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Normal Heart VT

Normal Heart VT. Syndromes. Long QT Short QT Brugada Catecholaminergic Polymorphic VT-CPVT Idiopathic VF Short coupled TdP Lev-Lenegre Syndrome. Channelopathies ECG and the Action Potential. Case 1. 13 yo girl presents with syncope while swimming QTc ≥500 msec

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Normal Heart VT

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  1. Normal Heart VT

  2. Syndromes • Long QT • Short QT • Brugada • Catecholaminergic Polymorphic VT-CPVT • Idiopathic VF • Short coupled TdP • Lev-Lenegre Syndrome

  3. ChannelopathiesECG and the Action Potential

  4. Case 1 • 13 yo girl presents with syncope while swimming • QTc ≥500 msec • ß-blocker initiated with no further events • Presents five years later inquiring about stopping medications • Do you stop ß-blocker? • Is an ICD indicated?

  5. 1957 1963-1964 1958-1970 1971 1979 1991-2007 1st LQTS family reported Romano-Ward Syndrome 25 LQTS cases reported 1st treatment – left stellate ganglionectomy LQTS registry started 10 LQTS genes identified Long QT SyndromeHistory

  6. Long QT SyndromeOverview • Incidence: 1/7,000 • Presentation: mean age 8-14 years • Symptoms: • Syncope, palpitations, seizures, sudden death • Syncope in pediatric population should be considered malignant until proven otherwise • Arrhythmia: Torsades des pointes

  7. LQT 1Broad T waves

  8. LQT 2Bifid T waves

  9. LQT 3Prolonged ST segment

  10. Long QT SyndromeDiagnosis • ECG definition • QTc > 460 females • QTc > 450 males • Challenges • 25 to 50% of LQT 1, 2, & 3 individuals will have QTc ≤ 460 msec • Genetic testing has 3 to 5% false (+) rate • Epinepherine challenge • Useful in evaluating LQT1 (∆QT 30 msec) • Iks response to epinepherine in LQT1 impaired • NPV 93%, PPV 76%, Sens 92%, Spec 86% • Less useful when on beta-blockers

  11. LQTSGene Specific Triggers Lethal and Non-lethal CV Events Schwartz PJ et al. Circulation 103:89-95, 2001

  12. Aborted cardiac arrest Family history (< 50 y) of cardiac arrest or unexplained syncope History of “seizures” or congenital deafness Prolonged QTc ≥500 msec on ECG Positive genetic test Long QT SyndromeHigh Risk Features

  13. LQTSRisk of Cardiac Event (syncope, cardiac arrest, or sudden death) • Risk > 50% • QTc ≥ 500 msec: LQT1, LQT2, Male LQT3 • Risk 30-50% • QTc ≥ 500 msec: Female LQT3 • QTc < 500 msec: Female LQT2/3, Male LQT3 • Risk < 30% • QTc < 500 msec: LQT1, Male LQT2

  14. LQT Subtypes

  15. LQTSManagement Options • Lifestyle modification (IB) • Beta-blockers (IB) • Very effective LQT1, Moderate LQT2 • Minimal effect LQT3 • ICD plus BB • Cardiac arrest (IA) • Syncope / VT (IB) • Prophylactic in LQT2 or LQT3 (IIB) • Left stellate ganglionectomy (IIB)

  16. LQTResources • Cardiac Arrhythmias Research & Education (CARE) • www.longqt.org • Cardiac Arrest Survivors Network (CASN) • www.casn-network.org • International Registry for Drug Induced Arrhythmias • www.qtdrugs.com

  17. Case 1 Review • 13 yo girl with syncope during swimming and QTc ≥500 msec • Asymptomatic for 5 y on BB • Swimming…suggests LQT1 • High risk subgroup based LQT1 and QTc ≥500 msec • Recommendation • Continue BB given very effective in LQT1 • Consider ICD if has arrest, syncope, or VT

  18. Case 2 • 17 yo girl presents with atrial fibrillation • QT 268 msec at HR 69 • Mother, age 51, and brother, age 21 with QT intervals of <300 msec also • History, exam, and cardiovascular workup otherwise negative • First reported family • Cardiology 2000;94:99-102

  19. Short QT Syndrome1999 – Dr. P. Bjerregaard

  20. Short QTECG Characteristics • QT < 300msec • No significant QT change with HR ∆s • Short ST segment with tall, narrow peaked T-waves in V1-V6 • Reentrant arrhythmias • Other clues • Lone AF, VF • Family Hx of SCD

  21. Short QT • EP testing • Short atrial and ventricular refractory periods • Management • Pharmacological (small studies) • Only hydroquinidine effective in increasing QT • Fleicanide, sotalol, ibutilide ineffective • ICD experience (limited) • T wave oversensing/inappropriate shocks • Device selection (St.Jude – delay/decay)

  22. Case 2 Review • 17 yo girl with AF and short QT. Mother and brother with short QT. • Treated with quinidine • For atrial fibrillation suppression • QT prolongation via K+ channel blockade • Long-term follow-up unavailable

  23. Case 3 • 39y man c/o cp, palpitations, and presyncope • PMH: none • SH: married, carpenter, occasional beer • FH: (-) sudden death, arrhythmias, premature CVD • Normal cardiac markers, echo

  24. Brugada SyndromeOverview • Identified 1992 • Age spectrum - 2d to 84y • Mean age sudden death 40 ± 15y • Men > 5x risk of arrhythmic events • Prevalence • 5/10,000 - overall • #2 cause of death SE Asian men <40y • Dynamic but characteristic ECG changes • 1 in 5 have Na channel mutation (SCN5A)

  25. Brugada SyndromeDefinition • Type 1 pattern ECG in V1-V3 plus 1 of following: • Pharm conversion to Type 1 from Type 2/3 ECG • Na channel blocker (procainamide, fleicanide, ajmaline) • Documented VF/polymorphic VT • Family history of SCD < 45y • Inducible VT at EP study • Syncope • Nocturnal agonal respirations • ECG pattern only = Brugada pattern ECG but not Brugada Syndrome • Exclude other heart conditions

  26. Brugada pattern ECGST elevation V1-V3 • Type 1 (DIAGNOSTIC) • Coved ST elevation ≥ 2mm with negative T wave • sensitivity by moving V2/V3 from 4th to 2nd/3rd intercostal space • Type 2 • Saddleback ST elevation ≥ 2mm w/ ST trough ≥ 1mm • Positive/biphasic T wave • Type 3 • Coved/saddle ST elevation ≥ 2mm w/ ST trough < 1mm • Also reported in inferior leads and left precordial leads • Some individuals also had SCN5A mutation

  27. Utility of EP study Controversial 6-9% of healthy individuals of induced VF at EPS Brugada, +EPS associated w/ 8x risk Other conduction abnormalities QT prolongation (R > L precordial) Prolonged action potential duration in RV epicardium P, PR, & QRS PR prolongation associated with His-purkinje delay Brugada Syndrome

  28. Brugada Consensus ConferenceSpontaneous Type 1 ECG

  29. Brugada Consensus ConferenceSodium Channel Blocker Induced Type 1 ECG

  30. Case 3 Review • 39y man c/o cp, palpitations, and presyncope • Spontaneous type 1 ECG • “Asymptomatic” • Negative family hx • EP study (IIA indication) • Sustained VT with DES at 500 from RVA • No supraventricular arrhythmias induced • Normal AV node and His-Purkinje function • ICD was implanted (IIA indication) • Asymptomatic without events at 32 mo f/u • ** Most events occur at night - autonomic role? • Other tx options: ablation, quinidine

  31. Case 4 • 16 yo girl suddenly arrests running into store • History of exertional palpitations and syncope • Successful resuscitation by bystander nurse

  32. Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) • Clinical Features • Direct correlation with adrenergic stimulation (physical/emotional) • Threshold heart rate 120-130 bpm • Abnormal automaticity or triggered activity • Bidirectional VT • Symptom onset in childhood • Genetic mutations – Ryanodine / Calsequestrin

  33. CPVTGenetic Mutations • Calsequestrin (CASQ2) • Autosomal Recessive • Calcium storage protein w/in lumen of sarcoplasmic reticulum • Cardiac Ryanodine Receptor (RyR2) • Autosomal Dominant • Regulates Ca++ from sarcoplasmic reticulum • Delayed after-depolarizations • Associated with ARVC • RYR1 - malignant hyperthermia syndrome

  34. CPVTManagement • Anti-adrenergic treatment • Beta blockers are the mainstay of treatment • ICDs • B-blockers not always effective

  35. Case 4 Review • 16 yo with history of palpitations and syncope who collapses in store • Arrested 3 times en route to hospital • ICD implanted and atenolol started • 3 ICD revision procedures • 2 lead dislodgements resulting in inappropriate ICD therapies

  36. Case 5 • 24 yo man with recurrent syncope • Signs and symptoms • Recent decrease in exercise tolerance • Lower extremity edema • Mild elevation in liver transaminases • Family hx + for sudden death – paternal uncle • Telemetry strip below

  37. Arrhythmogenic Right Ventricular Cardiomyopathy - ARVC

  38. ARVCIndik JH et al. Indian Pacing Electrophys J. 2003:3:148 • Top picture: • Fibro-fatty replacement of the myocardium • Thin and enlarged RV wall. • Bottom picture: • Trichrome stain • Areas of mature fibrosis (F) and adipose tissue (A) within the epicardial (Epi) and mid-myocardial zones

  39. ARVC • Diffuse fibrosis of the RV wall with preservation of normal LV tissue • Fibrous tissue appears white • Normal cardiac tissue appears black • www.geneticheartdisease.org • Ventriculogram demonstrating fibrofatty infiltration • Indik JH et al. Indian Pacing Electrophys J. 2003:3:148

  40. ARVC • Signal-Averaged ECG -SAECG (below left): • Characteristic high-frequency low-amplitude late-potential • SAECG averages multiple QRS complexes that are then digitalized and filtered and further processed with spectral analysis to eliminate noise. • Late-potentials represent areas of delayed activation due to slowed conduction from either regions of scar or fibrosis  electrical substrate that initiates and perpetuates ventricular tachycardia.

  41. ARVC High Risk Features • Younger patients • Recurrent syncope • History of cardiac arrest or sustained VT • Clinical signs of RV failure or LV involvement • Patients with or having a family member with the high risk ARVD gene (ARVD2) • Increase in QRS dispersion ≥ 40 msec • QRS dispersion = max measured QRS minus min measured QRS • Naxos disease

  42. Case 5 Review • Diagnosis • Rhythm strip and ECG notable for epsilon waves and T wave inversion in right precordial leads • Risk • High risk features present – young age, recurrent syncope, signs of RV failure, family history of sudden cardiac arrest • Management • ICD implantation

  43. Idiopathic Ventricular Fibrillation • Sodium channel mutation • Short-coupled Torsades des Pointes • Normal QT interval with coupling interval of 1st ectopic beat < 300 msec • Prognosis poor with unproven tx (BB or CCB); ablation? • Lev-Lenegre Syndrome • Progressive cardiac conduction defect associated with bradyarrhythmias although tachyarrhythmias may also occur • Sodium channel defect

  44. Idiopathic Ventricular Fibrillation

  45. Lev-Lenegre Syndrome • Progressive Cardiac Conduction Defect • Acquired complete heart block • Idiopathic fibrosis and calcification of cardiac conduction system • Very rare • Sodium channel mutations (subtype-SCN 5A) • Often result in bradyarrhythmias although tachyarrhythmias may also result • Lev M. Anatomic basis for atrioventricular block. Am J Med 1964;37:742-8. • Lenegre J. Etiology and pathology of bilateral bundle branch block in relation to complete heart block. Prog Cardiovasc Dis 1964;6:409-444

  46. Hypertrophic Cardiomyopathy • #1 cause of SCA in athletes • > 1/3 of deaths • Often associated with physical activity • 60% high school age • >90% males • Genetic disorder left ventricular hypertrophy • First symptom often sudden death

  47. HCM - ECG

  48. HCM – Echowww.hcmny.org

  49. HCM Septum > 15mm Assymetrical (septum:posterior wall thickness > 1.5:1) Occasional family history No change with deconditioning Athletic Heart Septum < 15mm Symmetrical thickening No family history Resolves with deconditioning – 3 mo HCM vs. Athletic Heart

  50. Thank You Questions?

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