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Tehran Arrhythmia Center The Worst Scenario A 4 year old kid High heart rates first noted by parents at 20 months of age. Family physician detected rates as high as 220 bpm at that age. He was visited, treated and followed at two


  1. Tehran Arrhythmia Center

  2. The Worst Scenario • A 4 year old kid • High heart rates first noted by parents at 20 months of age. • Family physician detected rates as high as 220 bpm at that age. • He was visited, treated and followed at two large referral hospitals.

  3. 4 Yr-old Kid • An old ECG

  4. 4 Yr-old Kid • First echocardiogram was reported as enlarged LV sizes with good contraction and interpreted as ‘cardiomyopathy with normal EF’. Heart failure medications were prescribed. • Repeat echocardiograms one year later were reported as LV dysfunction with moderate MR. • Mitral repair surgery was performed at age 3 years, complicated by an apparently hypoxic brain damage. • The child was mentally and physically retarded and unable to talk or walk.

  5. 4 Yr-old Kid • He was referred by a pediatric cardiology fellow asking: ‘Isn’t it WPW?!’

  6. Hear Failure Survival Temporal Trends in Survival 5-yr mortality declined by 12% per decade. Levy et al. NEJM 2002, 347;1442

  7. Heart Failure Mortality Framingham: 80% of men and 70% of women under age 65 will die within 8 years. REACH: The overall median survival 4.5 yrs for women vs. 3.7 yrs for men.

  8. Arrhythmia-induced Cardiomyopathy • First reported as an isolated case almost a century ago* • Largely considered a rare cause of cardiac damage, however: • Over the past few years there have been several publications that have established beyond a reasonable doubt that this condition is much more prevalent. • Even more important is the fact that it is a reversible and curable cause of heart failure. *Gossage AM, Braxton Hicks JA. On auricular fibrillation. Q J Med 1913;6:435 – 440.

  9. Mechanisms • It can be seen in association with a variety of cardiac rhythm disorders: Atrial fibrillation Ventricular tachycardia Ectopic atrial tachycardia Isolated ventricular ectopies Atrial flutter Pacing at fast rates Incisional atrial tachycardia PMT PJRT JET Thyrotoxicosis WPW Glucagonoma

  10. Pathophysiology • LV dysfunction during the tachyarrhythmias has been attributed to myocardial stunning resulting from sustained fast ventricular rates. • High energy myocardial stores have been shown to get depleted including diminished Na-K-ATPase activity and lower myocardial ATP and Phosphocreatine stores. • Although the condition is typically reversible, it can return with “a vengeance” if the original clinical scenario is allowed to recur.

  11. Animal Experiments • In animal experiments, it has been observed as soon as 24 hours after rapid ventricular pacing. This deterioration continues for 3 – 5 weeks. • Within 48 hours of cessation of pacing, impaired ventricular function is noted to improve and returns to normal by 1 – 2 weeks. • It may not reverse completely in all cases. Tachycardia-Induced Cardiomyopathy: A Review of Literature. PACE 2005, 710-21.

  12. Atrial Fibrillation The most common cause of tachycardia-induced cardiomyopathy

  13. AF & Heart Failure • The relationship between atrial fibrillation and cardiomyopathy has been explored for several years. • Atrial fibrillation itself may be the cause of tachycardia-related or tachycardia- worsened cardiomyopathy. • An important finding in some studies is that cardiomyopathy with atrial fibrillation may occur even with apparently well controlled ventricular response rates. S. J. Asirvatham. J Cardiovasc Electrophysiol, Vol. 18, pp. 15-17, 2007

  14. DCM, AF and RVR Tehran Arrhythmia Center

  15. AV node ablation • Does not eliminate AF • Effective in controlling ventricular rate • Improves: • QoL • Exercise tolerance • Left ventricular function • No deleterious effect on survival • Induces dyssynchrony itself

  16. Pace and Ablate

  17. AVN ablation and PPM • Cons: • Permanent • Detrimental effects of RV pacing, especially if reduced LV function already • Still have thromboembolic risk • Continue to have loss of atrial contractile function

  18. Biventricular Pacing LV Tehran Arrhythmia Center RV Tehran Arrhythmia Center

  19. Biventricular Pacing Tehran Arrhythmia Center LV RV Tehran Arrhythmia Center

  20. AV Junctional Ablation RF LV Tehran Arrhythmia Center RV Tehran Arrhythmia Center

  21. Biventricular Pacing in AF Tehran Arrhythmia Center Tehran Arrhythmia Center

  22. AVJ Ablation and CRT

  23. Ablate and pace • Suitable for • AF with symptomatic rapid ventricular rate unresponsive to drug Rx, or when drug Rx not tolerated • Patients with a bradycardia indication for pacing • More suited to elderly (less requirement for generator changes and lead revision)

  24. AF Ablation & Heart Failure • We have data primarily from the AFFIRM and related studies that controlling the ventricular rate with continued anticoagulation is as good as or better than attempting to restore and maintain sinus rhythm. • However, there are data from typically nonrandomized trials that the quality of patients’ lives, atrial function, and ventricular function improve and perhaps mortality is reduced when successful ablation for atrial fibrillation is performed.

  25. Potentials inside Pulmonary Veins

  26. AF ablation: LA/PV Geometry

  27. PV Isolation

  28. PV potentials disappearing during radiofrequency current application

  29. Disappearance of PV Potentials

  30. Termination of AF during Burn

  31. Termination of AF during Burn

  32. Termination of AF during Burn

  33. Persistent Junctional Reciprocating Tachycardia (PJRT)

  34. PJRT

  35. Persistent Junctional Reciprocating Tachycardia

  36. PJRT

  37. Termination & Re-initiation

  38. RF Ablation

  39. Termination

  40. Sinus Rhythm

  41. Incessant Atrial Tachycardia

  42. Incessant Atrial Tachycardia

  43. Incessant Atrial Tachycardia

  44. Intra-cardiac Recordings

  45. Success Signal at High Crista

  46. Termination during Burn

  47. Sinus Rhythm

  48. Incisional Atrial Tachycardia/ Flutter 46 yr old woman with repaired ASD, incessant AT leading to heart failure

  49. Intracardiac Recordings

  50. Line of Low Voltage Double Potentials

  51. RF Lesions

  52. Termination during Burn

  53. A NEW SCENARIO PVC induced Cardiomyopathy

  54. Isolated Benign PVCs

  55. PVCs: Are they really benign? • Isolated premature ventricular contractions (PVCs) are common and occur in patients with any form of structural heart and valvular disease as well as in normal hearts when they are usually considered benign. • Frequent PVCs themselves sometimes can cause reversible LV dysfunction and PVC-induced cardiomyopathy.

  56. Outflow Tract VT/PVCs

  57. Frequent Ectopies

  58. Frequent Ectopies • At the first clinical encounter, patients often present with both PVCs and LV dysfunction, raising the question which condition came first. • Factors associated with the risk to develop PVC-cardiomyopathy have been in the focus of intense investigations and include the PVC burden, duration of symptoms, QRS width, site of origin, and others. Cardiomyopathy-inducing premature ventricular contractions: Not all animals are equal? Heart Rhythm, Vol 9, No 9, September 2012

  59. Success Site

  60. Loss of Ectopies Tehran Arrhythmia Center

  61. RFA for PVCs • Several studies have shown the reversibility of LV dysfunction after ablation of PVCs. • Can we use the results of this studies to make a case for targeting not just symptomatic but even asymptomatic PVCs in patients manifesting cardiomyopathy? • Should patients with NICM that meet current guidelines for undergoing prophylactic ICD implant be screened/ablated for asymptomatic PVCs first? Sanjay Dixit, MD. Heart Rhythm 2007

  62. AN EXTREME SCENARIO

  63. • 13 year old girl with progressive dyspnea during the last four years • Functional class III-IV • LVEF 15-20% • Frequent (almost incessant) ventricular arrhythmias • Unresponsive to intensive heart failure therapy and several anti-arrhythmic drugs including a combination of Amiodarone and Mexiletine. • Transplant candidate

  64. Presenting ECG

  65. ECG at Onset

  66. Success signal

  67. Termination

  68. Final Results Now in FC I without anti-arrhythmic drugs, LVEF 45%

  69. ANOTHER SCENARIO Tachycardia- Aggravated Cardiomyopathy

  70. • 40 year-old man with progressive dyspnea, now FC IV, on transplant list • Incessant ventricular ectopies and VT • Marked LV enlargement and dysfunction, LVEF 15% • Marked LV trabeculations (Non-compaction ?) • Frequent ectopies with normal LV function documented 11 years ago. RFA at that time had failed. • Biventricular ICD implanted at another center, obviously not working with almost incessant VT.

  71. ECG at Onset

  72. Anterolateral LV

  73. Disappearance of Ectopies during Burn

  74. Post-RF Rhythm

  75. Effective CRT Three years post-RFA, he is in FC I with LVEF of 30%

  76. FINAL SCENARIO Heart Failure with a Wide QRS

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