Sudden Cardiac DeathDR ABDULPGY3MEM

Definition

The natural death from cardiac causes, heralded by abrupt loss of consciousness within 1 hour of the onset of an acute change in cardiovascular status.

Epidemiology

300,000 cases per year 1-2/1000 Bimodal distribution

Causes

Long QT Syndrome Brugada Syndrome Hypertrophic Cardiomyopathy Arrhythmogenic Right Ventricular Dysplasia Commotio Cordis Coronary Anomaly

Long QT Syndrome

Autosomal Dominate- Romano-Ward Autosomal Recessive- Jervell and Lange-Nielsen Acquired 1/10,000

Presentation

Palpitations, Presyncope, Syncope, Seizures, or Cardiac arrest

Asymptomatic prolonged QTc Referred by family members Has a predilection for younger patient

http://askdrwiki.com/mediawiki/index.php?title=Image:LongQT045.jpg

http://www.torsades.org/

Diagnosis: Schwartz score

Therapy

NEJM 2008; 358:169-175

Brugada Syndrome

Autosomal dominate defect in cardiac Na channels- variable expression

0.4% US population Male predominance Average age of Dx=41

Texas Heart Inst J 2007;34:67-75

Presentation

Funny looking ECG SCD/Syncope Triggers

Fever Sleep Glucose/insulin Cocaine/ETOH Electrolytes

Unmasking Agents

Na channel blockers-ajmaline Calcium Channel blockers Beta blockers Nitrates Tricyclics SSRI

Prognosis & TREATMENT SCD or VF/VT Syncope

Hypertrophic Cardiomyopathy

defect in the myocardial contractile proteins

HCM is a familial disease

hallmark : myocardial hypertrophy that is inappropriate, often asymmetrical, and occurs in the absence of an obvious inciting hypertrophy stimulus.

Presentation

SCD DOE Syncope Heart failure Chest Pain Palpitation Asymptomatic

Physical Exam

Systolic Crescendo-decrescendo murmur LLSB/apex

Decreases w/ squatting, hand drip Increase w/ standing Bifid pulses EKG-LVH

Diagnosis

ECHO

Treatment

Pharmocologic Verapamil Beta blockers

ICD Class I- h/o sustained VT/VF One or more major risk factor

Ablation/Surgery

Arrhythmogenic Right Ventricular Dysplasia

Defective Desmosome Fibrofatty replacement of the RV myocardium 1:1000 Autosomal dominant -mcf

Presentation Asymptomatic Palpitation, syncope, atypical chest pain,

dyspnea Arrhythmias SCD

Exercise associated

Diagnosis

History Familial disease by histologyEKG Epsilon waves or QRS 110 ms+ in V1-V3

Biopsy Endomyocardial biopsy with fibrofatty replacementSevere isolated RV dilatation & dysfunction

Severe segmental dilatation of RVLocalized RV aneurysm (DK)

History Family history of SCDLBBB VT

Late potentials on SAECGTWI in V2 & V3 (no rbbb)

Mild isolated global RV dilatation and/or dysfunctionMild segmental dilatation of RV

Regional RV HKEcho/MRI

MINOR

Criteria for the Diagnosis of ARVD

MAJOR

Echo/MRI

EKG

2major or 1major + 2minor or 4minor

Treatment

ICD: Class I: History of sustained VT/VF Class IIa: extensive disease, LV involvement,

family members w/ SCD, syncope Amiodarone or Sotalol-Class IIA Ablation-Class IIA

Commotio Cordis

SCD due to low-impact precordial trauma Male predominance, young age High mortality Probability related to speed, time, hardness of

object and location of impact

Commotio cordis

Treatment CPR Shock

Prevention Safety baseballs Chest protectors

Follow up EKG Holter Echo

Congenital Coronary Artery Anomalies

Variation in the take off the Coronary Arteries Incidence 5.60.17% Deaths related to exertion

Pathophysiology Compression between the pulmonary artery

and aorta Acute angle take off Myocardial necrosis

Presentation

Angina Atypical chest pain Syncope Palpitation Dizziness SCD

Diagnosis

ECHO Cardiac MRI CT angiography Cardiac Cath

Military Significance

Treatment

Beta Blockers Surgery Coronary Stents Avoid strenuous activities

0/1-not advisable, 2/3 intermediate risk, 4/5-permitted

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