Surgical Treatment of Restrictive Cardiomyopathy

Danny Ramzy, MD PhD FRCSC FACC

Surgical Director Lung Transplant Program

Director Robotic and Minimally Invasive Cardiac Surgery

Cedars-Sinai Medical Center

Division of Cardiothoracic Surgery

Disclosures

• Cardiac Surgeon

– Bias -- Surgery is always better than medicine ALWAYS

– No Honorarium

– Free Dinner

– No free parking

Grievances

• 2013 - Heartmate II vs. Heartware for LVAD

– 1 week notice

• 2015 - Futility of ECMO

– Really REALLY

• 2017 - Asked to participate with lot of advance

notice

– Yes

Invitation

Danny and Dael, Would you both be interested in participating in a debate at the

Cedars’ Controversies Symposium (Thursday, November 16th) on the following:

Debate: Medical vs Surgical treatment of severe diastolic dysfunction

4:30– 4:40 PM Medical: Dael Geft, MD

4:40 – 4:50 PM Surgical: Danny Ramzy, MD

4:50 – 5:00 PM Roundtable Discussion

Response

Would love to participate. Thank you for the

invitation.

Looking forward to it.

What the … Why do you hate me.

2019 -- ??? Offpump heart transplant

Medical vs Surgical Treatment of

Restrictive Cardiomyopathy

Thank God for Diastolic Dysfunction…

Treatment of HFpEF Recommendations COR LOE

Systolic and diastolic blood pressure should be

controlled according to published clinical practice

guidelines I B

Diuretics should be used for relief of symptoms due

to volume overload I C

Coronary revascularization for patients with CAD in

whom angina or demonstrable myocardial

ischemia is present despite GDMT

IIa

C

Management of AF according to published clinical

practice guidelines for HFpEF to improve

symptomatic HF IIa C

Use of beta-blocking agents, ACE inhibitors, and

ARBs for hypertension in HFpEF IIa C

ARBs might be considered to decrease

hospitalizations in HFpEF IIb B

Nutritional supplementation is not recommended in

HFpEF III: No

Benefit C

Restrictive Cardiomyopathy

Restrictive Cardiomyopathy

• Diagnosis/work-up

• Pathophysiology

• Imaging

• Medical management

• Please refer to previous talk

Common comments about medical management

• There is no specific treatment.

• Not much works for HFpEF

• r/o constriction which is treatable (restriction poor

prognosis)

• Most are irreversible and require cardiac

transplantation

• Usually ineffective and generally consists of

supportive measures

Restrictive Cardiomyopathy

• Surgical Management

– LVAD

– TAH

– OHT

LVAD

RCM + LVAD

LVAD in Restrictive cardiomyopathy

• Placing the inflow cannula in a small LV cavity can be challenging but

not impossible

• Myomectomy is performed in addition to the regular coring out of the

LV apex to create an adequate space for the inflow cannulae

• Positioning the inflow cannula alignment with the long axis of the LV

pointing towards the mitral valve

Topilsky et al. Circulation Heart Failure. 2011

Improved Survival in LVAD Trials

Months

0 6 12 18 24

Perc

en

t S

urv

ival

0

10

20

30

40

50

60

70

80

90

100

HM II BTT Miller NEJM 2007

HM II DT Slaughter NEJM 2009

HM II BTT Pagani JACC 2009

HM II BTT Post-Approval study Starling JACC 2011

VE DT LVAD REMATCH Rose NEJM 2001

XVE DT LVAD Slaughter NEJM 2009

OMM REMATCH Rose NEJM 2001

OMM INTrEPID Rogers JACC 2007

Novacor DT LVAD INTrEPID Rogers JACC 2007

HM II DT Park CIRC HF 2012

HM II BTT Post Trial John ATS 2011

However, patients with end stage RCM were not represented in

these studies, and a detailed analysis describing the impact of

continuous axial flow LVAD on their outcome has not been

fully analyzed

• Retrospective review of 83 consecutive patients who received HMII

LVAD for either DCM/ICM or RCM

• February 2007 – May 2010

Topilsky et al. Circulation Heart Failure. 2011

Outcomes RCM vs DCM

Topilsky et al. Circulation Heart Failure. 2011

Outcomes RCM vs DCM

Topilsky et al. Circulation Heart Failure. 2011

LVAD in RCM

• Continuous flow axial LVAD therapy may be feasible in

patients with end stage restrictive or hypertrophic

cardiomyopathy and may prove to become a useful option to

treat these patients who have end stage heart failure.

• However, these patients can have persistent right heart

failure likely due to primary involvement of the right

ventricle from the underlying cardiomyopathic process

Topilsky et al. Circulation Heart Failure. 2011

• Retrospective study of prospectively collected data of 28 consecutive

patients with end-stage RCM who received LVAD

• January 2008 – August 2013

Survival rates for restrictive cardiomyopathy patients post-LVAD:

Transplanted vs non-transplanted patients

Grupper et al JHLT 2015;34:1042–1049

Survival rates for restrictive cardiomyopathy patients post-LVAD:

BTT vs DT patients

Grupper et al JHLT 2015;34:1042–1049

Survival rates for restrictive cardiomyopathy patients post-LVAD:

Amyloid vs non-amyloid

Grupper et al JHLT 2015;34:1042–1049

Study Findings

1) LVAD implantation is technically feasible in patient

with advanced RCM

2) LVAD therapy is associated with improved survival

compared to medical therapy regardless of etiology

3) LVAD therapy for RCM has good long term

survival in both the BTT and DT groups

• The INTERMACS Registry was queried for all patients with RCM

(n = 94) and HCM (n = 104) who underwent CF LVAD implantation

between March 2008 and March 2014.

Patel et al. J Card Fail. 2017

Survival by primary diagnosis group

Patel et al. J Card Fail. 2017

Competing outcomes analysis

No statistical difference was found in survival

among the 3 cohorts

At 1 year, 81.4%, 75.4%, and 81.7% of HCM,

RCM, and DCM patients, respectively, were alive

on support or transplanted.

RCM

HCM

DCM

Patel et al. J Card Fail. 2017

Concerns for AE in RCM with LVAD

• In regard to the concern for impaired LV filling

– Rates of arrhythmias were similar across groups

– Device thrombosis rates were similar

– No differences were seen in device malfunctions and

hemolysis rates

• Outcomes of LVAD in RCM are comparable to those

with a standard indication of DCM

Can we LVAD every RCM patient

Survival by LV size

Size matters

TAH

Implants June 2006 – March 2015: TAH – Survival Outcomes

TAH CFLVAD/BTT Listed Primary Diagnosis n=359 n=3473 Cancer 2 (0.5%) 3 (0.1%) Ischemic Cardiomyopathy/Coronary Artery Disease 108 (30.1%) 1331 (38.3%) Dilated Myopathy: Post partum 3 (0.8%) 58 (1.7%) Dilated Myopathy: Viral 10 (2.8%) 103 (3.0%) Dilated Myopathy: All others 173 (48.2%) 1826 (52.6%) Hypertrophic Myopathy 11 (3.1%) 46 (1.3%) Restrictive Myopathy 22 (6.1%) 32 (0.9%) Valvular Heart Disease 14 (3.9%) 32 (0.9%) Congenital Heart Disease 10 (2.8%) 29 (0.8%) Not specified 6 (1.7%) 13 (0.4%) Totals 359 (100%) 3473 (100%)

Comparison of TAH, n=359 and CFLVAD BTT: Listed, n=3473 Primary Diagnoses

Competing outcomes depiction for total artificial heart patients implanted June 2006 through April 2017 (n=450). At any point in time, the sum of the proportions of each outcome equals 1. TAH, total artificial heart

Intermacs Patient Profile: n (%)

1. Critical Cardiogenic Shock 189 (43.1)

2. Progressive Decline 163 (37.1)

3. Stable but Inotrope Dependent 43 ( 9.8)

4. Resting Symptoms 32 ( 7.3)

5-7. Less Sick 12 ( 2.7)

Heart Transplantation

All-Cause Mortality after OHT in

patients with and without RCM

Davis et al. AJT 2015;15:650-658

Heart Transplantation

• Idiopathic RCM – Treatment:

• Largely supportive

• OHT

• Cardiac Sarcoid – OHT has good outcomes with rare recurrence of sarcoid in the new heart or progression of extracardiac

sarcoid

• Perkel D, Czer LS, Morrissey RP, Patel J, Kobashigawa J, et al. Heart transplantation for end-stage heart failure due to cardiac

sarcoidosis. Transplant Proc 2013;45:2384-6.

• Cardiac Amyloid

Cardiac Sarcoid

The outcomes, including intermediate and long-term

survival, for these patients with CS are better than for

patients undergoing OHT for all other diagnoses. Rarely,

sarcoidosis may recur in the transplanted heart,

particularly in patients on no or low dose steroid

treatment.

Perkel D, Czer LS, Morrissey RP, Patel J, Kobashigawa J, et al. Heart transplantation for

end-stage heart failure due to cardiac sarcoidosis. Transplant Proc 2013;45:2384-6.

Discussion

• RCM is a heterogenous group of diseases with varied prognosis and

treatment (Treat underlying disease in secondary causes RCM)

• Treat heart failure symptoms

– Diuretics and ACE inhibitors

– Avoid digitalis, nifedipine, ACE-I and verapamil in Amyloid

• Most are irreversible and require surgical treatment (LVAD, TAH

and/or cardiac transplantation)

– Surgical therapies offer improved quality of life and survival

– Results are comparable to patients with DCM/ICM

Surgical challenge

MYOMECTOMY

How am I supposed to get…

Into…

Surgical challenge

Final words

Medical Therapy

Final words

Medical therapy for RCM

Surgical therapy for RCM

Thank You