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4/20/2018 2:17:47 PM Disclosures No conflicts of interest Alternatives and Bridges to No relevant funding disclosures Lung Transplantation in RV Failure: ECMO/Surgical/Palliation Confession: I am obligated to use and proud of

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Surgical Services

Alternatives and Bridges to Lung Transplantation in RV Failure: ECMO/Surgical/Palliation

George B. Mallory Jr. MD Texas Children’s Hospital Houston, TX

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Disclosures

  • No conflicts of interest
  • No relevant funding disclosures
  • Confession: I am obligated to use and proud of

prescribing medications off FDA labelling indications

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Background

  • Pulmonary vascular disease now a relatively less common

indication for lung TXP around the world since the advent of new pharmacologic therapies (?cause and effect)

  • Idiopathic PH and Eisenmenger-associated PH nevertheless

remain more slowly but still relentless disease processes.

  • Death from end-stage PH too common in all ages.
  • Decisions regarding and timing of referral for lung TXP remain

problematic and difficult.

  • “Suboptimal” survival outcomes of TXP (or perception thereof)

may influence clinical referral decisions

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Reasons Not to Pursue Lung Transplantation: The PH Clinician’s Viewpoint

  • Aggressively optimistic faith in PH pharmacotherapy
  • Strong therapeutic bond with patient +/- family
  • Physical distance to and cost of travel to lung TXP center
  • Uncertainties of wait time, survival to TXP and survival after
  • Frank skepticism about the benefits of lung TXP
  • Options beyond TXP: increasing prostanoid dose (?upper limit in

ng/kg/minute); Potts shunt

  • Lack of understanding of the evaluation and listing process
  • Risk of a stressed dilated RV/compressed LV aer lung TXP →

highest risk for primary graft dysfunction of all TXP recipients

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Reasons Not to Pursue Transplantation: Patient/Family General Viewpoint

  • Hope springs eternal (easily merges with denial)
  • Waiting for breakthroughs
  • Fear of unknown
  • “Bad outcomes”: bad news stories travel further and

faster than good news stories

  • Uncertain form of suffering worse than death from

familiar disease, which is often quick

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Reasons Not to Pursue Transplantation: Practical Concerns, Real and Imagined

  • Insurance difficulties
  • Would we be accepted and then die after long wait?
  • Geographic reality: few and far between pediatric lung

transplant centers

  • Resources – insurance rarely pays for living and travel

expenses

  • Serious disruption of family life – separation often for a

year or more

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Reasons Not to Pursue Transplantation: Specific Concerns and Fears

  • Recovery after lung TXP “too slow”
  • High complication risk with lung TXP
  • Side effects of lifelong immunosuppressants
  • Rejection hard to stop, then relentless
  • Pediatric lung TXP patients are “Guinea Pigs” = distrust
  • f TXP community
  • Downhill decline especially painful after TXP draining

the zeal for life

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Reasons Not to Pursue Transplantation: Patient/Family Viewpoint Specifics

  • “Quality of life after TXP difficult” with rejection meds,

diabetes mellitus, cancer

  • Meds for life with side effects, cancer risk and suffering
  • Best outcomes include expiration date: “Life

expectancy is 1.5 years”

  • Do lung TXP programs oppose Potts? Why?
  • Not a cure, exchanging one disease for another
  • Even if successful, another TXP may be needed?
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Addressing end-stage RV Failure: The Options

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RV Failure: Optimizing Pharmacotherapy

  • Should all TXP candidates be on triple

pharmacotherapy?

  • How about high dose supratherapeutic therapies in lieu
  • f foreboding prospects of lung TXP?
  • How long on uptitrated therapy to pronounce failure?
  • What is the optimal or maximal dose and agent of

prostacyclin therapy?

  • Trial of frontier therapies, e.g., inimitab, Potts shunt,

new agents in development, stem cell therapy

  • Waiting in expectation for breakthrough

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Can RV Failure Be Reversed?

  • Answer: YES (almost always in pediatrics)!
  • Conundrum of young patient who, at PH

diagnosis, has severe RV failure – what is mortality risk?

  • Risk of diagnostic RHC
  • Appropriateness of early shunt via catheterization
  • Rapid titration of combination therapies
  • How quickly can TXP referral get effectuated?
  • How long and how much “investment” will it take?

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This RV Recovered after Transplant

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Interventional Shunts

  • ASD: balloon versus blade +/- stent

versus surgical

  • VSD creation via stent (Justino,

TCH)

  • Reopening PDA (young children) =

“virtual Potts”

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Interventional Shunts

  • Potts Shunt (LPA to descending aorta) –surgical or

catheter-based

  • Experience limited, few centers including Paris and St. Louis
  • Not low risk procedure, operatively and post-operatively
  • Long term outcomes unclear
  • Careful prospective clinical trial in selected centers
  • Criteria for success? Survival, wean from meds, function, QOL
  • Apparently embraced by more families in the current era

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  • St. Louis Children’s Potts Shunt Experience
  • N= 16
  • No operative deaths but delayed deaths = 4 + 1 TXP
  • Clarification of contraindications with experience, e.g.,

ECMO and profound RV failure

  • Ideal candidate: suprasystemic PAP with compensated

RV function

  • Not able to wean systemic prostanoids in all patients in

contrast to French group but weaning largely deferred to PH providers in other locations

Personal communication from RM Grady

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Russian Potts Experience – Sergey Zaets

  • N = 12; mortality = 2
  • Separation of groups
  • PAP >120% of MAP, then a carefully calibrated graft is inserted and

designed to be restrictive

  • If PAP/MAP 1.2- 1.5, then graft to descending aorta cross-sectional

area should be 0.4 to 0.5

  • If PAP/MAP > 1.5, then the graft to descending aorta is sized to be

0.4 in CSA

  • No mention of RV dysfunction
  • All patients who survived the surgery have continued

to survive and all weaned to oral medication and WHO Class I-II.

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Venovenous ECMO as Bridge to LT

  • Lower risk ECMO intervention (less bleeding, lower

anticoagulant use, lower stroke risk) with possibility of rehabilitation during ECMO use via extubation and minimal sedation

  • Extubation and rehab less achievable in infants and toddlers
  • Limited application in lung transplant centers
  • Most centers consider contraindicated in RV failure
  • Due to lack of support for the failing RV, adaptation

with tip of cannula aimed into a large ASD/PFO has been utilized selectively. Positioning is critical.

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Venoarterial ECMO as Bridge to LT

  • An efficacious but high risk mode of acute intervention

in right heart failure

  • Upper body configuration with extubation ideal in adults
  • Limited experience but outcomes good when quick

access to lungs can be assured as in some national organ distribution algorithms (Germany and Canada)

  • In the USA, Lung Allocation Score does NOT give

priority to ECMO patients and patients less than 12 years

  • f age are put into status 1 and 2 → uncertainty of wait

= weeks to months

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Venovenous Arterial ECMO as Bridge to LT

  • The addition of a VV ECMO circuit (third cannula) to

increase blood oxygen content in the RV output to augment delivery of oxygenated blood to coronary arteries and brain

  • The number of size of cannulae may make this mode of

ECMO less suitable to smaller, younger patients and problematic for rehabilitation

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ECMO Clinical Experience: NYC

  • Adult lung transplant candidates 2007 -2016 on ECMO

at Columbia University Medical Center, NYC.

  • “Awake ECMO”
  • N = 72, 40 transplanted. Average wait for TXP = 14 days
  • Average LAS = 91 (0-100)
  • ECMO to death average = 12 days
  • VV = 45; VA = 23; VVA = 3; Nova Lung = 1
  • Tracheostomy = 34
  • Ambulation = 69%
  • PH = 9
  • Preferred VA configuration is IJ and subclavian artery

Biscotti et al. Ann Thorac Surg, 2017

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ECMO Options in 2018

  • The menu of options for end stage RV failure is likely to

be institution specific in 2018 based on ECMO team policies and priorities, availability and interest of an associated lung TXP team, and the preference of surgeons

  • Bridge to effective treatment?
  • An era where sharing of clinical data among

stakeholders has never been more important.

  • We must be honest and transparent with ourselves,
  • ur patients and their families

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Palliative Care

  • Newly emerged, respected subspecialty with new

definition

  • Focus on quality of life
  • Humility regarding prognosis
  • Language of goal setting in partnership with patient

and family

  • Respect for spiritual and humanistic values
  • Awareness of the “costs” of the therapies we offer
  • ?Indicated for all PH patients with RV dysfunction

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UNOS.org Update April 2018

  • Total pediatric lung candidates = 39 as opposed to >350

heart, > 500 liver and > 1000 kidney candidates

  • 0-1 yr = 3
  • 1-5 yr = 6
  • 6-10 yr = 12
  • 11-17 yr = 18
  • LAS pertains to 11 yrs and older with pediatric

preferences

  • National sharing of lung offers for < 11 yrs but only

two scores = status 1 and status 2 and wait list time

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Conclusions

  • Clinical context of progressive RV failure a high stakes

scenario for all involved.

  • No single option ideal for all patients and families.
  • Lung TXP a very big deal, offered in few centers and

requires extraordinary commitment and an embrace of

  • uncertainty. Avoid negativity of outcomes!
  • Clear communication and clarification for patient and

family available options an ethical imperative.

  • Improvements in therapies (PH and TXP) and our

understanding of specific indications likely in the future