Cardiac Transplant Symposium - Hartford Hospital Library/Services... · •D. Cardiac Transplant...

Challenges in the Management of Cardiac Transplant Candidates With Preserved Systolic Function: Focus

on Restrictive Cardiomyopathy Andrew D. Feingold, MD, FACC Hartford Hospital Cardiac Transplant Program

Disclosures

• Nothing to Disclose

Projected Mortality for Advanced Heart Failure Exceeds Other Terminal Diseases

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AIDS Leukemia Lung Cancer Pancreatic Cancer End-stage Heart Failure with Optimal Medical

Management Diagnosis

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rtal

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exp

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ear

Rose EA, et al. Long-term mechanical left ventricular assistance for end-stage heart failure. N Engl J Med. 2001 Nov 15;345(20):1435-43.

0

25

50

75

100

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23

Surv

ival

(%

)

Years

1982-1991 (N=21,478)

1992-2001 (N=40,077)

2002-2008 (N=26,039)

2009-6/2015 (N=26,164)

Adult Heart Transplants Kaplan-Meier Survival by Era

2017 JHLT. 2017 Oct; 36(10): 1037-1079

Median survival (years): 1982-1991=8.6; 1992-2001=10.5; 2002-2008=12.2; 2009-6/2015=NA

All pair-wise comparisons were significant at p < 0.05.

(Transplants: January 1982 – June 2015)

Median survival 10.9 years

Adult and Pediatric Heart Transplants Number of Transplants by Year and Location

0

500

1000

1500

2000

2500

3000

3500

4000

4500

5000

5500

Nu

mb

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f tr

ansp

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ts

Other

North America

Europe

NOTE: This figure includes only the heart transplants that are reported to the ISHLT Transplant Registry. As such, the presented data may not mirror the changes in the number of heart transplants performed worldwide.

2017 JHLT. 2017 Oct; 36(10): 1037-1079

4%

4%

31%

51%

3% 2%

4% 1%

Europe

3% 3%

36%

48% 4%

3% 2% 1%

North America

1%

2%

29%

58%

2% 2%

3%

3%

CHD

HCM

ICM

NICM

RCM

Retransplant

VCM

Other

Other

Adult Heart Transplants Diagnosis by Location

2017

JHLT. 2017 Oct; 36(10): 1037-1079

(Transplants: January 2009 – June 2016)

Pasquale, E, et al, The Journal of Heart and Lung Transplantation 2012 31, 1269-1275DOI: (10.1016/j.healun.2012.09.018)

Mortality Post OHT of RCM vs Non RCM Patients

• Analysis of UNOS Registry patient’s undergoing single organ heart transplant from 1987-2010

• 544 total patients representing 1.4% of all cardiac transplants during the period

• Patient’s less likely to supported by VAD pre transplant (10% vs 16%)

• Overall survival similar in comparison to non RCM patients. 1, 5, 10 year survival 84 vs 85%, 66 vs 70%, 45 vs 50%

Pasquale, E, et al. The Journal of Heart and Lung Transplantation 2012 31, 1269-1275DOI: (10.1016/j.healun.2012.09.018)

Outcomes of Restrictive CMP Post Transplant Compared to Other Etiologies

Pasquale, E, et al, The Journal of Heart and Lung Transplantation 2012 31, 1269-1275DOI: (10.1016/j.healun.2012.09.018)

Outcomes of Subsets of Restrictive CMP Post Transplant

• RCM further divided into individual diagnosis. • Idiopathic 42% of patients, N = 227 • Amyloid 26% of patients, N = 142 • Sarcoid 15% of patients, N = 81 • XRT/Chemo 6% of patients, N = 35 • Other 11% of patients, N = 59

• Decreased survival post OHT observed in radiation/chemotherapy group with 1, 5, 10 year survival 71%, 47%, and 32%

• Decreased survival for the amyloid group post transplant with 1, 5, 10 year survival 79%, 47%, and 28%.

Goals and Objectives

• Overview of Restrictive Cardiomyopathy • Clinical Presentation • Etiologies • Pathophysiology • Differentiating Constriction from Restriction

• Medical Management Options

• Candidacy for Cardiac Transplant – Review case • Are outcomes acceptable? • Selection of Appropriate Candidates

• Management of eligible patients for cardiac transplant • Challenges with medical therapy • Issues with Candidacy for VAD • Limitations with current allocation scheme

• Post Transplant Management and Consideration

Making the Diagnosis

Clinical Presentation: Restrictive CMP

• Can occur any age – Some variability dependent on nature

• Symptoms of heart failure with systemic and pulmonary congestion

• Symptoms of dyspnea, peripheral edema, fatigue, weakness, and exercise intolerance with failure of rise in CO with increase in heart rate

• More advanced cases have profound volume overload with significant elevations of right sided filling pressures leading to hepatosplenomegaly, ascites, and anasarca

Features of Restrictive Cardiomyopathy • Increased stiffness of ventricles leading to impaired diastolic filling

• Marked biatrial enlargement

• Non dilated left ventricle with relatively preserved systolic function

Mehra, MR, et al.;Journal of Heart and Lung Transplantation, 2016-01-01, Volume 35, Issue 1, Pages 1-23,

Mckenna, WJ, Clinical Manifestations and diagnosis of Amyloid, Up To Date 6/26/17

Is Ejection Fraction a Surrogate for Cardiac Output?

• Ejection Fraction (%)= 100 x (End Diastolic Volume – End Systolic Volume)

End Diastolic Volume

• Cardiac Output (LPM)=

Stroke Volume x Heart Rate

Etiologies

Muchtar, E, et al, Circ Research 2017; 121: 819-37

Amyloid

Geske JB, et al. JACC 2016; 68: 2329-47

Hemodynamics In Restrictive CMP


• Early rapid filling in diastole due to high LA pressure followed by limitations of filling from stiff myocardium

• Square root sign on ventricular pressure curve

• Stiff ventricles have difficulty accepting additional filling from atrial contraction

Concordant LV and RV tracings

Differentiating Constriction

• Constriction is a disease of the pericardium secondary to encasement of the heart by thickened, fibrous, and/or calcified material

• Leads to limitations of total volume of blood that heart can accommodate during diastole over the respiratory cycle

• Leads to equalization of right and left sided filling pressures

• Early rapid filling from high atrial pressure and unimpeded ventricular relaxation followed by sudden rise in pressure due to pericardial restraint

• Ventricular interdependence secondary to respirophasic filling differences leads to discordant LV and RV pressure tracings


Pericardial Constriction: Imaging


Echo – Constrictive Cardiomyopathy

Septal Bounce - Ventricular Interdependence


Hemodynamics in Constriction

Case 1 Presentation

• 29 year old female with a prior history of Ewing’s (chest wall) sarcoma as a young child for which she underwent resection requiring portion of her left lung removed as well. She was subsequently treated with radiation therapy. She underwent MV Repair in 2004 for severe MR secondary to presumed radiation induced endocardial fibrosis. She subsequently developed progressive exercise intolerance, hypotension, and cachexia presents for evaluation for advanced heart failure therapies.

• Medications: Valsartan/HCTZ 80/12.5 mg daily, Metoprolol xl 12.5 mg daily, Spironolactone 12.5 mg daily, Furosemide 40 mg daily, Metolozone 5 mg daily

• Exam: 85 lbs, BP 82/53, HR 97 bpm, 99%RA. Neck with JVP elevated, S1, S2, regular rhythm, Systolic murmur, LV heave, Decrease BS at left lung base, abdomen non distended, extremities without edema. Cool.

• Labs: Cr 0.8, BNP 2185

• PFTS: Fev1 0.63, FEV1/FVC 79.87, TLC 1.67 (40% of predicted) – Obstruction with severe restriction

• CXR: Status post sternotomy and thoracotomy with virtually

• complete opacification of the left hemithorax and shift of

• mediastinum to the left.

Echo

Echo LV mild global HK. LVEF 40-45%. MV repair with Moderate MR

Catheterization Data

• Left and Right heart catheterization: • RA 18 mmHg,

• PA 55/25 (37) mmHg,

• PCWP 26 mmHg,

• CO 2.33, CI 1.81.

• Large V wave in LA pressure tracing

• LV and RV pressure tracings concordant with respiration

• Conclusions: Findings consistent with a Restrictive Cardiomyopathy

Case Discussion

• Based on patient’s clinical presentation and review of her cath data what is the best strategy for treatment

• A. Continued medical therapy

• B. Initiation of inotropes

• C. Evaluation for LVAD

• D. Cardiac Transplant Evaluation

• E. Palliative care evaluation

Radiation Related Heart Disease • CAD: (6-18% pts develop) Increased capillary

wall permeability, generation of reactive O2 species, and inflammatory cascade. Plaque generation with higher fibrous and less prominent lipid core. Ostial lesions LM and RCA, smoother, and longer. More common second decade – mean 16 years

• Valvular disease: More common second decade, fibrotic thickening, valve retraction, and late calcification. Commonly involve Aortic and Mitral valve curtain

• Pericardial Disease – Common early (60-70% patients). 10-20% of patients go on to develop chronic constrictive pericarditis 5-10 years after radiation treatment

• Myocardial: (50-60%) Diffuse interstitial fibrosis lead to both systolic and diastolic dysfunction. Concomitant stress induced ischemia associated with worse prognosis Jaworski, C, et al; JACC 2013; 61: 2319-28

Veeragandham, R, et al., Ann Thorac Surgery 1998, 65: 1014-9

Radiation and CT Surgery

Wu, Willis, Et al, Circulation 2013; 127: 1476-84

Increased morbidity and mortality Post CT surgery

Medical Management

Question

• Established Treatment strategies to improve mortality in patient’s with a Restrictive Cardiomyopathy are:

• A. Beta-Blockers, Ace-inhibitors/ARB, Aldosterone antagonists

• B. ARB/Neprilysin Inhibitors (Sacubritil/Valsartan)

• C. BIV Pacing

• D. All of the above

• E. None of the above

Jessup M, Brozena S. N Engl J Med 2003;348:2007-2018.

Primary Targets of Treatment in Heart Failure.

Medical Therapy for Stage C HFrEF: Magnitude of Benefit Demonstrated in RCTs

GDMT RR Reduction in

Mortality

NNT for Mortality

Reduction

(Standardized to 36

mo)

RR Reduction

in HF

Hospitalizations

ACE inhibitor or ARB 17% 26 31%

Beta blocker 34% 9 41%

Aldosterone

antagonist 30% 6 35%

Hydralazine/nitrate 43% 7 33%

2013 ACCF/AHA Guideline for the Management of Heart

Failure

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

2013 ACCF/AHA Guideline for the Management of Heart

Failure

Jessup M, Brozena S. N Engl J Med 2003;348:2007-18.

Staging alludes to treatment

Restrictive Cardiomyopathy Medical Treatment Options

• No medical or device therapy known to improve mortality

• ARB and Aldactone when renal function ok • Beta-Blockers/Calcium Channel Blockers

limited efficacy due to risk of hypotension and relial of HR to maintain CO in advanced stages

• Anticoagulation for AFIB • Limitations in medical therapy for

symptoms • Digoxin – Risk for toxicity especially

with amyloid • Vasodilators – Often lead to

hypotension • Diuretics – Increase risk for

progressive renal dysfunction and risk for organ hypoperfusion with over diuresis

Candidacy for Cardiac Transplant

• Outcomes

• Eligibility

• Evaluation

• Management on Wait List

• Post Transplant Considerations

Post Transplant Outcomes Radiation-RCM

Al-Kindi, S, et al, J Cardiac Failure 2016; 22: 475-8

• Analysis of UNOS Registry 2000-2015 looking at Radiation related RT-RCM vs RCM vs Other

• Total of 87 patients with diagnosis of RT 0.2% of transplant during that period

• RT patients younger, more likely female, and more likely to have prior CT surgery (55% total)

• Post Transplant survival lower for RT-RCM vs RCM vs others at 1, 3, and 5 years at 76%, 66%, and 58% compared to RCM 88%, 79%, 73 %, and others 88%, 82%, and 76%

Post Transplant Outcomes Raditation-RCM – 6 Month Mortality

Al-Kindi, S, et al, J Cardiac Failure 2016; 22: 475-8

Radiation RCM Outcomes – Mayo Experience

Saxena P, et al; Ann Thorac Surg 2014; 98: 2115-21

• Retrospective analysis of patients undergoing OHT at Mayo from 1992-2010

• 12 patients radiation related diagnosis. • 9 had prior CT surgery • In hospital mortality 8.2% • 1, 5, 10 year survival 91.75, 75%, 46.7% • 3 patients with secondary malignancy post

transplant with one B cell lymphoma in one patient at year 15

Identifying Appropriate Candidates For Advanced Heart Failure Therapies

Indications for Cardiac Transplant

• NYHA class III or IV Heart Failure refractory to maximal med rx and/or conventional surgery or device therapy

• Inoperable CAD with intractable anginal symptoms

• Malignant ventricular arrhythmias unresponsive to medical or surgical therapy

Copyright ©2003 American Heart Association

Stevenson, L. W. et al. Circulation 2003;108:3059-3063

Definition of heart failure populations with decreasing estimated mortality

Contraindications to Heart Transplantation

Age > 70 years - Relative

Active Infection

Active ulcer disease

Malignancy

Severe diabetes mellitus with end-organ damage

Severe peripheral vascular disease

Pulmonary function (FEV1,FVC) < 60%; history of chronic bronchitis

Serum creatinine level > 2 mg/dL; creatinine clearance < 30 mL/min

Serum bilirubin level > 2.5 mg/dL; transaminase > 2X normal

Pulmonary artery systolic pressure > 60 mmHg

Mean transpulmonary gradient > 15 mmHg

Pulmonary vascular resistance > 3-5 Woods units

May differ from program to program

Adapted from Stevenson. Postgrad Med. 1993

Evaluation for Heart Transplant

• Thorough investigation to determine the etiology of restrictive cardiomyopathy. • Identify subsets with potential reversible cause and or need disease specific

therapies

• Differentiation from constriction

• Identify subset of amyloid

• Evaluation for prognostic markers.

• Exclude significant liver dysfunction from right sided failure

• Evaluation for significant extra organ involvement in the heart


Prognostic Markers


Getting to Transplant

Adult Heart Transplants % of Patients Bridged with Mechanical Circulatory Support* by Year and Device Type

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ECMO VAD+ECMO

TAH LVAD+RVAD

RVAD LVAD

* LVAD, RVAD, TAH, ECMO 2017 JHLT. 2017 Oct; 36(10): 1037-1079

Is Mechanical Support an Option Salvage/Emergency

Therapy Maintenance

Therapy

LVAD Implantation for Advanced Cardiac Amyloid – Mayo Experience

Swiecicki, P, et al; J Heart Lung Transplant 2013; 32: 563-568

• Retrospective study of patients undergoing LVAD for advanced cardiac amyloid with class IV NYHA HF symptoms at Mayo from 12/1/08-5/31/2012

• 9 patients underwent LVAD. 6 SSA, 2 TTR familial, 1 AL. 5 BTT and 4 DT

• 8/9 had non dilated LV • 2 patients died prior to

discharge. 3 patients died post discharge suriving median 13.7 months

Restrictive CMP LVAD Survival by LV Size

Grupper A, et al. J Heart Lung Transplant 2015; 1042-49

• Mayo Study • 28 patients RCM (amyloid,

Radiation, sarcoid, etc..) • One year survival post LVAD 66% • 10 patients went on to transplant • Increased risk when LVEDd > 4.6

cm

LVAD Issues with Restrictive CMP

• Increased risk for suck down events in smaller LV cavity with could result in ventricular arrhythmias and inflow canula obstruction

• Offloaded smaller LV can lead it septum shift to the left which could compromise RV output

• Smaller LV poses a technical challenge to appropriately position to LV inflow canula

Grupper A, et al. J Heart Lung Transplant 2015; 1042-49

UNOS Status: Current

• Status IA • VAD implantation 30 days • Total Artificial Heart • IABP • VAD + complication (i.e. infection, malfunction) • Mech Vent • High dose ionotrope + Swan

• Status IB • Continuous infusion of ionotrope • VAD > 30 days

• Status II – Patient who does not meet the criteria for status IA or IB

• Status VII – Temporarily Inactive

UNOS Proposed Allocation Scheme

OPTN/UNOS Thoracic Organ Transplantation Committe Public Comment Proposal

The Journal of Heart and Lung Transplantation 2017 36, DOI: (10.1016/j.healun.2017.01.333)

Copyright © 2017 Terms and Conditions

RCM/HCM Waitlist Mortality

Sridharan, R, Et al, J Heart and Lung Transplant 36; issue 4 (April 2017)

• RCM/HCM no significant difference in wait list mortality

• Mean wait list time 42 days left • Concerns that new allocation system which

is weighted towards MCS and complications with disadvantage Restrictive patients

http://www.elsevier.com/termsandconditions



Case Follow-up

• Patient underwent evaluation and listing for OHT as a high risk candidate

• Not a candidate for VAD based on her body size and restrictive CMP. Required inotropes and continuous monitoring with swan for stabilization prior to OHT

• After waiting, pediatric donor became available which was an appropriate match to her body size

• Post OHT had prolonged vent wean and inotrope wean. Able to be d/c to rehab after about a month

• Nearly 9 years later doing well

Restrictive CMP Guidelines for Transplant

• 2.1. Restrictive cardiomyopathies

• Recommendations: 2.1.1: RCM patients with severe heart failure symptoms (New York Heart Association Functional Classification III–IV) should be referred for HT evaluation (Class I, Level of Evidence: B).

• 2.1.2: RCM patients evaluated for HT should undergo a complete diagnostic workup to elucidate etiology (infiltrative forms vs idiopathic) and to exclude constrictive pericarditis (Class I, Level of Evidence: C).

• 2.1.3: The decision to list a RCM patient on the cardiac transplant waiting list should take into consideration specific prognostic indicators (the presence and degree of LV systolic dysfunction, atrial enlargement, pulmonary hypertension, and a low cardiac output) (Class I, Level of Evidence: B).

• 2.1.4: In RCM, efficacy and safety of LVAD as a bridge to transplant cannot be recommended as standard procedure. MCS with an LVAD or a total artificial heart may be considered in highly selected cases and at experienced centers (Class IIb, Level of Evidence: C).


Case 2: 70 YO Male with Anasarca and Recurrent Effusions

Amyloid

• Extracellular deposition of misfolded amyloid protein throughout the heart leads to cardiomyocyte separation, cellular toxicity, apoptosis, and tissue stiffness

• Infiltration can occur in the ventricles, atria, perivascular, and valves

• Subsets • AL Amyloid – Immunoglobin derived from plasma cell

• Transthyretin (TTR) amyloid – Transporter synthesized in liver • Familial – Mutated TTR gene

• Senile – Non genetic – misaggregation of wild type TTR

• Exam: Low Pulse volume, Low BP, Elevated JVP, Pleural Effusion, Enlarged Liver, Ascites, Peripheral Edema

• ECG: Low Voltage. Risk for arrhythmias (heart block, afib, VT)

• Echo: Concentric increase LV Wall thickness, Increase thickening of RV free wall, and atrial septum. Granular speckled pattern. Restrictive Filling pattern in diastolic assessment

• Diagnosis: Serum and urine Light Chains. Biopsy apple green bifringence on congo red stain

• Suspect if other organs involved • Peripheral neuropathy – Carpal Tunnel (ATTR-m)

• Swallowing Disorder – Delayed gastric emptying

• Nephrotic Syndrome

• Macroglossia (AL)

• Hepatomegaly


Amyloid Subtypes

Bhogal, Sukhdeep, et al, Current Problems in Cardiology 2017- article in press

Amyloid Subtypes and Clinical Course

Rapezzi, C et al; Circulation 120: 1203-1212

• Overall survival at 2 years for AL 63%, ATTRm 98%, ATTRwt 100%

• AL Amyloid median survival without treatment 6 months

• TTR amyloid median survival 24-66 months.


Amyloid Diagnostic Algorithm

Bhogal, Sukhdeep, et al, Current Problems in Cardiology 2017- article in press

Amyloid Treatment Options


Stanford Experience OHT – Amyloid

Davis MK, et al. Am J of Transplant 2015; 15: 650-8

• 276 patient’s evaluated from 2008-2013, 174 with cardiac involvement

• 19 patient’s underwent heart transplant ( 9 AL, 10 ATTR). Patient’s with significant extracardiac disease not considered for transplant

• No deaths on the waiting list, 35% listed as an exception

• Median age at time of transplant 59 • 8/9 patient with AL treated with chemo prior to OHT.

5 underwent autologous stem cell transplant at median of 245 days

• None of the ATTR patient’s underwent concomitant liver transplant. Reserved for subtypes prone to neuropathy

• 100% survival AL, 2 death’s ATTR (infectious)

Davis, MK, et al, The Journal of Heart and Lung Transplantation 2015 34, 658-666DOI: (10.1016/j.healun.2014.09.006)

UNOS Outcomes of Amyloid Patients with OHT by Era

• Retrospective analysis of UNOS database from 1987 to 2013 divided into two eras (1987-2007 vs 2008-2013)

• Total of 188 underwent OHT and 33 patients Heart/Liver transplant

• Preop VAD 3.6% era 1 and 10% era 2 • Pre-op Inotropes 46% era 1 and 52% era 2 • Unadjusted mortality higher ACM (HR

1.53) compared to other etiologies • Era 2 compared to Era 1 had lower

mortality with HR 0.54

Cardiac Transplant using Extended Donor Criteria for Amyloid

Maurer, M, et al; Transplantation 2007; 83: 539-45

• Columbia Study • Retrospective study of patient’s referred for

OHT evaluation at Columbia • 25 total patient’s referred, 20 listed for

transplant. • Primary outcome survival from time of

evaluation in patient’s transplanted vs not transplanted

• Used extended donor criteria organs (older age, non obstructive CAD or no cath, LVH, prolonged ischemic time, hep c

• Survival one year patient’s undergoing transplant 75% vs 23% for those not undergoing transplant

Cardiac Transplant using Extended Donor Criteria for Amyloid

Maurer, M, et al; Transplantation 2007; 83: 539-45

• 12/25 patient’s underwent OHT • 10/12 AL Amyloid, 8 of which underwent

autologous stem cell transplant at median time of 183 days

• 2/12 ATTTR Amyloid. Both went at liver transplant in second year post transplant

• 3 deaths post transplant( 2 sepsis and 1 lymphoma from donor)

• Survival in short term similar compared to extended donor criteria patients not amyloid and all other post OHT

Amyloid Prognosis


• BNP – Marker of myocardial stretch and stress

• Troponin marker of myocyte damage

MGH Waiting List and Post Transplant Amyloid Survival

Waiting List

Post Transplant

Gilstrap, LG, et al; J Heart Lung Transplant 2014; 33: 149-56

Post Transplant Considerations

Dubrey, S, et, al. The Journal of Heart and Lung Transplantation 2004 23, 1142-1153

Cardiac transplantation for amyloid heart disease: The United Kingdom experience

Non amyloid

Amyloid - chemo

Amyloid – no chemo

• UK – 24 total patients underwent OHT 1984-2002

• 17 patient AL – 7/17 underwent chemo post OHT

• Survival 1,2, and 5 year for amyloid receiving chemo vs no chemo was 50%, 50%, and 20% vs 71%, 71%, and 36%

• 5 year survival for all amyloid patients 38% vs 67% non amyloid patients

Transplant Strategies for Amyloid

• AL Amyloid • Systemic Chemotherapy – Start Before transplant if possible to decrease light

chain production

• Autologous stem cell transplant at around 6 months post transplant

• TTR Amyloid • Liver transplant to eliminate abnormal TTR Production

• Concomitant heart transplant in patients with end stage heart failure


Amyloid Guidelines For Transplant

• 2.2. Cardiac amyloidosis

• Recommendations: 2.2.1 Selected patients with HF due to AL amyloidosis who are not candidates for disease-specific therapies due to cardiovascular compromise may be considered for HT in experienced centers with established collaborations between cardiovascular and hematology teams. Autologous stem cells transplantation (ASCT) should be planned as soon as clinically feasible after recovery from HT (Class IIA, Level of Evidence: B).

• 2.2.2 Patients with transthyretin related (TTR) amyloidosis involving the heart may be considered for HT. Familial TTR cardiac amyloidosis patients should be considered for combined heart and liver transplantation in experienced centers with established collaboration between cardiology, hepatology, and neurology teams (Class IIA, Level of Evidence: B).

• 2.2.3 Amyloid involvement of extracardiac organs must be carefully evaluated when considering AL amyloid patients for sequential HT/ASCT (AL patients) or TTR amyloid patients for HT or combined HT with liver transplantation. Severe extracardiac amyloid organ dysfunction should be considered a contraindication to proceeding with HT (Class IIA, Level of Evidence: B).


Take Home Points

• Restrictive Cardiomyopathy poses challenges to providers in making the diagnosis, treatment, and in the management of patients with advanced heart failure who are candidates for cardiac transplant

• Patients with end stage heart failure can be selected for OHT with reasonable post transplant outcomes. Patient’s with subset diagnosis of amyloid and radiation related restrictive cardiomyopathy have higher risk for OHT

• Patients with AL Amyloid related CMP who undergo heart transplantation should additionally undergo chemotherapy and autologous stem cell transplant in the post transplant period.

Questions

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