3. Failure of this channel to inactivate
prolongs the action potential duration. Mutations in MinK and MiRP1 are also linked to
compromised potassium flux and inherited LQT5 and LQT6. The mutation responsible for LQT4
(chromosome 4) is found in ANKB, which encodes ankyrin-B, an adapter protein thought to
anchor ion channels to the cell membrane. Mutations in one allele (heterozygous condition) of
any one of the six known genes responsible for LQTS cause the Romano-Ward syndrome,
whereas homozygous mutations in KvLQT1 or minK or the compound heterozygous condition
(simultaneous heterozygous mutations in the two genes) results in the Jervell and Lange-
Nielsen syndrome, thus suggesting that mutation of a single allele is sufficient to produce QT
prolongation whereas mutation of both alleles is necessary to produce congenital deafness.
Two other ion channelopathies have been identified that result in multisystem diseases and
LQTS. Andersen-Twail syndrome (LQT7), which is associated with periodic paralysis, skeletal
abnormalities, and a prolonged QT interval, is due to mutations in KCNJ2, which encodes the α
subunit (Kir2.1) of the inward rectifier potassium channel. Timothy's syndrome (LQT8), which in
addition to being associated with a long QT interval can also be manifested as cognitive
impairment, autism, and webbing of the fingers and toes, is due to a splice variant of the gene
CACNA1, which encodes the α subunit (Cav1.2) of the L-type calcium channel.
The corrected QT interval (QTc) in LQTS is usually greater than 0.46 in men and 0.47 in
women (each associated with 100% specificity), although a third of affected individuals may
have QTc intervals that fall within the normal or nondiagnostic range. A QTc interval of 0.40 or
less in men and 0.42 or less in women virtually excludes LQTS. The QT interval fails to shorten
normally or may be prolonged with exercise in patients with LQT1 and to a lesser extent in
patients with LQT2. Other diagnostic features on the surface ECG include ST-T wave patterns.
In LQT1, the T wave may be broad based or normal with a late onset. Very young patients may
have a juvenile ST-T wave pattern. LQT2 patients generally have low-amplitude, bifid or
notched T waves, whereas LQT3 patients have either late-onset peaked/biphasic T waves or
asymmetrical peaked T waves. T wave alternans can also be observed in LQTS.
The genetic locus, QTc, and gender affect the prognosis. The most common trigger for a
cardiac event in patients with LQT1 is exercise (particularly swimming). In LQT2 patients, the
most frequent precipitant is emotional stress (auditory stimuli). Most events in patients with
LQT3 occur during sleep or rest, thus suggesting that they are at higher risk at slow heart rates.
Syncope and sudden death are more of a risk in individuals with mutations at the LQT1 locus
than in those with LQT2 and LQT3. Events are more frequent in women than men with LQT2
but are more frequent in men than women with LQT3. In general, a QTc interval of 500 msec or
longer is associated with a worse prognosis.
A genotype-specific approach to therapy may potentially improve the long-term outcome in

patients with congenital LQTS. Patients with LQT1 respond well to β-blockers (e.g., propranolol,
80 to 240 mg/day) and should refrain from strenuous physical activity. β-Blockers may also
have a role in patients with LQT2 because of their risk associated with auditory stimuli, but they
are less effective than in patients with LQT1. An ICD should be considered in high-risk patients,
including survivors of sudden death, those with recurrent syncope despite therapy with β-
blockers, and those with marked prolongation of the QTc interval or a family history of sudden
death from LQTS. Because of the unpredictable nature of the disease, it is not unreasonable to
broaden the indications for an ICD in selected cases. Although patients with LQT1 and LQT2
have higher rates of syncope, patients with LQT3 have the highest mortality rate, and ICDs
should be strongly considered, particularly in male patients and those with a QTc of 500 msec
or longer. β-Blockers may cause potential harm and should not be used in these patients.
Acquired Long QT Syndrome
Acquired LQTS, which predisposes to TdP, occurs more frequently in women, is usually related
to drugs that block the potassium channel IKr, and is potentiated by subclinical LQTS,
hypokalemia, hypomagnesemia, bradycardia, heart block, and heart failure. Evidence suggests
that there may be a genetic predisposition to these types of arrhythmias because sporadic
mutations and single-nucleotide polymorphisms in ion channel genes have been identified in
some patients. Offending agents may include antidepressants (most commonly tricyclics),
neuroleptics, antibiotics (macrolides and quinolones), antifungal agents, tamoxifen, methadone,
prokinetic agents, and class IA (quinidine, procainamide, and disopyramide) and class III
(sotalol, dofetilide, and ibutilide) antiarrhythmic medications. A liquid protein diet, starvation,
grapefruit juice, and central nervous system disease may also predispose to QT prolongation
and TdP. Drugs that interfere with the hepatic metabolism of agents that directly prolong the QT
interval by inhibiting one or more cytochrome P-450 isoenzymes (most commonly CYP3A4) can
be a particularly hazardous combination; such drugs include calcium-channel blockers,
macrolide antibiotics (e.g., erythromycin), antiretroviral agents, antifungal agents, selective
serotonin reuptake inhibitors, and cyclosporine. A polymorphism in the CYP2D6 isoenzyme,
found in approximately 7% of white and African American individuals, causes loss of function
and therefore impairs the elimination of drugs that prolong the QT interval and are metabolized
by CYP2D6, such as the antipsychotic drug thioridazine. Therapy for acquired LQTS is directed
at reversing the metabolic abnormalities or withholding the offending medication. Infusion of
magnesium and temporary pacing decrease the QT interval and prevent pause-dependent
arrhythmias, whereas isoproterenol is a temporizing measure used to increase the sinus rate.
Lidocaine (IV bolus, 1 to 2 mg/kg × 2, then an IV infusion at 2 to 4 mg/min), which tends to
shorten the action potential duration and decrease the QT interval, may also be used.
Brugada's Syndrome
Brugada's syndrome is typically seen in individuals without known structural heart disease. It is
thought to be responsible for the sudden and unexplained nocturnal death syndrome in
Southeast Asian men and may account for 20% of sudden deaths in patients with normal
hearts. The syndrome is associated with malignant ventricular arrhythmias, most commonly
polymorphic VT occurring during rest or sleep.
Brugada's syndrome is associated with an autosomal dominant mode of inheritance with

variable penetrance. In 20 to 30% of patients, mutations in the SCN5A gene (encodes the α
subunit of the sodium channel) result in a diminished inward sodium current, which leads to
heterogeneous loss of the action potential dome in the right ventricular epicardium, and ST
segment elevation.
The characteristic finding on the ECG is a coved right precordial ST segment elevation (at least
0.2 mV) followed by a negative T wave. However, right precordial ST segment elevation can
also be observed in normal subjects and in patients with other clinical conditions. The ECG
findings in Brugada's syndrome may be transient and can be provoked by sodium-channel
blockers such as procainamide and flecainide (approximate positive predictive value of 35%),
as well as by vagotonic agents and β-adrenergic blockade. Magnetic resonance imaging,
cardiac catheterization, and myocardial biopsy findings are normal. The diagnosis is supported
by ST segment elevation in two or more right precordial leads, a spontaneous episode of VF or
polymorphic VT, inducibility of VT on electrophysiologic testing, a family history of sudden death
before 45 years of age, or a history of syncope.
An ICD is the most effective treatment for symptomatic patients. Preliminary data suggest that
quinidine (1200 to 1500 mg/day) may have some therapeutic effect because of its inhibitory
effects on the transient inward current Ito, the current responsible for loss of the action potential
dome in these patients. Although asymptomatic patients with a spontaneous Brugada ECG
pattern have a more favorable prognosis than symptomatic patients do, they are still at
increased risk for sudden death as compared with the general population. Therefore, the
general recommendation for asymptomatic patients is to undergo an electrophysiologic study
and, for patients with an inducible ventricular arrhythmia, to receive an ICD.
Catecholaminergic Polymorphic Ventricular Tachycardia
Sporadic and familial forms of catecholaminergic polymorphic VT have been identified in

children and adults with normal hearts. Autosomal dominant missense mutations in the
myocardial ryanodine receptor gene, which encodes the ryanodine receptor (RyR2) that
controls calcium release from the sarcoplasmic reticulum during excitation-contraction coupling,
have been identified in patients with this arrhythmia. Mutant RyR2 has diminished binding
affinity for the binding protein (FKBP12.6), which stabilizes interactions between RyR2s by
coordinating opening and closing of the channels. Catecholamine activation of protein kinase A
and phosphorylation of RyR2 cause dissociation of FKBP12.6 from the channel; however, in the
presence of mutated channels, protein kinase A markedly augments calcium release (leak)
during diastole and thereby potentiates ventricular arrhythmias. An autosomal recessive form of
catecholaminergic polymorphic VT is due to mutations in the calsequestrin gene (CASQ2).
Patients can have bidirectional VT, polymorphic VT, or idiopathic VF. The arrhythmias are
precipitated by exercise, catecholamines, or emotional stress and are usually associated with
syncope or SCD. In children, the condition may be misdiagnosed as a seizure disorder.
Provocative testing with adrenaline (starting at 0.05 µg/kg/min and increasing to 0.10, 0.20,
0.30, and 0.40 µg/kg/min at 5-minute intervals) may unmask polymorphic ventricular ectopy or
nonsustained VT. Patients often respond to β-blockers (e.g., metoprolol, 50 to 200 mg/day;
propranolol, 120 to 240 mg/day), and strong consideration should be given to use of an ICD
because the disease is highly lethal and β-blockers are not fully protective.
Short QT Syndrome
A consistently short QTc interval (<320 msec), termed short QT syndrome (SQTS), is
associated with syncope and sudden death in people without structural heart disease. Three
forms of SQTS have been identified, all associated with gain-of-function mutations in three
different potassium currents, each of which results in accelerated repolarization and
abbreviation of the action potential, thereby shortening the QT interval. SQT1 is caused by a
mutation in the KCNH2 gene, which encodes the α subunit of IKr; SQT2 is caused by a mutation
in KCNQ1, which encodes the α subunit of IKs; and SQT3 is due to a mutation in the KCNJ2
gene, which encodes for the inward rectifier IK1. The ST segment may be abbreviated or
absent. The QT interval does not shorten with exercise and may even paradoxically shorten at
slow heart rates. Young patients may initially be seen with atrial fibrillation. SQTS occurs over
the entire spectrum of age. Secondary causes of a short QT interval should be considered,
including hyperkalemia, hypercalcemia, and acidosis.
Patients in whom SQTS is diagnosed should be treated with an ICD. Preliminary data suggest
that quinidine (800 mg/day) may have an adjunctive role in patients with mutations in KCNH2.
Postoperative Tetralogy of Fallot
Patients who have undergone surgical repair of the tetralogy of Fallot through a right
ventriculotomy are at an increased risk for sudden death and VT (Chapter 68). The tachycardia
is due to re-entry around the right ventriculotomy scar in the infundibulum. Catheter or surgical
ablation or resection is effective in preventing recurrent VT.
Bundle Branch Re-entry
Patients with dilated cardiomyopathy and disease in the His-Purkinje system are prone to the
development of a specific form of VT known as bundle branch re-entry, in which the right and
left bundle branches participate in a macro re-entrant tachycardia circuit, with one branch
involved in the antegrade limb and the other in the retrograde limb. The tachycardia typically
has a left bundle branch block morphology and is often associated with presyncope or syncope.
The diagnosis is made by invasive electrophysiologic testing, and the tachycardia may be cured
by catheter ablation of one of the bundle branches.
Digitalis Toxicity
Ventricular arrhythmias seen in patients with digitalis toxicity include single PVCs, nonsustained
VT, and sustained polymorphic or monomorphic VT. Some digitalis toxic rhythms are due to
triggered activity from the intracellular calcium overload that results from inhibition of Na + , K + -
ATPase. A characteristic digitalis toxic rhythm is bidirectional VT, characterized by a right
bundle branch block configuration and alternating right and left axis deviation. Therapy for
severe digitalis toxic arrhythmias includes infusion of digoxin immune Fab fragments, which
may be life-saving. Alternatives are class IB antiarrhythmic medications such as lidocaine (IV
bolus, 1 to 2 mg/kg × 2, then an IV infusion, 2 to 4 mg/min) or phenytoin (3.5 to 5 mg/kg
intravenously).
VENTRICULAR FLUTTER AND FIBRILLATION
Definition
VF is a malignant arrhythmia characterized by disorganized electrical activity resulting in failure

of sequential cardiac contraction and an inability to maintain cardiac output (see Fig. 64-3). If
not promptly terminated (Chapter 62), VF results in hypoxemia and eventually SCD. Ventricular
flutter is an extremely rapid, hemodynamically unstable VT that typically progresses to VF. The
evaluation and management of ventricular flutter should parallel that for VF.
Epidemiology and Pathobiology
With rare exceptions, VF occurs in patients with underlying structural heart disease, especially
ischemic heart disease with left ventricular systolic dysfunction. In patients resuscitated from an
episode of VF, it is imperative to identify the cause of the arrhythmia and to search for evidence
of an acute ischemic event. Patients who survive an episode of VF within 48 hours of acute MI
generally have a good prognosis, with a 2% recurrence rate at 1 year.
De novo VF can be caused by myocardial ischemia, which results in complex changes in the
electrophysiologic properties of the ventricle, including delays in conduction and changes in
refractoriness, that potentiate the multiple re-entrant wave fronts that characterize VF.
Alternatively, PVCs during the vulnerable period of ventricular repolarization (R-on-T
phenomenon) may initiate VF. Prolonged VT can result in hypotension and myocardial ischemia
and cause degeneration of VT to VF.
Electrocardiographic Features
The ECG during ventricular flutter is characterized by a sinusoidal QRS complex, without a
distinct ST segment or T wave, at a rate of 240 to 280 beats per minute. In contrast, VF is an
irregular rhythm with an undulating low-amplitude baseline without organized QRS complexes
or T waves.
SUDDEN CARDIAC DEATH
Definition and Epidemiology
Half of all cardiac deaths are sudden, and they account for approximately 300,000 deaths per
year in the United States. SCD is death from instantaneous, unanticipated circulatory collapse
within 1 hour of initial symptoms and is often, but not always, due to a cardiac arrhythmia. More
than 70% of all sudden natural deaths have a cardiac cause, and 80% of them are attributable
to coronary artery disease (Table 64-2). Approximately 70% of SCDs occur in men. As
compared with routine activities, vigorous exercise increases the immediate risk for SCD 17-
fold. In more recent years, the incidence of SCD has declined in parallel with the decrease in
coronary artery disease, probably secondary to a reduction in cardiac risk factors, more
effective secondary preventive measures, improved resuscitative efforts, and expansion of
emergency medical services. SCD has a diurnal pattern, with a primary peak in the morning
hours after awakening, from 8 to 11 am, and a secondary peak from 4 to 7 pm. β-Blockers
attenuate the morning peak pattern. SCD also shows a seasonal predilection, with a higher
incidence in December and January than in the summer months (in the Northern Hemisphere),
a trend that is attributed to colder temperatures, holiday stress, and shorter daylight hours.
Prodromal symptoms in the 2 weeks preceding collapse may include fatigue, dyspnea, and
chest pain. Risk factors for SCD are identical to those for coronary artery disease and include
age, male gender, hypertension, tobacco use, hypercholesterolemia, and left ventricular
hypertrophy.
TABLE 64-2 -- CAUSES OF SUDDEN CARDIAC DEATH

STRUCTURAL HEART DISEASE
Coronary artery disease

Myocarditis
Cardiomyopathy
Hypertrophic
Infiltrative (e.g., amyloidosis, hemochromatosis,
sarcoidosis)
Cardiac tumors
Valvular heart disease
Congenital heart disease
Anomalous origin of coronary arteries

Arrhythmogenic right ventricular dysplasia
WITHOUT STRUCTURAL HEART DISEASE
Long QT syndrome
Congenital
Acquired (e.g., drugs, electrolyte abnormalities)
Short QT syndrome
Brugada's syndrome
Catecholaminergic polymorphic ventricular tachycardia
Chest wall trauma (commotio cordis)
Wolff-Parkinson-White syndrome
Idiopathic ventricular fibrillation
Holter monitor data indicate that approximately 85% of the rhythms leading to SCD are
ventricular tachyarrhythmias, with the remaining 15% being bradyarrhythmias. Among
tachyarrhythmias, 75% are due to VT, either monomorphic (two thirds) or polymorphic (one
third), and 25% are due to TdP and primary VF. When VT precedes VF, it usually persists for
30 seconds to 3 minutes before degenerating into VF. By 4 minutes after collapse, VF is
identified in nearly 90% of SCD cases, whereas asystole is identified in 10%. As more time
elapses, asystole and pulseless electrical activity are identified in more than 40% of victims,
which suggests that these rhythms reflect prolonged hypoxemia. This prolonged hypoxemia
probably explains the lower long-term survival rates (1 to 4%) in SCD patients with these
arrhythmias, in contrast to an approximate 30% rate of survival to hospital discharge in patients
found to be in VF after a witnessed arrest (Chapter 62).
Pathobiology
Most cardiac arrest survivors have structural heart disease, with nearly 75% having coronary
artery disease. VF may be the first manifestation of coronary artery disease in 25 to 50% of
patients with ischemic heart disease. Only 20% of patients have evidence of a new Q wave MI
at the time of cardiac arrest, whereas a remote MI is present in 40 to 80% of victims. Acute
coronary occlusion is found in 50% of survivors of out-of-hospital cardiac arrest.
Of SCD survivors, 10 to 15% have a dilated cardiomyopathy that may be idiopathic or due to
viral myocarditis, sarcoidosis, hemochromatosis, or amyloidosis (Chapter 296). In patients with
dilated cardiomyopathy, the risk for SCD is related to symptomatic status. The annual mortality
in patients who are New York Heart Association functional class II is estimated to be 5 to 15%,
of which 50 to 80% is due to SCD. In patients who are functional class IV, the annual mortality
is 30 to 70%, of which 5 to 30% is arrhythmogenic. Another cause of SCD is valvar heart
disease. In younger patients, particularly those who sustain arrests during physical activity,
causes such as hypertrophic cardiomyopathy (with or without outflow obstruction), ARVD,
LQTS, anomalous origin of the coronary arteries, and Wolff-Parkinson-White syndrome should
be considered in those who can conduct rapidly over the accessory pathway during atrial
fibrillation. Other causes include acute myocarditis, cardiac tumors, and repair of congenital
anomalies such as transposition of the great arteries and tetralogy of Fallot (Chapter 68). In a
small subset of patients, including those with Brugada's syndrome, exercise-related
polymorphic VT, LQTS (congenital and acquired), SQTS, drug toxicity (including cocaine use),
and electrolyte disturbances, no structural heart disease is detected. More recently, it has been
appreciated that a nonpenetrating blow to the chest caused by bodily collision or a projectile,
such as a baseball or a hockey puck (commotio cordis; Chapter 113), can precipitate VF,
presumably by occurring during the vulnerable phase of the T wave.
Although SCD is not usually associated with an acute Q wave MI, transient ischemia often
precedes SCD. In patients with stable high-grade atherosclerotic plaques (>75% occlusion) but
no previous MI or unstable ischemia, VF may be due to coronary vasospasm. The true
prevalence and significance of ischemia in precipitating VF are unknown, especially in view of
the fact that ischemic ST segment changes are rarely present at the time of SCD. An acute
coronary thrombus may be observed in 50% of patients with preexisting coronary artery
disease, however. The electrophysiologic consequences of acute ischemia that ultimately result
in VF are mediated through acidosis, potassium efflux from the cell with membrane
depolarization, increased intracellular calcium, and an increase in adrenergic tone.
Diagnosis
See Chapter 62.
Treatment
A key element for success is bystander initiation of cardiopulmonary resuscitation (see Fig.
62-2), but the most important factor that determines the outcome of cardiac arrest secondary
to VF or pulseless VT is the time until defibrillation (Chapter 62).
Prognosis
Despite public education in bystander cardiopulmonary resuscitation and efforts to train

emergency medical technicians, early in-hospital mortality is 50 to 60%, and only 4 to 40% of
patients with out-of-hospital SCD survive to discharge from the hospital, thus underscoring the
importance of primary and secondary prevention. A third of deaths are attributable to heart
failure or cardiogenic shock; 90% of patients who recover from coma with meaningful function
do so by the third hospital day. Public access defibrillation with the automatic external
defibrillator, which recognizes VF and delivers high-energy shocks, allows laypersons and
nonmedical personnel to function as first responders and has the potential to have a positive
impact on survival.
A primary episode of SCD has a 10 to 30% 1-year recurrence rate. Identifiable reversible
precipitants of secondary VF include transient ischemia, possibly related to vasospasm;
hypokalemia resulting from diuretics; hyperkalemia secondary to renal failure, angiotensin-
converting enzyme inhibitors, prostaglandin inhibitors, or potassium-sparing diuretics;
proarrhythmia secondary to antiarrhythmic medications, tricyclic antidepressants, and
antihistamines; or substance abuse with drugs such as cocaine and amphetamines. SCD
related to acute ischemia in the absence of a previous MI is often associated with severe
proximal coronary artery disease, normal left ventricular function, and noninducibility (absence
of VT) during electrophysiologic study. In patients who undergo early defibrillation after an MI
and survive to discharge from the hospital, the 5-year survival rate is equivalent to that of age-,
sex-, and disease-matched controls.
Prevention
Primary Prevention of Sudden Cardiac Death
Primary preventive approaches to SCD involve the prophylactic treatment of patients identified
as being at high risk and generally include reduction or elimination of myocardial ischemia with
antianginal agents or coronary revascularization, or both (Fig. 64-4). Regardless of whether
residual ischemia is present, initial therapy in all patients without contraindications should
include a β-blocker, which reduces SCD and total mortality (25%) in survivors of MI. Results are
similarly persuasive in patients with heart failure who can tolerate β-blockers. The beneficial
effects of β-blockers in prevention of SCD occur independently of their limited effect on
suppression of PVCs. Angiotensin-converting enzyme inhibitors also reduce SCD and overall
mortality in survivors of MI with a left ventricular ejection fraction of 35% or less (Chapter 72).
Fish oil, tested in two randomized trials, does not appear to be beneficial.
FIGURE 64-4 Approach to a patient resuscitated from ventricular fibrillation. EF = ejection fraction; EP =
electrophysiology; ICD = implantable cardioverter-defibrillator; MI = myocardial infarction. *Diagnose and treat reversible causes
of ventricular fibrillation, such as drug toxicity and electrolyte imbalance. †Consider an electrophysiologic study. ‡ If the EF is
low, consider an ICD.
Antiarrhythmic drugs have proved to be proarrhythmic and often decrease survival (Table 64-3).
In contrast, amiodarone reduces arrhythmic deaths in MI survivors who have an ejection
fraction of 40% or less and either frequent PVCs or nonsustained VT, but it does not seem to
improve overall mortality.
TABLE 64-3 -- CLINICAL TRIALS FOR PREVENTION OF SUDDEN CARDIAC DEATH

Study Background/Premise Purpose Entry Criteria Design
PRIMARY PREVENTION AFTER MYOCARDIAL INFARCTION: DRUGS

CAST PVCs in survivors of Assess Asymptomatic/mildly 1. Primary
MI are a risk factor for whether symptomatic PVCs (≥6/hr) prevention
SCD suppression after MI
of PVCs with 2. Titration:
antiarrhythmic encainide
drugs flecainide
reduces risk moricizine
for SCD 3. Randomiz
suppressi
drug vs.
placebo
4. End point
SCD
SWORD Post-MI patients with Assess 1. Recent MI 1. Primary

decreased EF have whether D- prevention
increased mortality sotalol vs. 2. EF ≤40%
placebo 2. Randomiz
reduces D-sotalol
v
mortality placebo
3. End point
mortality
CAMIAT Frequent or repetitive Assess effect 1. Post-MI status 1. Primary

PVCs after MI of prevention
increase mortality 1–2 amiodarone 2. ≥10 PVCs/hr or
yr after event on risk for VF ≥one 3-beat run 2. Randomiz
amiodaro
vs. placeb
3. End point
resuscitat
VF or
arrhythmi
death
EMIAT Post-MI patients with Assess 1. Post-MI status 1. Primary

decreased EF are at whether prevention
increased risk for amiodarone 2. EF ≤40%
SCD reduces 2. Randomiz
mortality in amiodaro
post-MI vs. placeb
patients with
decreased EF
PRIMARY PREVENTION AFTER MYOCARDIAL INFARCTION/CORONARY ARTERY DISEASE:
DEFIBRILLATOR
CABG Patients with CAD, Assess effect 1. CAD 1. Primary

patch decreased EF, and of prevention
(+) SAECG are at prophylactic 2. EF ≤35%
increased risk for ICD at time of 2. Randomiz
3. (+) SAECG
SCD elective ICD vs.
CABG control
surgery on 3. End point
survival mortality
MADIT Post-MI patients with Assess 1. Previous MI 1. Primary

NSVT and decreased whether prevention
EF have 30% 2-yr prophylactic 2. EF ≤35%
mortality ICD vs. 2. Randomiz
3. NSVT
conventional ICD vs.
4. Inducible but conventio
medical
nonsuppressible VT medical
therapy
with antiarrhythmic therapy
improves
drug during EPS
survival 3. End point
mortality
MUSTT Post-MI patients with Assess 1. Previous MI/CAD 1. Primary

decreased EF are at whether EPS- prevention
high risk for SCD guided 2. EF ≤40%
antiarrhythmic 2. Antiarrhyt
3. NSVT
therapy drugs or I
4. Induced sustained vs. no
decreases
VT antiarrhyt
risk for SCD
therapy
3. End point
SCD
MADIT-II Post-MI patients with Assess effect 1. Previous MI 1. Primary

decreased EF are at of ICD on prevention
high risk for SCD survival 2. EF ≤30%
2. Randomiz
ICD vs.
conventio
therapy
3. End point
mortality
SCD- SCD is a major cause Assess 1. NYHA class II/III 1. Primary

HeFT of death in CHF relative CHF prevention
efficacy of
amiodarone 2. EF ≤35% 2. Randomiz
or ICD vs. conventio
conventional CHF thera
therapy vs.
amiodaro
vs. ICD
3. End point
mortality
DEFINITE Patients with Assess effect 1. Nonischemic 1. Primary

nonischemic dilated of ICD on cardiomyopathy prevention
cardiomyopathy are survival
at increased risk for 2. EF ≤35% 2. Randomiz
SCD conventio
3. PVCs/NSVT
therapy vs
ICD
3. End point
mortality
DINAMIT ICDs improve survival Assess 1. Recent MI (6–40 1. Primary

of high-risk cardiac benefit of ICD days) prevention
patients early after MI
2. EF ≤35% 2. Randomiz
ICD vs.
3. Impaired autonomic
control
function
3. End point
mortality
SECONDARY PREVENTION OF SUDDEN CARDIAC DEATH/HYPOTENSIVE VENTRICULAR TA

CARDIOVERTER-DEFIBRILLATOR
AVID Patients who survive Assess 1. VF 1. Secondar
VF or hypotensive VT relative prevention
are at increased risk efficacy of 2. VT and
for recurrence ICD vs. syncope/presyncope 2. Randomiz
amiodarone (EF ≤40%) ICD vs.
(majority) or antiarrhyt
sotalol on drugs
mortality 3. End point
mortality
CIDS Patients who survive Assess 1. VF 1. Secondar

VF or hypotensive VT relative prevention
are at increased risk efficacy of 2. VT and
for recurrence ICD vs. syncope/presyncope 2. Randomiz
amiodarone ICD vs.
3. Syncope and
on mortality amiodaro
spontaneous/induced
VT 3. End point
mortality
CASH Survivors of SCD are Assess 1. 1.

at high risk for relative Survivor of SCD Secondar
recurrent event efficacy of caused by VF/VT prevention

class I, II, and
III 2. Randomiz
antiarrhythmic propafeno
drugs and vs. metop
ICD vs.
amiodaro
vs. ICD
3. End point
mortality
AVID = Antiarrhythmics Versus Implantable Defibrillators; CABG = coronary artery bypass

graft; CAD = coronary artery disease; CAMIAT = Canadian Amiodarone Myocardial Infarction
Arrhythmia Trial; CASH = Cardiac Arrest Study Hamburg; CAST = Cardiac Arrhythmia
Suppression Trial; CHF = congestive heart failure; CIDS = Canadian Implantable Defibrillator
Study; DEFINITE = Defibrillators in Non-Ischemic Cardiomyopathy Treatment Evaluation;
DINAMIT = Defibrillator in Acute Myocardial Infarction Trial; EF = ejection fraction; EMIAT =
European Myocardial Infarct Amiodarone Trial; EPS = electrophysiology study; ICD =
implantable cardioverter-defibrillator; MADIT = Multicenter Automatic Defibrillator Implantation
Trial; MI = myocardial infarction; MUSTT = Multicenter Unsustained Tachycardia Trial; NSVT =
nonsustained ventricular tachycardia; NYHA = New York Heart Association; PVCs = premature
ventricular complexes; SCD = sudden cardiac death; SAECG = signal-averaged
electrocardiogram; SCD-HeFT = Sudden Cardiac Death in Heart Failure Trial; SWORD =
Survival With Oral D-Sotalol; VF = ventricular fibrillation; VT = ventricular tachycardia.
Most data regarding risk stratification have been derived from post-MI patients, in whom left
ventricular dysfunction (particularly an ejection fraction ≤35%) is the strongest independent
predictor of SCD. The combination of an ejection fraction less than 30% and 10 or more PVCs
per hour carries a greater risk for SCD than either risk factor does alone, but drug suppression
of ventricular ectopy does not improve the prognosis. However, electrophysiologic testing in
post-MI patients without sustained ventricular arrhythmias helps identify those who will benefit
from an ICD (Fig. 64-5).
FIGURE 64-5 Risk stratification for primary prevention of death in patients with ischemic and nonischemic
cardiomyopathy. Standard therapy may include aspirin, anti-ischemic therapy, lipid-lowering therapy, a β-blocker, or an
angiotensin-converting enzyme inhibitor (Chapter 72). EP = electrophysiology; ICD = implantable cardioverter-defibrillator; VT =
ventricular tachycardia.
ICDs offer no mortality benefit in acute survivors of MI with left ventricular dysfunction when
implanted between 6 and 40 days after the event. [1] However, ICDs reduce mortality in MI
survivors who have a low ejection fraction, nonsustained VT, and inducible sustained VT. ICDs
also reduce total mortality in patients who are risk-stratified solely on the basis of severely
impaired left ventricular function (ejection fraction ≤30%). [2,3] This effect is more pronounced
as the time from MI increases and remains apparent for at least 15 years after MI. In contrast,
ICDs do not decrease mortality in patients undergoing elective coronary revascularization who
have reduced left ventricular function and a positive signal-averaged ECG. These discrepant
results may be due to several factors, including the positive effects of revascularization on
outcome, the relative limitations of the signal-averaged ECG to stratify risk (vs. an
electrophysiologic study), and the high incidence of nonarrhythmic deaths (70%), which
overwhelms the 45% reduction in arrhythmic deaths by ICDs.
An ejection fraction of 35% or less in patients with heart failure is considered sufficient
justification to proceed directly to an ICD. [2,4] Patients with syncope and left ventricular
dysfunction are known to have rates of SCD and total mortality similar to those who survive
cardiac arrest. Sustained VT is induced in more than 40% of patients with syncope and
impaired left ventricular function secondary to coronary artery disease. Despite receiving ICDs
and having a high incidence of appropriate therapy, these patients have a considerably worse
prognosis than do those who are not inducible. Likewise, syncope in patients with nonischemic
dilated cardiomyopathy carries a poor prognosis.
Identification of polymorphisms linked to SCD will probably play a more prominent role in risk
stratification in the future. For example, a polymorphism in the SCN5A gene that results in a
substitution of tyrosine for serine (S1102Y) is found in 13% of African Americans and is
associated with SCD.
Prevention of Recurrent Sudden Cardiac Death
Most patients who have survived SCD should undergo a comprehensive evaluation of
myocardial function and coronary anatomy (see Fig. 64-4). Echocardiography is useful for
excluding hypertrophic cardiomyopathy and valvar heart disease (Chapter 53), magnetic
resonance imaging for diagnosing ARVD (Chapter 55), and myocardial biopsy for identifying
infiltrative diseases such as myocarditis, amyloidosis, hemochromatosis, and sarcoidosis
(Chapter 59). Coronary angiography should be performed to assess for the presence of
coronary occlusive disease and to exclude coronary artery anomalies (Chapters 56 and 68).
Myocardial perfusion scintigraphy provides complementary data for assessing ischemic burden
(Chapter 54). Left ventricular function can be assessed by contrast ventriculography,
radionuclide ventriculography, or echocardiography.
Sustained monomorphic VT is inducible by electrophysiologic testing (Chapter 61) in 40 to 50%

and polymorphic VT in 10 to 20%; in 30 to 50%, no sustained arrhythmia is induced. In patients
with ischemic heart disease and left ventricular dysfunction, inducibility of sustained VT carries
a poor prognosis. A low ejection fraction is associated with a poor prognosis, however,
regardless of whether sustained VT is inducible; patients who have an ejection fraction of 30%
or less and who are noninducible have a 25% arrhythmia recurrence rate at 1 year, whereas
noninducible patients with an ejection fraction greater than 30% have a 10 to 15% recurrence
rate. An ICD is routinely recommended in such patients unless electrophysiologic testing shows
a specific problem that can be cured by catheter ablation.
Coronary revascularization is the principal mode of secondary prevention in patients with

significant coronary disease and those in whom VT cannot be induced during electrophysiologic
study (Chapter 74). Revascularization is not effective, however, in preventing VT in patients
with sustained monomorphic VT secondary to scars from a previous MI; treatment with catheter
ablation may be effective in patients with hemodynamically tolerated VT. However, data
suggest that 3-year mortality rates in patients with stable VT are equivalent to rates in patients
with unstable VT. An ICD should be strongly considered in these patients.
Secondary prevention trials for sudden death survivors secondary to VF/VT or patients with
hemodynamically unstable VT in the aggregate show a 20 to 30% reduction in mortality at 3
years with ICD therapy versus amiodarone, which is the best antiarrhythmic therapy. Thus, an
ICD is the most effective therapy for primary and secondary prevention of SCD.
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Chapter 64VENTRICULAR ARRHYTHMIAS from Goldman: Cecil Medicine on MD...

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Goldman: Cecil Medicine, 23rd ed.

Chapter 64 – VENTRICULAR ARRHYTHMIAS

PREMATURE VENTRICULAR COMPLEXES

Definition and Electrocardiographic Features

ACCELERATED IDIOVENTRICULAR RHYTHM

Definition and Electrocardiographic Features

An accelerated idioventricular rhythm (AIVR) refers to an ectopic ventricular rhythm

It is important to distinguish monomorphic VT from supraventricular tachycardia with aberrant

TABLE 64-1 -- ELECTROCARDIOGRAPHIC CHARACTERISTICS OF VENTRICULAR

Left bundle branch block >160 msec

Right bundle branch block >140 msec

Extreme left axis (− 90 to − 180 degrees)

Right bundle branch block

Left bundle branch block

Onset of R wave to nadir of S wave >100 msec in any precordial lead

Clinical Manifestations and Acute Treatment

Physical examination during VT may reveal signs of cardiogenic shock, including

Diagnosis and Treatment

Invasive electrophysiologic testing (Chapter 65) can guide pharmacologic or device-based

Ischemic Heart Disease

VT during acute MI (Chapter 72) may be monomorphic or polymorphic. Sustained ventricular

Monomorphic VT and polymorphic VT occur in patients with nonischemic dilated

Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD) is a cardiomyopathy characterized by loss of

Treatment of ARVD is an ICD, sometimes with the addition of an antiarrhythmic medication

Idiopathic Ventricular Tachycardia/Ventricular Fibrillation

Idiopathic monomorphic VT may be manifested as a sustained arrhythmia facilitated by

Congenital Long QT Syndrome

A genotype-specific approach to therapy may potentially improve the long-term outcome in

Acquired Long QT Syndrome

Brugada's syndrome is associated with an autosomal dominant mode of inheritance with

Catecholaminergic Polymorphic Ventricular Tachycardia

Sporadic and familial forms of catecholaminergic polymorphic VT have been identified in

Postoperative Tetralogy of Fallot

Bundle Branch Re-entry

VENTRICULAR FLUTTER AND FIBRILLATION

VF is a malignant arrhythmia characterized by disorganized electrical activity resulting in failure

Epidemiology and Pathobiology

SUDDEN CARDIAC DEATH

Definition and Epidemiology

TABLE 64-2 -- CAUSES OF SUDDEN CARDIAC DEATH

Coronary artery disease

Anomalous origin of coronary arteries

WITHOUT STRUCTURAL HEART DISEASE

See Chapter 62.

Despite public education in bystander cardiopulmonary resuscitation and efforts to train

Primary Prevention of Sudden Cardiac Death

TABLE 64-3 -- CLINICAL TRIALS FOR PREVENTION OF SUDDEN CARDIAC DEATH

PRIMARY PREVENTION AFTER MYOCARDIAL INFARCTION: DRUGS

SWORD Post-MI patients with Assess 1. Recent MI 1. Primary

CAMIAT Frequent or repetitive Assess effect 1. Post-MI status 1. Primary

EMIAT Post-MI patients with Assess 1. Post-MI status 1. Primary

CABG Patients with CAD, Assess effect 1. CAD 1. Primary

MADIT Post-MI patients with Assess 1. Previous MI 1. Primary

MUSTT Post-MI patients with Assess 1. Previous MI/CAD 1. Primary

MADIT-II Post-MI patients with Assess effect 1. Previous MI 1. Primary