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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
45 year old woman Presented with syncope No prior cardiac history Recently started on Azithromycin Prior history of a “seizure disorder”
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
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
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
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
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
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
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 Treatment
Minimal data Ablation – multiple foci and high
rate of recurrence Antiarrhythmic drugs - ? Sotalol ICD Avoid competitive athletics