The 2014 Mayo Approach to the Management of HCM and Non-Compaction

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1 The 2014 Mayo Approach to the Management of HCM and Non-Compaction R A Nishimura MD MACC MACP Judd and Mary Morris Leighton Professor Mayo Clinic No disclosures or conflict of interest CP

2 Let s start with a case 52 y/o man Class III DOE Loud murmur 300 mg lopressor Gradient 100 mmhg CP

3 Next step? 1. More meds 2. Dual chamber PM 3. Septal ablation 4. Septal myectomy 5. AICD CP

4 Treatment of HCM Relieve Symptoms Prevent Sudden Death Dynamic LVO Obstruction CP

5 Relieve obstruction Improve diastolic filling Reduce MR Improve symptoms Gradient LAP CP

6 Hypertrophic Cardiomyopathy Medical therapy : symptomatic pt Intolerant Beta Blockers Ca Blockers Intolerant Disopyramide Continued symptoms Myectomy DDD pacing Ablation CP

7 Dual Chamber Pacing NSR PACE CP CP

8 New medicines and other cures always work miracles for awhile William Heberden 1877 CP

9 Hypertrophic Cardiomyopathy Dual chamber pacing update 2014 Placebo effect wears out 6 months Overall improvement < 30% patients Possible detrimental effect Long-term pacing : myocardial dysfunction Mayo data CP

10 Septal Reduction Therapy CP

11 Septal Reduction Therapy Septal Myectomy CP

12 Surgical septal myectomy for symptomatic HOCM is safe and effective In the hands of experienced surgeons Operative mortality 0.8% Gradient 3 mm Post-op NYHA % CP

13 Septal Myectomy: Mayo Data pts F/U 10 yrs 80 % Pts I-II III-IV 20 0 Before Postop CP

14 Myectomy and Survival Nonobstructive Myectomy (Mayo) Overall survival Same results for cardiac survival and sudden death Nonoperated obstructive P< Ommen et al: JACC, 2005 Years CP CP

15 Hypertrophic Cardiomyopathy It is clear that surgical myectomy will result in marked long-lasting symptomatic improvement in over 90% of patients with severe symptoms and obstruction CP

16 Septal Ablation The New Kid on the Block A localized heart attack CP

17 Septal Ablation CP

18 Hypertrophic Cardiomyopathy 150 mm Hg 150 mm Hg 0 mm Hg Baseline 0 mm Hg After ablation CP CP

19 Surgery now has no role in the management of HCM Ablation is the new gold standard for the 21 st century Myectomy is only an impediment to the development of alcohol ablation Heart 2006:92:1339 JACC 2004:44:2054 Br J Card 2006:13:58 CP

20 Septal Ablation Success rate Structural Anatomy Anatomy Coronaries CP

21 No Systolic Anterior Motion of Mitral Valve Ablation ineffective Fixed subaortic stenosis 8-10% of referrals CP

22 MR jet directed anteriorly Ablation ineffective Flail leaflet 7-8% CP

23 Septal Ablation Success Structural Anatomy Anatomy Coronaries CP

24 Septal perfusion: just right CP

25 Septal perfusion: too much CP

26 Septal perfusion:?????? CP

27 Septal Ablation There are a subgroup of patients in whom the targeted septum cannot be reached by septal perforators CP

28 Septal myectomy Pre Post CP

29 Septal ablation Pre Post CP

30 Septal Ablation Complicatons Short Term Follow-up CP

31 Complete Heart Block (10-18%) CP

32 Ventricular Fibrillation Incidence unknown Sudden unexpected CP

33 Septal Ablation Other acute complications Coronary dissection: 0-2.5% (1.8%) Large infarction:???? Tamponade:.8-5% (3%) Stroke: 1.1% Nagueh JACC 2001 Faber EJE 2004 Fernandez JACC CV Int 2008 Firoozi EHJ 2002 Qin JACC 2001 Ralph-Edwards JTCVS 2005 Alam J int C 2006 Baggish Heart 2006 CP

34 This needs to be done in an experienced center!!!! 25 y/o Ablation done Now on transplant list CP

35 Septal Ablation Clinical Outcome CP

36 Survival without Severe Sx Pts aged <65 yrs Myectomy 90% Ablation p= % 20 0 No. at risk Myectomy Ablation Sorajja et al, Circ 2008 Follow-up (yrs) CP CP

37 Septal Ablation 4 yr survival free of death, NYHA Class III/IV or myectomy : 76% 1 of 4 will not have benefit CP

38 Ablation Myectomy Elderly Co-morbidities Limited life-span Sedentary Younger Healthy Long life-span Active CP

39 Patients/year 250 Septal Reduction Therapy Mayo Clinic Myectomy Ablation Year CP

40 Treatment of HCM Relieve Symptoms Prevent Sudden Death CP

41 Can we identify those patients at risk for sudden death? CP

42 Risk Factors for Sudden Death Really Bad Arrest Sustained VT Bad FH HCM and Sudden death LVH > 30 mm Unexplained syncope Somewhat Bad NSVT BP drop TMET Gadolinium DE LVO CAD CP

43 AICD: Caveats 72 y/o with four episodes syncope over 5 years 18 y/o with one episode syncope 1 week ago CP

44 Let s finish with a case LV RV 54 y/o woman Atypical chest pain Normal exam CP

45 What would you do? 1. Right and left heart cath / bx 2. Observation only 3. AICD 4. Anticoagulation 5. Surgery CP

46 Noncompaction of the myocardium A failure of the normal embryologic development of the heart Myocardium noncompacted (ratio 2:1 of trabeculated to solid) Early studies High rate of sudden death, stroke, etc CP

47 Noncompaction Wide spectrum of prognosis Benign Malignant Systolic function Symptoms Family history CP

48 Noncompaction Wide spectrum of prognosis Benign Malignant Observe Reassure AICD Anticoagulation CP

49 The 2014 Mayo Approach to the Management of HCM and Non-Compaction R A Nishimura MD MACC MACP Judd and Mary Morris Leighton Professor Mayo Clinic No disclosures or conflict of interest CP

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