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