Alcoholic septal ablation

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SEPTAL ABALATIVE PROCEDURES IN HYPERTROPHIC CARDIOMYOPATHY

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Alcoholic septal ablation

  1. 1. Hypertrophic cardiomyopathy
  2. 2. Define • In adults • HCM is defined by a wall thickness ≥ 15 mm(>13 mm in first-degree relatives) in one or more left ventricular (LV) myocardial segments, whatever the imaging technique (echocardiography, cardiac magnetic resonance[CMR] or computed tomography), without any explained loading conditions • In children • Diagnosis of HCM requires an LV wall thickness more than two standard deviations greater than the predicted mean
  3. 3. Aetiology • Sarcomeric HCM (by mutations in cardiac sarcomere protein genes):40–60% • Unknown :25–30% • Genetic or non-genetic causes:5–10% • Metabolic disorders • Mitochondrial cardiomyopathies • Neuromuscular disease • Malformation syndromes • Infiltrative disease, endocrine disorders, heart disease and chronic use of drugs [anabolic steroids and hydroxychloroquine
  4. 4. Assessment by ECHO • The presence or absence of a left ventricular outflow tract(LVOT) obstruction must be assessed at rest and during physiological provocation, such as the Valsalva manoeuvre. The threshold remains at 30 mmHg for the instantaneous peak Doppler LV outflow tract pressure gradient at rest, and exercise echocardiography is not recommended in asymptomatic patients with a gradient > 50 mmHg at rest.
  5. 5. Syncope evaluation • ECG • Upright exercise test • 48-hour ambulatory ECG monitoring • Recurrent episodes of unexplained syncope: implantable loop recorder • Palpitations :48-hour Holter ECG • Electrophysiological testing :Not recommended for risk stratification or for the exploration of syncope; testing is indicated for the assessment of persistent or recurrent supraventricular tachycardia, for ventricular pre-excitation or for sustained monomorphic ventricular tachycardia.
  6. 6. Genetic counselling • The relations of proband • Post-mortem for deceased Proband • If no causative mutation was characterised in the proband with HCM, then family screening is solely based on cardiac screening with ECG and echocardiography, which should be considered in first-degree relatives aged ≥ 10years, and should be repeated every 1 or 2 years between 10—20 years of age and every 2 or 5 years thereafter, as delayed cardiac expression of HCM is observed quite often, even in adults • Mutation carrier without cardiac expression (preclinical phase of HCM), the guidelines mention that sporting activity may be allowed, taken into account the type of sport, the underlying mutated gene and the results of regular and repeated cardiac examinations.
  7. 7. Symptomatic • Still high-dose beta-blockers • verapamil if does not Beta blocker • Disopyramide maybe combined with beta-blockers to reduce the gradient in symptomatic
  8. 8. Symptomatic • Septal reduction • NYHA III—IV despite maximum-tolerated medical Rx and with a gradient > 50 mmHg; • Unexplained recurrent syncope with < 50 mmHg LOVT gradient • Morrow procedure in young with septal hypertrophy ≥ 17 mm • In case of indication for ICD, a dual-chamber ICD may be considered in patients with LVOT obstruction ≥ 50 mmHg, NSR and drug- refractory symptoms • Rx CHF • Rx for Afib
  9. 9. 7 risk of sudden death • Age • family history of sudden cardiac death at a young age • maximum LV wall thickness • left atrial diameter • LVOT obstruction • non-sustained ventricular tachycardia • unexplained syncope.
  10. 10. 5 Year risk of SCD for primary prevention using 7 risk factors • low risk (< 4%)-No ICD • intermediate risk (4—6%)-May be ICD • high risk (> 6%)-ICD 1. Age 2. Family history of sudden cardiac death at a young age 3. Maximum LV wall thickness 4. Left atrial diameter 5. LVOT obstruction 6. Non-sustained ventricular tachycardia 7. Unexplained syncope • http://www.doc2do.com/hcm /webHCM.html
  11. 11. Routine follow-up • 12-lead ECG and ECH every 12—24 months • Close F/U of symptomatic • A 48-hourambulatory ECG is recommended every 12—24 months in stable patients, every 6—12 months in patients in sinus rhythm with left atrial dimension ≥ 45 mm and whenever patients complain of new palpitations • CMR may be considered systematically every 5 years in clinically stable patients and every 2—3 years in patients with progressive disease • Exercise testing may be considered every 2—3 years in stable patients and every year in case of progressive symptoms
  12. 12. Reproduction • No gradient or mild gradient are low risk • Significant gradient across the LVOT posses risk and should be on Beta blocker with fetal monitoring
  13. 13. Lifestyle  Competitive sports activities are contraindicated  Watch on weight , dehydration and excess alcohol  Sexual activity  Normal  PDE-5 inhibitors avoided in LVOT obstruction  Close watch on Rx and side effects  Eligible for an ordinary driving licence  With ICD, follow EHRA and local recommendations  Except for heavy manual jobs with strenuous activity  Life insurance for children of the patients
  14. 14. Naked truth
  15. 15. Epidemiology •  Genetic disorder •  1:500 in the general adult population • Men= women  • Heterogeneous Geno and phenotypes
  16. 16. Spectrum • Myocardial disease • Genetic origin(acquired or inherited) • Asymptomatic  to heart failure arrhythmias and sudden death.
  17. 17. HCM vs. HOCM • HCM  No gradient at rest or at exercise • HOCM Outflow gradients are common in  HCM, present in 70% of patients at  rest or with physiological exercise
  18. 18. Risk • Annual mortality rate in obstructive HCM is ~ 4 %  • High risk  • Onset of  age • frequent unstained VT •  syncope •  resuscitated sudden death • Family history of sudden death • Effort angina • Effort dyspnea
  19. 19. ACC/ESC Consensus-2003 1. Cardiac arrest (ventricular fibrillation) 2.   spontaneous sustained VT 3. family history of premature sudden death 4. Unexplained syncope 5. LV thickness ≥ 30mm 6. Abnormal exercise blood pressure 7. Non-sustained ventricular tachycardia (on Holter monitoring)
  20. 20. Goal of Medical treatment • Reduce HR • Reduce contractility • Reduce oxygen consumption • Reduce filling pressure • Beta blocker • Verapamil • Disopyramide
  21. 21. Therapeutic options Without Obstruction With obstruction After medical Rx exhausted Dyspnoea-Beta  blocker and  diuretic ASA Myome ctomy 
  22. 22. 1st ALCOHOL SEPTAL ABALATION[ASA] • Nonsurgical  • Professor Ulrich Sigwart •  Royal Brompton Hospital • 1994 • Injection of 1 to 4 mL of 96% ethanol into the first septal perforator branch of the left anterior descending coronary artery to produce a basal  septal myocardial infarction and ultimately remodeling of the LV outflow tract
  23. 23. Map before ASA • Inject  Levovist and map the hypertrophy topology using TTE 
  24. 24. ASA in cath Lab  Pig tail catheter in the LV    ATW angioplasty guide in the first septal artery  OTW 1.5-20 mm balloon into 1st   septal artery   Injecting two boluses of  1-4 ml of 96%  into septal artery distal to  the balloon, 0.5 to 1.0 mL aliquots at 1 mL/min  .   Assess for   Chest pain  Cardiac enzymes  RBBB  Gradient reduction  VSD
  25. 25. Ablation Cath: Septal Perforator
  26. 26. Septal abalation
  27. 27. Alcohol septal ablation
  28. 28. • Pre-ablation • Post-ablation
  29. 29. Advantages of ASA 1. No chest opening 2. No CPB 3. Myomectomy is contra indicated
  30. 30. Eligibility criteria for ASA 1. Symptomatic even with optimum MM 2. Dynamic LVOT obstructioncaused by systolic anterior motion of the mitral valve (gradient30 mm Hg at rest or 50 mm Hg with provocation) 3. ventricular septal thickness > 15 mm but <25 mm 4. the absence of significant intrinsicmitral valve disease;
  31. 31. Complications of ASA 1. Mortality -1-4% 2.Conduction abnormalities are relatively common complications of PTSMA, with permanentright bundle branch block and transitory heart block in about50% and high-gradeAV block requiring permanent pacemakers in 5% to 20%. 1.completeheart block - monitoring for 4 to 5 days. 1.AWMMI due to ethanol reflux
  32. 32. Procedural success • 50% reduction in the peakLVOT gradient observed at rest or, after provocation with a final residualresting gradient of <20 mm Hg in the absence of death orneed for emergency surgery up to 3 months
  33. 33. Risk factors for CHB 1. Rapidadministration, large volumes of ethanol. 2. Smaller doses of ethanol (1 to2 ml) over longer time periods (5 to 10 min) has decreased theincidence of CHB 3. Risk factors for CHB • LBBB • first-degree atrioventricular block • female • volume of alcohol • number of septal perforators treated
  34. 34. Different mechanisms ASA Myectomy • Scar less• SCAR
  35. 35. Morrow procedure-30% of LV or 10% IVS • Cut 30% IVS or 10% LV mass
  36. 36. Bad days of myomectomy In the early 1990s, a time when myectomy was associated with relatively high mortality (up to 8%), dual-chamber pacing with short A-V delay was promoted as a surgical alternative for gradient and symptom relief
  37. 37. Survival benefit  Myectomy also promoteslong-term survival. Operated patients experience enhanced longevityindistinguishable from that expected in the general populationand superior to that of non operated patients with obstruction  After Myectomy, survival free from all-cause mortalityis 98%, 96%, and 83% at 1, 5, and 10 years, and survival freefrom HCM- related mortality (heart failure and sudden death)is 99%, 98%, and 95%, respectively.Therefore, surgicalseptal myectomy favorably alters the natural course of HCM,providing a reasonable expectation for normal or nearly normallife expectancy
  38. 38. Surgical Myectomy 0.5 0.6 0.7 0.8 0.9 1.0 0 2 4 6 8 10 I 1 30 II 2 24 III 48 7 IV 14 0 NYHA Pre PostNYHA Pre Post ObstructiveObstructive Obstructive Post-myectomyObstructive Post-myectomy Operative mortality 0.8% Ommen S et al. J Am Coll Cardiol 2005
  39. 39. Operative mortality 0.8% Gradient reduction 67.3% Post-op NYHA 1-2 94% Myectomy for severely symptomaticMyectomy for severely symptomatic HOCM is safe and effectiveHOCM is safe and effective Ommen S et al. J Am Coll Cardiol 2005
  40. 40. Ablation vs. Myectomy 4 Comparison Studies - 279 patients Procedural Mortality (%) 0.9 1.3 0 0.2 0.4 0.6 0.8 1 1.2 1.4 Myectomy Ablation Nagueh et al.Nagueh et al. JACCJACC 2001 38(6)2001 38(6) Qin et al.Qin et al. JACCJACC 2001 38(7)2001 38(7) Firoozi et al.Firoozi et al. Eur Heart JEur Heart J 2002 23(20)2002 23(20) Ralph-Edward et al.Ralph-Edward et al. CircCirc 20052005
  41. 41. Ablation vs. Myectomy 4 Comparison Studies - 279 patients Gradient reduction 71 7 75 15 0 10 20 30 40 50 60 70 80 Myectomy Ablation Nagueh et al.Nagueh et al. JACCJACC 2001 38(6)2001 38(6) Qin et al.Qin et al. JACCJACC 2001 38(7)2001 38(7) Firoozi et al.Firoozi et al. Eur Heart JEur Heart J 2002 23(20)2002 23(20) Ralph-Edward et al.Ralph-Edward et al. CircCirc 20052005
  42. 42. Ablation vs. Myectomy 4 Comparison Studies - 279 patients Symptom Improvement 2.97 1.31 3.17 1.55 0 1 2 3 4 Myectomy Ablation Nagueh et al.Nagueh et al. JACCJACC 2001 38(6)2001 38(6) Qin et al.Qin et al. JACCJACC 2001 38(7)2001 38(7) Firoozi et al.Firoozi et al. Eur Heart JEur Heart J 2002 23(20)2002 23(20) Ralph-Edward et al.Ralph-Edward et al. CircCirc 20052005
  43. 43. Ablation vs. Myectomy 4 Comparison Studies - 279 patients Procedural Mortality (%) 0.9 1.3 0 0.2 0.4 0.6 0.8 1 1.2 1.4 Myectomy Ablation
  44. 44. Ablation vs. Myectomy 4 Comparison Studies - 279 patients Non-fatal Complications (%) 2 13 0 2 4 6 8 10 12 14 Myectomy Ablation At least 5 VF arrests Nagueh et al.Nagueh et al. JACCJACC 2001 38(6)2001 38(6) Qin et al.Qin et al. JACCJACC 2001 38(7)2001 38(7) Firoozi et al.Firoozi et al. Eur Heart JEur Heart J 2002 23(20)2002 23(20) Ralph-Edward et al.Ralph-Edward et al. CircCirc 20052005
  45. 45. Dynamic ObstructionDynamic Obstruction MoreMore DiffuseDiffuse Valvular anatomyValvular anatomy AbnormalAbnormal NormalNormal BasalBasal LVOTO onlyLVOTO only MyectomyMyectomy MyectomyMyectomy Septal ablationSeptal ablation MyectomyMyectomy
  46. 46. What makes an ideal alcohol septal ablation candidate? 1. Basal septal bulge 2. SAM 3. Posterior MR 4. Moderate labile gradient 5. Comorbidities
  47. 47. Radiofrequency catheter septal ablation • Alternative
  48. 48. Coil embolization • Few number
  49. 49. Conclusion • Myomectomy for persistent LVOT obstruction is gold Standard care for HOCM when symptoms persist after optimal medical management to further improve in the quality of life and symptom and consistent reduction in left ventricular outflow tract gradient • ASA is an alternative treatment
  50. 50. Laugh loudly if you already know

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