Life Threatening EKG’s In The Office

Joseph A Manfredi, MD, FACC, FHRS

GHS Cardiovascular Symposium January 24th, 2015

Disclosures

Speaker Honorariums: STJM, Boston Scientific Advisory role: Medtronic Stock: Boston Scientific

Willem Einthoven 1903

Willem Einthoven’s Nobel Prize Lecture

December 11, 1925

PERICARDITIS

Wolff-Parkinson-White Syndrome

45 year old woman Presented with syncope No prior cardiac history Recently started on Azithromycin Prior history of a “seizure disorder”

LONG QT SYNDROME

What is a prolonged QTc ?

>0.44 s M vs. >0.46s F

Symptoms Range from SCD to syncope or near syncope Patients who have suffered a SCA have a

ominous prognosis with a 12.9 RR of experiencing another event.

Reference: 1. Moss AJ, Zareba W, Hall WJ, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation. 2000;101:616-623.

Beta-blocker Therapy for LQTS

17 Reference: 1. Moss AJ, Zareba W, Hall WJ, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation. 2000;101:616-623.

Management Beta-Blockers (Less helpful in LQT3) Exercise restriction Pace or Isoproterenol Avoid QT prolonging drugs www.qtdrugs.org Ischemia/electrolytes AICD Cervico-sympathectomy Beta-Blockers immediate post-partum

Copyright ©2007 American College of Cardiology Foundation. Restrictions may apply.

Sauer, A. J. et al. J Am Coll Cardiol 2007;49:329-337

Probability of Any Cardiac Event

Copyright ©2008 American College of Cardiology Foundation. Restrictions may apply.

Goldenberg, I. et al. J Am Coll Cardiol 2008;51:2291-2300

Suggested Risk-Stratification Scheme for ACA or SCD in LQTS Patients

Medications to Avoid Cardiac: Sotalol, Amiodarone, Quinidine,

Dofetilide, and Procainamide Psychiatric: Haldol, Fluoxetine, Sertraline Antibiotics: Flouroquinolones, Mycins Narcotics: Methadone

Torsades de Pointes

SHORT QT SYNDROME

Background

No evidence on whether specific triggers precipitate events

Mutations in at least 3 genes have been identified……. KCNQ1 (Iks), KCNH2 (Ikr), nd KCNJ2 (Ikl)

These are similar to LQTS but with gain of function as opposed to loss of function

Autosomal Dominant Inheritence EPS ? (usually shows easily inducible ventricular

Arrhythmias but it is not known if this is predictive of adverse outcomes)

When to Suspect SQT

Atrial fibrillation at a very young age Family member with short QT Family member with SCD EKG with tall/peaked T waves QT interval of < 350 ms at 60 BPM

15 year old presents to the ER with atypical chest pain

Brugada Syndrome

Brugada Syndrome Risk Stratification Original Brugada description Circ

1998;97;457-460. 63 patients 30% risk of cardiac arrest at 3 years EPS is predictive of high risk 60 –90 % inducible ICD usually advised

Brugada Syndrome

ST elevation V1 and V2, RBBB ECG changes are variable and

dynamic (can be provoked by sodium channel blocking agents – ajmaline, procainamide) M > F 3rd and 4th decades Worldwide, but common in SE Asia

Brugada Syndrome Genetics

30% with mutations in SCN5A Autosomal Dominant Usually a loss of function mutation

(this gene is also implicated in LQTS3) At least 28 different mutations

identified

Clinical Presentation

Syncope SCA Usually in males in the 3rd to 4th decade of life

(Reported cases of SCA in Neonates) Fever is a predisposing factor for SCA Patients with Syncope and a spontaneous Type I

ECG have a 6 fold higher risk of SCA than those without syncope and Type I ECG

BRUGADA EKG

Wilde, A. A.M. et al. Circulation 2002;106:2514-2519

ECGs from a 35-year-old male who has been successfully resuscitated

1 5

Brugada Syndrome Risk Stratification

High risk pts: baseline ST elevation and syncope –44% had cardiac arrest; therefore ICD

Intermediate risk patients: spontaneous ST elevation > 2mm without syncope –14% had cardiac arrest; treatment undefined (? ICD)

Low risk patient: diagnostic ECG only after provocation – 5% had cardiac arrest over 4 decades of follow up – reassurance – no ICD –avoid Na channel blockers and TCA’s

Presymptomatic screening of family members without resting ECG abnormalities is not useful

Sudden Cardiac Death

Rapid VF or PMVT frequently occurring at rest or during sleep Holter monitoring does not usually show frequent

PVC’s or NSVT

Bidirectional VT

Catecholaminergic Polymorphic Ventricular

Tachycardia (CPMVT)

CPMVT Background

Is one of the most lethal channelopathies if not recognized and treated.

Characterized by ventricular arrhythmias induced by emotional stress or exercise (present with syncope during exercise, similar to Long QT but with a normal EKG)

Onset usually in childhood and adolescence Caused by mutations in the cardiac ryanodine

receptor (RyR2) in the Autosomal Dominant form

Resting EKG is usually normal

Challenges in Diagnosing CPMVT

Exercise stress testing is an important part of a CPVT workup. - However, in as many as 20% of CPVT

patients, formal exercise stress testing will not produce ventricular ectopy.

During exercise stress testing, bidirectional VT with a beat-to-beat 180 degree rotation of the QRS complex is often observed.1

References: 1. Mohamed U, Napolitano C, Priori SG. Molecular and electrophysiological bases of catecholaminergic polymorphic ventricular tachycardia. J Cardiovasc Electrophysiol. 2007;18(7):791-797. 2. Kontula K, Laitinen PJ, Lehtonen A, Toivonen L, Viitasalo M, Swan H. Catecholaminergic polymorphic ventricular tachycardia: recent mechanistic insights. Cardiovasc Res. 2005;67:379-387.

16 year old boy Cardiac arrest while playing high

school basketball Successfully resuscitated No prior history of cardiac disease

Hypertrophic Cardiomyopathy

Sudden Death in Young Athletes

NEJM 2003;349:1064

Hypertrophic Cardiomyopathy Apical Variant

Hypertrophic Cardiomyopathy

Hypertrophic Cardiomyopathy

Risk Stratification Clinical: syncope, FH of SCD, younger

age Testing: VT-NS, Exercise decrease in

BP. LVH (septum > 30 mm), LVOT gradient > 30 mm Hg (NEJM 2003;348:295.)

3 Risk Factors 6%/yr SCD risk

Hypertrophic Cardiomyopathy

NEJM 2003;349:1064

14 year old boy Passed out while swimming

competitively Required CPR but no defibrillation No prior history of cardiac disease No family history of SCD

ARVD-Epsilon wave

ARVD - MRI

ARVD Treatment

Minimal data Ablation – multiple foci and high

rate of recurrence Antiarrhythmic drugs - ? Sotalol ICD Avoid competitive athletics

Thank you