Management of Patients With Ventricular Arrythmias and the Prevention of Sudden Cardiac Death

Publication Date: August 1, 2018

Key Points

Key Points

Table 1. Table of Definitions of Commonly Used Terms in this Document

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Term Definition or Description
Ventricular tachycardia (VT)

Cardiac arrhythmia of ≥3 consecutive complexes originating in the ventricles at a rate >100 bpm (cycle length: <600 ms). Types of VT:

  • Sustained: VT >30 s or requiring termination due to hemodynamic compromise in <30 s.
  • Nonsustained/unsustained: ≥3 beats, terminating spontaneously.
  • Monomorphic: Stable single QRS morphology from beat to beat
  • Polymorphic: Changing or multiform QRS morphology from beat to beat.
  • Bidirectional: VT with a beat-to-beat alternation in the QRS frontal plane axis, often seen in the setting of digitalis toxicity or catecholaminergic polymorphic VT
Torsades de pointes (TdP)

TdP is polymorphic VT that occurs in the setting of a long-QT interval and is characterized by a waxing and waning QRS amplitude. It often has a long-short initiating sequence with a long coupling interval to the first VT beat and may present with salvos of nonsustained ventricular tachycardia (NSVT). The twisting of the points, although characteristic, may not always be seen, especially if the episode is nonsustained or if only a limited number of leads are available. TdP can result from bradycardia including high-grade atrioventricular (AV) block that leads to a long-short sequence initiating TdP.

Ventricular flutter

A regular ventricular arrythmia (VA) ≈300 bpm (cycle length: 200 ms) with a sinusoidal, monomorphic appearance; no isoelectric interval between successive QRS complexes.

Ventricular fibrillation (VF)

Rapid, grossly irregular electrical activity with marked variability in electrocardiographic waveform, ventricular rate usually >300 bpm (cycle length: <200 ms).

Sudden cardiac arrest (SCA) SCA is the sudden cessation of cardiac activity such that the victim becomes unresponsive, with either persisting gasping respirations or absence of any respiratory movements, and no signs of circulation as manifest by the absence of a perceptible pulse. An arrest is presumed to be of cardiac etiology unless it is known or likely to have been caused by trauma, drowning, respiratory failure or asphyxia, electrocution, drug overdose, or any other noncardiac cause.
Sudden cardiac death (SCD) Sudden and unexpected death occurring within an hour of the onset of symptoms, or occurring in patients found dead within 24 h of being asymptomatic and presumably due to a cardiac arrhythmia or hemodynamic catastrophe.
VT/VF storm VT/VF storm (electrical storm or arrhythmic storm) refers to a state of cardiac electrical instability that is defined by ≥3 episodes of sustained VT, VF, or appropriate shocks from an implantable cardioverter-defibrillator (ICD) within 24 h.
Primary prevention ICD ICD placement with the intention of preventing SCD in a patient who has not had sustained VT or SCA but who is at an increased risk for these events.
Secondary prevention ICD ICD placement in a patient with prior SCA, sustained VT, or syncope caused by VA.
Structural heart diseasea This term encompasses ischemic heart disease (IHD), all types of cardiomyopathy, valvular heart disease, and adult congenital heart disease.
Cardiac channelopathy Arrhythmogenic disease due to a genetic abnormality that results in dysfunction of a cardiac ion channel (e.g., long-QT syndrome [LQTS], catecholaminergic polymorphic ventricular tachycardia [CPVT]).
a The definition of this term may differ across publications.

Figure 1A. SCD Incidence and Total Events


Figure 1B. SCD and Clinical Subsets


Diagnosis

...gnosis...

...ncope...

Patients presenting with syncope for which VA is d...


...mportant Considerations in the Evaluat...


Assessment

...ssessment

...ECG and Exercise Testing...

...patients with sustained, hemodynamically sta...

...ith VA symptoms associated with exerti...

...h suspected or documented VA, a 12-lead ECG should...


...ry Electrocardiograp...

...lectrocardiographic monitoring is use...


...anted Cardiac Monit...

...ients with sporadic symptoms (incl...


...ninvasive Cardiac Imaging...

...ith known or suspected VA that may be a...

...atients presenting with VA who are s...


...markers...

...ts with structural heart disease, measurem...


...elling (Please refer to Cardiac Channelopathi...

...s and family members in whom genetic testing fo...


...aging: Cardiac Catheterization...

...s who have recovered from unexplained S...


...lectrophysiological S...

...ts with ischemic cardiomyopathy, noni...

...patients who meet criteria for ICD implan...

...siological study is NOT recommended for risk st...


Treatment and Prevention

...atment and Prevent...

...3. Pharmacological Characteristics of Avail...


...ological Prevention of S...

...n patients with heart failure with reduced eject...


...nd Revascularization Procedures in Patients With...

...ith sustained VA and survivors of SCA should...

...h anomalous origin of a coronary artery suspected...


...Arrhythmia Management...

...nts with monomorphic VT refractory to antiarrhythm...


Autonomic Modulation

...with symptomatic, non–life-threa...

...s with VT/VF storm in whom a beta bloc...


...ment of Cardiac Arre...

...opulmonary resuscitation (CPR) should b...

...with hemodynamically unstable VA that persist or...

...resenting with VA with hemodynamic instability...

...ts with polymorphic VT or VF with ST-elevati...

...wide-QRS tachycardia should be pr...

...ith hemodynamically stable VT, administration...

...n patients with a witnessed cardiac arrest...

...patients with polymorphic VT due to...

...patients with a recent MI who have VT...

...patients in cardiac arrest, administrati...

...ts with hemodynamically stable VT, adminis...

...with cardiac arrest, administration of hig...

...nts with refractory VF not related to Td...

...patients with suspected acute myocardial infarctio...

...ients with a wide QRS complex tachycardi...


Figure 2. Management of Sustained Monomorphi...


...Prevention of SCD in Patients with...

unstable VT (I, B-...

...stable VT (I, B-NR)573...

...nsvenous ICD provides intermediate...

...atients with ischemic heart diseas...


...Secondary Prevention in Patients With I...


...oronary Artery Sp...

...VA due to coronary artery spasm,...

...patients resuscitated from SCA du...

...uscitated from SCA due to coronary artery spa...


...Prevention of SCD in Patients with Ischemic Heart...

...th left ventricular ejection fractio...

...th LVEF of ≤30% that is due to ische...

...transvenous ICD provides high value in the pr...

...with NSVT due to prior MI, LVEF of ≤40%...

...nonhospitalized patients with NYHA...

...dicated for NYHA class IV patients with me...


...y Prevention of SCD in Patients With I...


...Patients With Ischemic Heart Disease...

In patients with ischemic heart di...

...rone (I, B-R)573...

...tiarrhythmic medications (I, B-NR)573...

...h ischemic heart disease and ICD shock...

...tients with prior MI, class IC antiarrh...

...patients with incessant VT or VF, an ICD sh...

...ith ischemic heart disease and sustained mon...


...atment of Recurrent VA in Patients With Isch...


...ic Cardiomyopathy (NICM)...

...with suspected NICM from myocardial infiltrat...

...ith suspected NICM, cardiac MRI with...

...ts with NICM who develop conduction...


...revention of SCD in Patients With NIC...

unstable VT (I, B-...

stable VT (I, B-NR)573

...with NICM who experience syncope pres...

...ients with NICM who survive a cardiac arrest,...


...ary Prevention of SCD in Patients With N...

...atients with NICM, HF with NYHA class II...

...ts with NICM due to a Lamin A/C mutation wh...

...h NICM, HF with NYHA class I symptoms and an L...

...tients with medication-refractory NYHA class IV HF...


...t of Recurrent VA in Patients With...

...s with NICM and an ICD who experience spontane...

In patients with NICM and recurrent...


...ondary and Primary Prevention of SCD in Patie...


...rhythmogenic Right Ventricular Cardiomyopa...

...ected first-degree relatives of patie...

...ents with suspected arrhythmogenic right...

...nts with arrhythmogenic right ventricula...

...s with arrhythmogenic right ventric...

In patients with a clinical diagnosis of arrhyth...

...th clinically diagnosed or suspected arrhy...

...patients with arrhythmogenic right ven...

...nts with clinical evidence of arrhythmog...

...s with arrhythmogenic right ventricular cardiomy...

...suspected arrhythmogenic right ventricular...

...asymptomatic patients with clinical...


...rophic Cardiomyopathy (HC...

...patients with HCM, SCD risk stratificat...

...atients with HCM who have survived an SCA...

...gree relatives of patients with HCM, a...

...patients with polymorphic VT or VF with ST-elevati...

...irst-degree relatives of patients with HCM due to...

...ts with clinically suspected or diagnosed HC...

...V wall thickness ≥30 mm (IIa, B-NR)573...

...CD in ≥1 first-degree relatives presumably caus...

...episodes of unexplained syncope w...

...aneous NSVT (IIa, C-LD)573...

...n abnormal blood pressure response wit...

NSVT (IIb, B-NR)57...

...l blood pressure response with exerci...

...th HCM and a history of sustained...

In patients with HCM, an invasive...

...an identified HCM genotype in the absence of SCD...


...or Clinical Features Associated With I...


...re 7. Prevention of SCD in Patients...


Myocarditi...

...n patients with life-threatening VT...

...with giant cell myocarditis with VF o...


...ardiac Sarcoid...

...ith cardiac sarcoidosis who have sustained...

...with cardiac sarcoidosis and LVEF >35% who ha...

...patients with cardiac sarcoidosis and LVEF...

...ents with cardiac sarcoidosis who ha...

...tients with cardiac sarcoidosis with freq...


...ure 8. Prevention of SCD in Patient...


HFrEF

...ith HFrEF who are awaiting heart transplan...


...VAD

...ients with an LVAD and sustained VA, an ICD c...


...Use After Heart Transplantation...

In patients with a heart transplant and sev...


...muscular Disorde...

...ients with neuromuscular disorders, primar...

...ents with Emery-Dreifuss and limb-girdle type...

...h muscular dystrophy, follow-up for developm...

...atients with myotonic dystrophy type 1 w...


...e 5. Neuromuscular Disorders Associated With He...


Cardiac Channelopathies

Cardiac Channelopat...

...iac Channelopathie...

...gree relatives of patients who have a causative...

...nts with a cardiac channelopathy and SCA, an ICD i...


...al Long QT Syndrome...

...ith long QT syndrome with a resting QTc...

...atients with symptomatic long QT syndrome in whom...

...LQTS and recurrent appropriate ICD sh...

...ts with clinically diagnosed LQTS, genetic cou...

...ents with suspected LQTS, ambulatory electrocar...

...c patients with LQTS and a resting QTc...

...tomatic patients with LQTS and a r...

...atients with LQTS, QT-prolonging medica...


...ommonly Used QT-Prolonging MedicationsHavi...


Figure 9. Prevention of SCD in Patie...


...10. Long-QT Syndrome Type 1...


Figure 11. Long-QT Syndrome Type 2


...e 12. Long-QT Syndrome Type...


...rcise-Induced Polymorphic VT in Catecholamine...


...gic Polymorphic Ventricular Tachycardia...

...ts with CPVT, a beta blocker is rec...

...with CPVT and recurrent sustained...

...with CPVT and with clinical VT or e...


...ugada Syndr...

...tic patients with only inducible type 1 Brugada e...

...atients with Brugada syndrome with sponta...

...atients with Brugada syndrome experiencing recu...

...nts with spontaneous type 1 Brugada elec...

...nts with suspected Brugada syndrome in the...

...with asymptomatic Brugada syndrome...

...patients with suspected or established Br...


...ention of SCD in Patients With Brugada Syndrome...


...ure 15. Brugada Syndr...


...y Repolarization “J-wave” Syndrome...

...tic patients with an early repolarization pattern...

...th early repolarization pattern on EC...

...ith early repolarization pattern on ECG...


Short QT Syndro...

...asymptomatic patients with a short...

...s with SQTS who have a cardiac arrest or sustaine...

...ents with SQTS and recurrent sustained VA, treatme...

...with SQTS and VT/VF storm, isoproterenol infusion...

...atients with SQTS, genetic testing may be cons...


Other Conditions

...r Condition...

VA in the Structurally Normal Hea...

...ients with symptomatic PVCs in an otherwise...

...with symptomatic VA in an otherwise no...


...low Tract V...

...tients with symptomatic outflow tract VA i...

...patients with symptomatic outflow tract VT...


...Muscle VA (PVCs and VT)...

...symptomatic VA arising from the papillar...


...Reentrant VT (Belhassen Tachycardia)...

...h verapamil-sensitive, idiopathic LVT related to...

...nts with sustained hemodynamically tolerated verap...

...n patients with recurrent verapamil-sensitive...


Idiopathic Polymorphic V...

...young patients (

...resuscitated from SCA due to idiopathic polymorp...

...ith recurrent episodes of idiopathic V...


...nduced Cardiomyopat...

...require arrhythmia suppression for s...

...with PVC-induced cardiomyopathy, pharmacologic...


...regnancy

...mothers with LQTS, a beta blocker should be...

...pregnant patient with sustained VA, electr...

...tients needing an ICD or VT ablation...


...er Patients With Comorbid...

...tients and those with significant comorbiditi...


...-Induced Arrhythmias...

Digoxi...

...tion of digoxin antibodies is recommended fo...

...-Induced QT Prolongation and TdP

...ents with recurrent TdP associated w...

...r patients with QT prolongation due to...

...or patients with TdP associated with acquired Q...

...Channel Blocker–Related Toxicity...

...nts taking sodium channel blockers who...

...ents with congenital or acquired LQTS, QT-prolong...


...ongenital Heart Disease...

...s with repaired complex congenital heart disease p...

...h adult congenital heart disease and complex...

...nts with adult congenital heart disease and hem...

...adult congenital heart disease with...

...with repaired tetralogy of Fallot physi...

...h repaired tetralogy of Fallot physiology and i...

...ients with adult congenital heart disease...

...lts with repaired severe complexity adult...

...with repaired moderate or severe com...

...s with adult congenital heart disease an...

...with adult congenital heart disease...


...ongenital Heart Disease: Risk Factors...


...re 16. Prevention of SCD in Patients With...


Subcutaneous Implantable Cardioverter-D...

...ts who meet criteria for an ICD who...

In patients who meet indication for an ICD...

...n patients with an indication for br...


...able Cardioverter-Defibrillator...

...an ICD and a history of SCA or sustained VA...

...ts at an increased risk of SCD but who ar...


...theter Ablat...

...h bundle-branch reentrant VT, catheter abla...

...structural heart disease who have fai...


...mortem Evaluation of SCD

...victims of SCD without obvious causes, a standa...

...relatives of SCD victims who were...

...s of SCD with an autopsy that implic...

...ms of SCD with a previously identified phenotype f...


...rminal Care

...ime of ICD implantation or replacement, and duri...

...with refractory HF symptoms, refractory sustaine...


...red Decision-Making...

...ts with VA or at increased risk for SCD,...

...sidering implantation of a new ICD or replacem...

...e 17. Incremental Cost-Effectiveness of ICD by...