CP1288794-1

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

CP1288794-2

Let’s start with a case

52 y/o man – Class III DOE

Loud murmur – 300 mg lopressor

Gradient 100 mmHg

CP1288794-3

Next step?

1. More meds

2. Dual chamber PM

3. Septal ablation

4. Septal myectomy

5. AICD

CP1288794-4

Treatment of HCM

Relieve

Symptoms

Prevent

Sudden Death

Dynamic

LVO Obstruction

CP1288794-5

Gradient

LAP

Relieve obstruction Improve diastolic filling

Reduce MR

Improve symptoms

CP1288794-6

Beta Blockers

Ca Blockers

Disopyramide

Intolerant

Intolerant

Continued

symptoms

Myectomy

DDD pacing

Ablation

Hypertrophic Cardiomyopathy

Medical therapy : symptomatic pt

CP1288794-7 CP980117-19

NSR PACE

Dual Chamber Pacing

CP1288794-8

“New medicines and

other cures always work

miracles for awhile…”

William

Heberden

1877

CP1288794-9

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

CP1288794-10

Septal Reduction Therapy

CP1288794-11

Septal Reduction Therapy

Septal

Myectomy

CP1288794-12

Operative mortality 0.8%

Gradient 3 mm

Post-op NYHA 1-2 94%

Surgical septal myectomy for symptomatic HOCM is safe

and effective

In the hands of experienced surgeons

CP1288794-13

0

20

40

60

80

100

Before Postop

I-II

III-IV%

Pts

Septal Myectomy: Mayo Data

256 pts – F/U 10 yrs

CP1288794-14 CP1160937-13

Myectomy and Survival

Overall survival

Years Ommen et al: JACC, 2005

Same results for cardiac survival and sudden death P<0.001

0.5

0.6

0.7

0.8

0.9

1.0

0 2 4 6 8 10

Nonobstructive

Nonoperated obstructive

Myectomy (Mayo)

CP1288794-15

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

CP1288794-16

Septal

Ablation

The “New Kid

on the Block”

A localized heart attack

CP1288794-17

Septal Ablation

CP1288794-18 CP980117-28

Hypertrophic Cardiomyopathy

Baseline After ablation

0 mm Hg

150 mm Hg

0 mm Hg

150 mm Hg

CP1288794-19

“Surgery now has no role in the

management of HCM…”

“Ablation is the new gold standard for

the 21st 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

CP1288794-20

Septal

Ablation

Structural

Anatomy Anatomy

Coronaries

Success rate

CP1288794-21

Fixed subaortic stenosis

8-10% of referrals

No Systolic Anterior

Motion of Mitral Valve

Ablation

ineffective

CP1288794-22

Flail leaflet

7-8%

MR jet directed

anteriorly

Ablation

ineffective

CP1288794-23

Septal

Ablation

Structural

Anatomy Anatomy

Coronaries

Success

CP1288794-24

Septal perfusion: just right

CP1288794-25

Septal perfusion: too much

CP1288794-26

Septal perfusion: ??????

CP1288794-27

Septal Ablation

There are a subgroup of patients in whom the

“targeted septum” cannot be reached by septal

perforators

CP1288794-28

Septal myectomy

Pre Post

CP1288794-29

Septal ablation

Pre Post

CP1288794-30

Septal

Ablation

Short

Term Follow-up

Complicatons

CP1288794-31

Complete Heart Block

(10-18%)

CP1288794-32

Ventricular Fibrillation

Incidence unknown

Sudden unexpected

CP1288794-33

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

CP1288794-34

This needs to be done in

an experienced center !!!!

25 y/o

Ablation done

Now on

transplant list

CP1288794-35

Septal

Ablation

Clinical

Outcome

CP1288794-36

0

20

40

60

80

100

0 1 2 3 4

CP1286515-6

Survival without Severe Sx

Pts aged <65 yrs

No. at risk

Myectomy 72 69 61 50

Ablation 52 37 24 18

Follow-up (yrs) Sorajja et al, Circ 2008

Myectomy

Ablation

p=0.02

71%

90%

CP1288794-37

Septal Ablation

4 yr survival free of death, NYHA Class III/IV or myectomy : 76%

1 of 4 will not have benefit

CP1288794-38

Ablation Myectomy

Younger

Healthy

Long life-span

Active

Elderly

Co-morbidities

Limited life-span

Sedentary

CP1288794-39

0

50

100

150

200

250

MyectomyAblation

Pa

tie

nts

/ye

ar

1973 1978 1983 1988 1993 1998 2003 2008 2013

Year

Septal Reduction Therapy

Mayo Clinic

CP1288794-40

Treatment of HCM

Relieve

Symptoms

Prevent

Sudden Death

CP1288794-41

Can we identify

those patients at

risk for sudden

death?

CP1288794-42

Risk Factors for Sudden Death

Arrest

Sustained VT

FH HCM and Sudden death

LVH > 30 mm

Unexplained syncope NSVT

BP drop TMET

Gadolinium DE

LVO

CAD

Really Bad

Somewhat Bad

Bad

CP1288794-43

AICD: Caveats

•72 y/o with four episodes syncope over 5 years

•18 y/o with one episode syncope 1 week ago

CP1288794-44

Let’s finish with a case

54 y/o woman

Atypical chest pain

Normal exam LV RV

CP1288794-45

What would you do?

1. Right and left heart cath / bx

2. Observation only

3. AICD

4. Anticoagulation

5. Surgery

CP1288794-46

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

CP1288794-47

Benign Malignant

Noncompaction Wide spectrum of prognosis

Systolic function

Symptoms

Family history

CP1288794-48

Noncompaction Wide spectrum of prognosis

AICD

Anticoagulation

Observe

Reassure

Benign Malignant

CP1288794-49

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