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 prescribing medications off FDA labelling indications George B. Mallory Jr. MD Texas Children’s Hospital Houston, TX Surgical Services Page 1 Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM Reasons Not to Pursue Lung Transplantation: Background The PH Clinician’s Viewpoint •Pulmonary vascular disease now a relatively less common •Aggressively optimistic faith in PH pharmacotherapy indication for lung TXP around the world since the advent of new •Strong therapeutic bond with patient +/- family pharmacologic therapies (?cause and effect) •Idiopathic PH and Eisenmenger-associated PH nevertheless •Physical distance to and cost of travel to lung TXP center remain more slowly but still relentless disease processes. •Uncertainties of wait time, survival to TXP and survival after •Death from end-stage PH too common in all ages. •Frank skepticism about the benefits of lung TXP •Decisions regarding and timing of referral for lung TXP remain •Options beyond TXP: increasing prostanoid dose (?upper limit in problematic and difficult. ng/kg/minute); Potts shunt •“Suboptimal” survival outcomes of TXP (or perception thereof) may influence clinical referral decisions •Lack of understanding of the evaluation and listing process •Risk of a stressed dilated RV/compressed LV a�er lung TXP → highest risk for primary graft dysfunction of all TXP recipients Page 2 Page 3 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:56 PM 1
4/20/2018 2:17:47 PM Reasons Not to Pursue Transplantation: Reasons Not to Pursue Transplantation: Patient/Family General Viewpoint Practical Concerns, Real and Imagined •Insurance difficulties •Hope springs eternal (easily merges with denial) •Would we be accepted and then die after long wait? •Waiting for breakthroughs •Geographic reality: few and far between pediatric lung •Fear of unknown transplant centers •Resources – insurance rarely pays for living and travel •“Bad outcomes”: bad news stories travel further and expenses faster than good news stories •Serious disruption of family life – separation often for a •Uncertain form of suffering worse than death from year or more familiar disease, which is often quick Page 4 Page 5 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:56 PM Reasons Not to Pursue Transplantation: Reasons Not to Pursue Transplantation: Specific Concerns and Fears Patient/Family Viewpoint Specifics •“Quality of life after TXP difficult” with rejection meds, •Recovery after lung TXP “too slow” diabetes mellitus, cancer •High complication risk with lung TXP •Meds for life with side effects, cancer risk and suffering •Side effects of lifelong immunosuppressants •Best outcomes include expiration date: “Life •Rejection hard to stop, then relentless expectancy is 1.5 years” •Pediatric lung TXP patients are “Guinea Pigs” = distrust •Do lung TXP programs oppose Potts? Why? of TXP community •Not a cure, exchanging one disease for another •Even if successful, another TXP may be needed? •Downhill decline especially painful after TXP draining the zeal for life Page 6 Page 7 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:56 PM 2
4/20/2018 2:17:47 PM RV Failure: Optimizing Pharmacotherapy •Should all TXP candidates be on triple pharmacotherapy? •How about high dose supratherapeutic therapies in lieu of foreboding prospects of lung TXP? Addressing end-stage RV Failure: •How long on uptitrated therapy to pronounce failure? •What is the optimal or maximal dose and agent of The Options prostacyclin therapy? •Trial of frontier therapies, e.g., inimitab, Potts shunt, new agents in development, stem cell therapy •Waiting in expectation for breakthrough Page 8 Page 9 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:56 PM Can RV Failure Be Reversed? This RV Recovered after Transplant •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? Page 10 Page 11 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:56 PM 3
4/20/2018 2:17:47 PM Interventional Shunts Interventional Shunts •Potts Shunt (LPA to descending aorta) –surgical or catheter-based •ASD: balloon versus blade +/- stent •Experience limited, few centers including Paris and St. Louis versus surgical •Not low risk procedure, operatively and post-operatively •VSD creation via stent (Justino, •Long term outcomes unclear TCH) • Careful prospective clinical trial in selected centers • Criteria for success? Survival, wean from meds, function, QOL •Reopening PDA (young children) = •Apparently embraced by more families in the current era “virtual Potts” Page 12 Page 13 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:56 PM Russian Potts Experience – Sergey Zaets St. Louis Children’s Potts Shunt Experience •N= 16 •N = 12; mortality = 2 •No operative deaths but delayed deaths = 4 + 1 TXP •Separation of groups •Clarification of contraindications with experience, e.g., - PAP >120% of MAP, then a carefully calibrated graft is inserted and designed to be restrictive ECMO and profound RV failure -If PAP/MAP 1.2- 1.5, then graft to descending aorta cross-sectional •Ideal candidate: suprasystemic PAP with compensated area should be 0.4 to 0.5 RV function -If PAP/MAP > 1.5, then the graft to descending aorta is sized to be 0.4 in CSA •Not able to wean systemic prostanoids in all patients in •No mention of RV dysfunction contrast to French group but weaning largely deferred to •All patients who survived the surgery have continued PH providers in other locations to survive and all weaned to oral medication and WHO Personal communication from RM Grady Class I-II. Page 14 Page 15 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:56 PM 4
4/20/2018 2:17:47 PM Venovenous ECMO as Bridge to LT Venoarterial ECMO as Bridge to LT •Lower risk ECMO intervention (less bleeding, lower •An efficacious but high risk mode of acute intervention anticoagulant use, lower stroke risk) with possibility of in right heart failure rehabilitation during ECMO use via extubation and •Upper body configuration with extubation ideal in adults •Limited experience but outcomes good when quick minimal sedation access to lungs can be assured as in some national organ •Extubation and rehab less achievable in infants and toddlers •Limited application in lung transplant centers distribution algorithms (Germany and Canada) •Most centers consider contraindicated in RV failure •In the USA, Lung Allocation Score does NOT give •Due to lack of support for the failing RV, adaptation priority to ECMO patients and patients less than 12 years with tip of cannula aimed into a large ASD/PFO has been of age are put into status 1 and 2 → uncertainty of wait utilized selectively. Positioning is critical. = weeks to months Page 16 Page 17 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:56 PM xxx00.#####.ppt 4/20/2018 2:17:57 PM 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 Rosenzweig E, ASAIO, 2013 Page 18 Page 19 Surgical Services Surgical Services xxx00.#####.ppt 4/20/2018 2:17:57 PM xxx00.#####.ppt 4/20/2018 2:17:57 PM 5
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