CHAPER 11: SUDDEN CARDIAC DEATH

INTRODUCTION
CLINICAL DISEASE
1) Coronary Artery Disease
2) Cardiomyopathy
3) Congenital Heart Disease
4) Valvular Heart Disease
5) Cardiac Pacemaker and Conducting System Disease
6) Hereditary Channelopathies
 Sudden Arrhythmic Death Syndrome
 Ion Channel Disease
 Brugada Syndrome
 Early Repolarization Syndrome
 Long QT Syndrome
 Short QT Syndrome
 Catecholaminergic Polymorphic Ventricular Tachycardia
PREVENTION OF SUDDEN CARDIAC DEATH
Antiarrhythmic drug and device therapy
SUDDEN CARDIAC DEATH RESCUE
Pulseless Ventricular Tachycardia/ Ventricular Fibrillation
Pulseless Electrical Activity

INTRODUCTION:

1) Most episodes of sudden cardiac arrest at public places have V. Fib.

2) Arrest at public places have higher survival than at home.
3) Sudden cardiac arrest is more common among lower social economic
status
4) There is a circadian pattern of sudden cardiac death and acute
myocardial infarction. Usually occurring within the first few hours after
awakening from sleep due to increased sympathetic stimulation.
5) Bimodal peaks in sudden cardiac death: infancy (SIDS) and males age
>45.
6) Undiagnosed CAD is a major cause of sudden cardiac death in adults.
CLINICAL DISEASES:
1) Coronary artery disease
 most commonly due to an acute plaque rupture.
 A documented initial cardiac arrest rhythm of V. Fib (or shockable
rhythm) suggests that an ACS is the cause but is not conclusive.
2) Cardiomyopathy
Severe Left Ventricular dysfunction with reduced ejection fraction (EF <35) is
the best predictor of sudden cardiac death.
Cardiomyopathy with reduced EF regardless of cause or presence of
decompensation.
 The Dilated ventricles create islands of ventricular tissue that
depolarize and polarize at different rates (circus movement reentry).
 Hx of myocardial infarction or ischemia
 Left ventricular hypertrophy (hypertension or vascular disease)
 Conduction disturbances (RBBB and LBBB)
 V. Fib is the [mc] rhythm disturbance in heart failure patients
 NYHA II and III – [mc] sudden cardiac death vs.
 NYHA IV – [mc] pump failure
Hypertrophic cardiomyopathy is the [mc] cardiovascular cause of sudden
cardiac death in young athletes, accounting for a third of such events.
Indications for implantable cardioverter-defibrillator placement:
 Prior documented cardiac arrest
 Ventricular fibrillation
 Hemodynamically significant or non-sustained ventricular tachycardia
 Patients with a first degree relative who has had sudden cardiac
death
 Unexplained episodes of syncope
 A maximum left ventricular wall thickness >30mm
 An abnormal BP response to exercise in the presence of other RFs.
 High risk children with unexplained syncope
 Massive left ventricular hypertrophy
 FMHx of sudden cardiac death
Arrhythmogenic right ventricular cardiomyopathy is a hereditary form of
cardiac muscle disease characterized by right-sided heart failure. Ventricular
arrhythmia of right ventricular origin, syncope and sudden cardiac death.
 The ECG shows T-wave inversion in the right precordial leads (V1-V3)

Table 11-2 CHD commonly associated with sudden cardiac death: ALCAPA,
AS, CoA. ToF, TGA, Ebstein A, Single Ventricle
3) Congenital Heart Disease
 Sudden cardiac death is a frequent case of morbidity and mortality in
CHD patients who survive to adulthood.
 The most frequent coronary artery anomaly associated with sudden
cardiac death is anomalous origin of the left coronary artery from the
pulmonary artery [ALCAPA] syndrome. The left coronary artery
traverses between the aorta and the main pulmonary artery.
 The greatest risk of sudden cardiac death in children and adults with
congenital heart disease exists in those with left heart obstructive
lesions (e.g. Aortic stenosis and aortic coarctation) and cyanotic
defects.
 Most sudden deaths occur during exercise usually resulting to
Ventricular Fibrillation.
4) Valvular Heart Disease
 Hemodynamically sever aortic stenosis can cause effort-induced
dyspnea myocardial ischemia and ventricular arrhythmias, which can
trigger syncope and sudden cardiac death.
 [mc] of aortic stenosis are congenital bicuspid aortic valve (mid-
adulthood) and tricuspid aortic valve calcification (>70-80 yrs of age).
 A harsh, late-peaking systolic murmur at the upper-right sternal border
with radiation to the neck is a typical finding in hemodynamically
significant aortic stenosis.
5) Cardiac pacing and conducting system
 Sick sinus syndrome, a degenerative disorder, affects the heart’s
primary pacemakers and conduction systems and can cause
intermittent lightheadedness, syncope, or sudden cardiac death.
 Due to degradation of the sinoatrial node, may also involve AV node
and other conduction tissues.
 Lenegre’s disease – idiopathic sclerodegeneration of the AV node
and bundle branches
 Lev’s disease – fibrosis and calcification of the conduction system
 Symptomatic bradycardia is treated with pacemaker placement.
6) Hereditary Channelopathies
Sudden Arrhythmic Death Syndrome – characterized by sudden cardiac
death occurring out of hospital in relatively young adults [mc] men, often
during sleep or at rest, usually without any premonitory symptoms
(including syncope)and with no anatomic abnormality identified at autopsy.
Ion Channel Disease – Cardiovascular and genetic examination of first-
degree relatives show familial cardiac diseases “channelopathy” or “ion
channel disease”.
 e.g.. Brugada syndrome, Early repolarization syndrome (ERS), Long QT
syndrome (LQTS), Short QT syndrome (SQTS) and Catecholaminergic
polymorphic ventricular tachycardia (CPVT)
Brugada Syndrome – Brugada syndrome most commonly affects men and
consists of a prominent J-wave with a characteristic down sloping ST-
segment elevation in electrocardiogram leads V1-V3.
 Commonly looks like an RBBB and is associated with polymorphic
ventricular tachycardia that degenerates into ventricular fibrillation
and sudden cardiac death.
 Pathology: Autosomal Dominant, a sodium channel disorder.
 [mc] in south east asia called sudden unexplained nocturnal death
syndrome “bangungut – Philippines”, “pokkuri – Japan ” and “lai tai –
Thailand”
 IMPORTANT: identify the condition in ECG Figure 11-1 page 61
because the risk of sudden cardiac death is high and can be
prevented by internal cardioverter-defibrillator placement.
Early Repolarization Syndrome: It is [mc] seen in 100% of endurance
athletes with unestablished clinical significance.
 Classic ECG: prominent, notch-like J wave on the QRS down-slope,
followed by upsloping ST-segment elevation. (Fig 11-2)
Long QT Syndrome – the Long QT syndrome is characterized by prolongation
of the corrected QT interval (QTc), syncope, and sudden death caused by
torsades de pointes and ventricular fibrillation.
 Calculate: Bazett’s equation which corrects the measured QT interval
to a HR of 60bpm
 Prolongation of the QTc represents dispersion in ventricular
repolarization and can be hereditary or acquired (hypokalemia,
hypomagnesemia, hypocalcemia, anorexia, ischemia, CNS,
terfenadine-ketoconazole combination, antipsychotics or
antiarrythmics)
 Management: avoidance of QT-prolonging drugs and avoidance of high
intensity sports, as well as a cardiology referral.
 Tx: B Blockers as sudden death prophylaxis. Persistence of syncope
despite B-blocker Tx warrants implantable cardioverter-defibrillator.
 Autosomal Recessive: Jervell and Lange-Nielsen syndrome
 Autosomal Dominant: Romano-ward syndrome without nerve
deafness.
Short QT syndrome – an abnormally short QTc (<.34s) can be secondary to
hypercalcemia, hyperkalemia, acidosis, systemic inflammatory syndrome,
myocardial ischemia, or increase vagal tone or can be inherited.
 Classic ECG: early repolarization in inferolateral leads.
Catecholaminergic Polymorphic Ventricular Tachycardia
 Pathology: Defective myocardial cellular calcium handling
 S/Sx: exercise and stress related v. tach + syncope and sudden cardiac
death. [mc] presents with epilepsy as the cause of recurrent syncope.
 Classic ECG: none but! most individual exhibit unexplain sinus
bradycardia.

PREVENTION OF SUDDEN CARDIAC DEATH

 Prodromal symptoms are uncommon.
 [mc] symptoms, if present, chest dyscomfort, dyspnea and “not
feeling well”
 Screening: no sensitive and specific test. (options: early detection of  Problem: compromised cardiac output.
ventricular late potentials, inducibility of v.tach, presence of T-wave  Outcome: despite intervention bradyasystolic rhythms have poor
alternans. outcomes.
 The only opportunity for prevention is to recognize signs and  Bardycardia occurs frequently during cardiac arrest
symptoms of syndromes that place a patient at higher risk of sudden  To ensure that ventricular fibrillation is not masquerading as asystole,
cardiac death and to admit or refer such patient for proper evaluation rescuers can switch to another lead whenever a “flat line” is recorded
and prophylaxis. on the ECG during resuscitation or use US if available.
 Antiarrythmic drug and device therapy:
 Avoid: Class I antiarrythmics are proarythmogenic
 Implantable cardio-defib are superior vs. B-blocker and amiodarone

SUDDEN CARDIAC DEATH RESCUE

 [mc] is either pulseless V.tach or V.fib
 Survival is inversely related to duration of event. (onset and
termination of abnormal rhythm)
 EMS and In-hospital resuscitation systems are the most effective
rescue interventions.
 Early CPR, ACLS, Defibrillation and drug therapy.
 Early Defibrillation confers the best survival among the interventions.
 By-standers capable of using external defibrillators reduces mortality
by half compared to CPR alone.

Pulseless Ventricular Tachycardia/ Ventricular Fibrillation

 Two rhythms with highest survivability: V. tach and V. fib.
 Unwitnessed Asystolic patients usually have neurologic deficits.
 Exception: witnessed cardiac arrest with asystole due to increase
vagal tone or hypoxia of brief duration.

Pulseless Electrical Activity

 Defined as an organized rhythm without a pulse.
 Tx: directed at identifying and treating the underlying cause or
causes.
 Bradyasystole: defined as a ventricular rate <60 bpm or period of
absent heart rhythm. Can be with or without a pulse.
 Primary Bradyasystole: heart fails to general electrical activity to
generate ventricular depolarizations that maintain end organ
function.
 Secondary Bradyasystole: factors external to the heart eg. Hypoxia.