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 CP1288794-1
Let s start with a case 52 y/o man Class III DOE Loud murmur 300 mg lopressor Gradient 100 mmhg CP1288794-2
Next step? 1. More meds 2. Dual chamber PM 3. Septal ablation 4. Septal myectomy 5. AICD CP1288794-3
Treatment of HCM Relieve Symptoms Prevent Sudden Death Dynamic LVO Obstruction CP1288794-4
Relieve obstruction Improve diastolic filling Reduce MR Improve symptoms Gradient LAP CP1288794-5
Hypertrophic Cardiomyopathy Medical therapy : symptomatic pt Intolerant Beta Blockers Ca Blockers Intolerant Disopyramide Continued symptoms Myectomy DDD pacing Ablation CP1288794-6
Dual Chamber Pacing NSR PACE CP980117-19 CP1288794-7
New medicines and other cures always work miracles for awhile William Heberden 1877 CP1288794-8
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 CP1288794-9
Septal Reduction Therapy CP1288794-10
Septal Reduction Therapy Septal Myectomy CP1288794-11
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 1-2 94% CP1288794-12
Septal Myectomy: Mayo Data 100 256 pts F/U 10 yrs 80 % Pts 60 40 I-II III-IV 20 0 Before Postop CP1288794-13
Myectomy and Survival 1.0 0.9 Nonobstructive Myectomy (Mayo) Overall survival 0.8 0.7 0.6 Same results for cardiac survival and sudden death Nonoperated obstructive P<0.001 0.5 0 2 4 6 8 10 Ommen et al: JACC, 2005 Years CP1160937-13 CP1288794-14
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 CP1288794-15
Septal Ablation The New Kid on the Block A localized heart attack CP1288794-16
Septal Ablation CP1288794-17
Hypertrophic Cardiomyopathy 150 mm Hg 150 mm Hg 0 mm Hg Baseline 0 mm Hg After ablation CP980117-28 CP1288794-18
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 CP1288794-19
Septal Ablation Success rate Structural Anatomy Anatomy Coronaries CP1288794-20
No Systolic Anterior Motion of Mitral Valve Ablation ineffective Fixed subaortic stenosis 8-10% of referrals CP1288794-21
MR jet directed anteriorly Ablation ineffective Flail leaflet 7-8% CP1288794-22
Septal Ablation Success Structural Anatomy Anatomy Coronaries CP1288794-23
Septal perfusion: just right CP1288794-24
Septal perfusion: too much CP1288794-25
Septal perfusion:?????? CP1288794-26
Septal Ablation There are a subgroup of patients in whom the targeted septum cannot be reached by septal perforators CP1288794-27
Septal myectomy Pre Post CP1288794-28
Septal ablation Pre Post CP1288794-29
Septal Ablation Complicatons Short Term Follow-up CP1288794-30
Complete Heart Block (10-18%) CP1288794-31
Ventricular Fibrillation Incidence unknown Sudden unexpected CP1288794-32
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 CP1288794-33
This needs to be done in an experienced center!!!! 25 y/o Ablation done Now on transplant list CP1288794-34
Septal Ablation Clinical Outcome CP1288794-35
Survival without Severe Sx Pts aged <65 yrs 100 80 Myectomy 90% 60 40 Ablation p=0.02 71% 20 0 No. at risk Myectomy 72 69 61 50 Ablation 52 37 24 18 0 1 2 3 4 Sorajja et al, Circ 2008 Follow-up (yrs) CP1286515-6 CP1288794-36
Septal Ablation 4 yr survival free of death, NYHA Class III/IV or myectomy : 76% 1 of 4 will not have benefit CP1288794-37
Ablation Myectomy Elderly Co-morbidities Limited life-span Sedentary Younger Healthy Long life-span Active CP1288794-38
Patients/year 250 Septal Reduction Therapy Mayo Clinic 200 150 100 Myectomy Ablation 50 0 1973 1978 1983 1988 1993 1998 2003 2008 2013 Year CP1288794-39
Treatment of HCM Relieve Symptoms Prevent Sudden Death CP1288794-40
Can we identify those patients at risk for sudden death? CP1288794-41
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 CP1288794-42
AICD: Caveats 72 y/o with four episodes syncope over 5 years 18 y/o with one episode syncope 1 week ago CP1288794-43
Let s finish with a case LV RV 54 y/o woman Atypical chest pain Normal exam CP1288794-44
What would you do? 1. Right and left heart cath / bx 2. Observation only 3. AICD 4. Anticoagulation 5. Surgery CP1288794-45
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 CP1288794-46
Noncompaction Wide spectrum of prognosis Benign Malignant Systolic function Symptoms Family history CP1288794-47
Noncompaction Wide spectrum of prognosis Benign Malignant Observe Reassure AICD Anticoagulation CP1288794-48
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 CP1288794-49