Subscribe to RSS
DOI: 10.1055/s-0037-1606223
Lung Transplantation for Pulmonary Hypertension and Strategies to Bridge to Transplant
Publication History
Publication Date:
15 October 2017 (online)
Abstract
Despite an expanded armamentarium of medical therapies, pulmonary arterial hypertension (PAH) remains a progressive disease associated with significant morbidity and premature mortality. Lung transplantation (LT) is the last safety net for patients failing medical therapy, providing excellent overall long-term survival and health-related quality-of-life outcomes in line with more common parenchymal lung disease indications. Waitlist mortality remains disproportionally elevated, however, reflecting an inability of the lung allocation score to completely capture PAH disease severity, and a propensity for PAH to deteriorate rapidly without warning, even in patients who appear externally “well.” Early referral to a LT center can mitigate these risks and facilitate rapid listing if necessary. Several bridging therapies are available to support severely unwell patients to LT, such as extracorporeal life support (ECLS) and atrial septostomy. Unique perioperative considerations include higher rates of primary graft dysfunction and dynamic right ventricular outflow obstruction which may, at least in part, reflect rapid afterload reduction in the face of a conditioned right ventricle. Extending ECLS into the perioperative period may ameliorate these risks by allowing more gradual adaptation of both ventricles to their new loading conditions. Chronic lung allograft dysfunction, particularly bronchiolitis obliterans syndrome, remains a major cause of long-term morbidity and mortality, and complications from corticosteroid and immunosuppressive therapy are common. Nevertheless, the morbidity, mortality, and burden of disease management after LT continue to improve and compare favorably to that of refractory PAH in carefully selected patients.
-
References
- 1 McGoon MD, Benza RL, Escribano-Subias P. , et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013; 62 (25, Suppl): D51-D59
- 2 Humbert M, Sitbon O, Chaouat A. , et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122 (02) 156-163
- 3 Campo A, Mathai SC, Le Pavec J. , et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med 2010; 182 (02) 252-260
- 4 Benza RL, Miller DP, Gomberg-Maitland M. , et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 2010; 122 (02) 164-172
- 5 D'Alonzo GE, Barst RJ, Ayres SM. , et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115 (05) 343-349
- 6 Galiè N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses. Eur Heart J 2010; 31 (17) 2080-2086
- 7 Kemp K, Savale L, O'Callaghan DS. , et al. Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: an observational study. J Heart Lung Transplant 2012; 31 (02) 150-158
- 8 Sitbon O, Jaïs X, Savale L. , et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43 (06) 1691-1697
- 9 Galiè N, Barberà JA, Frost AE. , et al; AMBITION Investigators. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015; 373 (09) 834-844
- 10 Farber HW, Miller DP, Meltzer LA, McGoon MD. Treatment of patients with pulmonary arterial hypertension at the time of death or deterioration to functional class IV: insights from the REVEAL Registry. J Heart Lung Transplant 2013; 32 (11) 1114-1122
- 11 Tonelli AR, Arelli V, Minai OA. , et al. Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med 2013; 188 (03) 365-369
- 12 Galiè N, Humbert M, Vachiery JL. , et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37 (01) 67-119
- 13 Nickel N, Golpon H, Greer M. , et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2012; 39 (03) 589-596
- 14 Miller DP, Farber HW. “Who'll be the next in line?” The lung allocation score in patients with pulmonary arterial hypertension. J Heart Lung Transplant 2013; 32 (12) 1165-1167
- 15 Galiè N, Corris PA, Frost A. , et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 2013; 62 (25, Suppl): D60-D72
- 16 George MP, Champion HC, Pilewski JM. Lung transplantation for pulmonary hypertension. Pulm Circ 2011; 1 (02) 182-191
- 17 Orens JB, Estenne M, Arcasoy S. , et al; Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006; 25 (07) 745-755
- 18 Egan TM, Murray S, Bustami RT. , et al. Development of the new lung allocation system in the United States. Am J Transplant 2006; 6 (5, Pt 2): 1212-1227
- 19 Gottlieb J, Greer M, Sommerwerck U. , et al. Introduction of the lung allocation score in Germany. Am J Transplant 2014; 14 (06) 1318-1327
- 20 Eurotransplant. Introduction of the LAS in the Netherlands. Eurotransplant; 2014
- 21 NHSBT. Donor Lung Distribution and Allocation. 2017 . Policy 230/7, URL: http://odt.nhs.uk/pdf/lung_allocation_policy.pdf
- 22 The Transplantation Society of Australia and New Zealand. Organ Transplantation from Deceased Donors: Consensus Statement on Eligibility Criteria and Allocation Protocols. 2011
- 23 Chen F, Oga T, Yamada T. , et al. Lung allocation score and health-related quality of life in Japanese candidates for lung transplantation. Interact Cardiovasc Thorac Surg 2015; 21 (01) 28-33
- 24 Davis SQ, Garrity Jr ER. Organ allocation in lung transplant. Chest 2007; 132 (05) 1646-1651
- 25 Egan TM, Edwards LB. Effect of the lung allocation score on lung transplantation in the United States. J Heart Lung Transplant 2016; 35 (04) 433-439
- 26 Singer JP, Katz PP, Soong A. , et al. Effect of lung transplantation on health-related quality of life in the era of the lung allocation score: a U.S. prospective cohort study. Am J Transplant 2017; 17 (05) 1334-1345
- 27 Yusen RD, Edwards LB, Dipchand AI. , et al; International Society for Heart and Lung Transplantation. The Registry of the International Society for Heart and Lung Transplantation: Thirty-third Adult Lung and Heart-Lung Transplant Report-2016; Focus Theme: Primary Diagnostic Indications for Transplant. J Heart Lung Transplant 2016; 35 (10) 1170-1184
- 28 Chen H, Shiboski SC, Golden JA. , et al. Impact of the lung allocation score on lung transplantation for pulmonary arterial hypertension. Am J Respir Crit Care Med 2009; 180 (05) 468-474
- 29 Gomberg-Maitland M, Glassner-Kolmin C, Watson S. , et al. Survival in pulmonary arterial hypertension patients awaiting lung transplantation. J Heart Lung Transplant 2013; 32 (12) 1179-1186
- 30 Benza RL, Miller DP, Frost A, Barst RJ, Krichman AM, McGoon MD. Analysis of the lung allocation score estimation of risk of death in patients with pulmonary arterial hypertension using data from the REVEAL Registry. Transplantation 2010; 90 (03) 298-305
- 31 OPAT Network. Policies, Effective Date 06/29/2017. URL: https://optn.transplant.hrsa.gov/media/1200/optn_policies.pdf#nameddest=Policy_10
- 32 Keogh AM, Mayer E, Benza RL. , et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 2009; 54 (1, Suppl): S67-S77
- 33 de Perrot M, Granton JT, McRae K. , et al. Impact of extracorporeal life support on outcome in patients with idiopathic pulmonary arterial hypertension awaiting lung transplantation. J Heart Lung Transplant 2011; 30 (09) 997-1002
- 34 Dellgren G, Riise GC, Swärd K. , et al. Extracorporeal membrane oxygenation as a bridge to lung transplantation: a long-term study. Eur J Cardiothorac Surg 2015; 47 (01) 95-100 , discussion 100
- 35 Javidfar J, Brodie D, Iribarne A. , et al. Extracorporeal membrane oxygenation as a bridge to lung transplantation and recovery. J Thorac Cardiovasc Surg 2012; 144 (03) 716-721
- 36 Del Sorbo L, Boffini M, Rinaldi M, Ranieri VM. Bridging to lung transplantation by extracorporeal support. Minerva Anestesiol 2012; 78 (02) 243-250
- 37 Abrams DC, Brodie D, Rosenzweig EB, Burkart KM, Agerstrand CL, Bacchetta MD. Upper-body extracorporeal membrane oxygenation as a strategy in decompensated pulmonary arterial hypertension. Pulm Circ 2013; 3 (02) 432-435
- 38 Gulack BC, Hirji SA, Hartwig MG. Bridge to lung transplantation and rescue post-transplant: the expanding role of extracorporeal membrane oxygenation. J Thorac Dis 2014; 6 (08) 1070-1079
- 39 Lehmann S, Uhlemann M, Leontyev S. , et al. Fate of patients with extracorporeal lung assist as a bridge to lung transplantation versus patients without--a single-center experience. Perfusion 2015; 30 (02) 154-160
- 40 Roussel A, Al-Attar N, Khaliel F. , et al. Arterial vascular complications in peripheral extracorporeal membrane oxygenation support: a review of techniques and outcomes. Future Cardiol 2013; 9 (04) 489-495
- 41 Aziz F, Brehm CE, El-Banyosy A, Han DC, Atnip RG, Reed AB. Arterial complications in patients undergoing extracorporeal membrane oxygenation via femoral cannulation. Ann Vasc Surg 2014; 28 (01) 178-183
- 42 Javidfar J, Brodie D, Sonett J, Bacchetta M. Venovenous extracorporeal membrane oxygenation using a single cannula in patients with pulmonary hypertension and atrial septal defects. J Thorac Cardiovasc Surg 2012; 143 (04) 982-984
- 43 Strueber M, Hoeper MM, Fischer S. , et al. Bridge to thoracic organ transplantation in patients with pulmonary arterial hypertension using a pumpless lung assist device. Am J Transplant 2009; 9 (04) 853-857
- 44 Patil NP, Mohite PN, Reed A, Popov AF, Simon AR. Modified technique using Novalung as bridge to transplant in pulmonary hypertension. Ann Thorac Surg 2015; 99 (02) 719-721
- 45 Fischer S, Simon AR, Welte T. , et al. Bridge to lung transplantation with the novel pumpless interventional lung assist device NovaLung. J Thorac Cardiovasc Surg 2006; 131 (03) 719-723
- 46 Mayes J, Niranjan G, Dark J, Clark S. Bridging to lung transplantation for severe pulmonary hypertension using dual central Novalung lung assist devices. Interact Cardiovasc Thorac Surg 2016; 22 (05) 677-678
- 47 Fuehner T, Kuehn C, Hadem J. , et al. Extracorporeal membrane oxygenation in awake patients as bridge to lung transplantation. Am J Respir Crit Care Med 2012; 185 (07) 763-768
- 48 Rosenzweig EB, Chicotka S, Bacchetta M. Right ventricular assist device use in ventricular failure due to pulmonary arterial hypertension: lessons learned. J Heart Lung Transplant 2016; 35 (10) 1272-1274
- 49 Bein T, Weber F, Philipp A. , et al. A new pumpless extracorporeal interventional lung assist in critical hypoxemia/hypercapnia. Crit Care Med 2006; 34 (05) 1372-1377
- 50 Iacono A, Groves S, Garcia J, Griffith B. Lung transplantation following 107 days of extracorporeal membrane oxygenation. Eur J Cardiothorac Surg 2010; 37 (04) 969-971
- 51 Hopkins WE. The remarkable right ventricle of patients with Eisenmenger syndrome. Coron Artery Dis 2005; 16 (01) 19-25
- 52 Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996; 15 (1, Pt 1): 100-105
- 53 Sandoval J, Gomez-Arroyo J, Gaspar J, Pulido-Zamudio T. Interventional and surgical therapeutic strategies for pulmonary arterial hypertension: Beyond palliative treatments. J Cardiol 2015; 66 (04) 304-314
- 54 Sandoval JTA. The right ventricle in health and disease: atrial septostomy. In: Voelkel NF, Schranz D. , eds. The Right Ventricle in Health and Disease. New York, NY: Springer; 2015: 419-437
- 55 Zierer A, Melby SJ, Voeller RK, Moon MR. Interatrial shunt for chronic pulmonary hypertension: differential impact of low-flow vs. high-flow shunting. Am J Physiol Heart Circ Physiol 2009; 296 (03) H639-H644
- 56 Austen WG, Morrow AG, Berry WB. Experimental studies of the surgical treatment of primary pulmonary hypertension. J Thorac Cardiovasc Surg 1964; 48: 448-455
- 57 Weimar T, Watanabe Y, Kazui T. , et al. Impact of differential right-to-left shunting on systemic perfusion in pulmonary arterial hypertension. Catheter Cardiovasc Interv 2013; 81 (05) 888-895
- 58 Chiu JS, Zuckerman WA, Turner ME. , et al. Balloon atrial septostomy in pulmonary arterial hypertension: effect on survival and associated outcomes. J Heart Lung Transplant 2015; 34 (03) 376-380
- 59 Potts WJ, Smith S, Gibson S. Anastomosis of the aorta to a pulmonary artery in certain types of congenital heart disease. Case Rep Child Meml Hosp Chic 1946; 5 (12) 704-718
- 60 Blanc J, Vouhé P, Bonnet D. Potts shunt in patients with pulmonary hypertension. N Engl J Med 2004; 350 (06) 623
- 61 Baruteau AE, Serraf A, Lévy M. , et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg 2012; 94 (03) 817-824
- 62 Kim SH, Jang WS, Lim HG, Kim YJ. Potts shunt in patients with primary pulmonary hypertension. Korean J Thorac Cardiovasc Surg 2015; 48 (01) 52-54
- 63 Fiszer R, Karwot B, Chodór B. , et al. Potts' shunt in a child with idiopathic pulmonary arterial hypertension - one-and-a-half year observation. Kardiochir Torakochirurgia Pol 2015; 12 (02) 170-172
- 64 Grady RM, Eghtesady P. Potts shunt and pediatric pulmonary hypertension: what we have learned. Ann Thorac Surg 2016; 101 (04) 1539-1543
- 65 Baruteau AE, Belli E, Boudjemline Y. , et al. Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients. Eur J Cardiothorac Surg 2015; 47 (03) e105-e110
- 66 Keogh AM, Nicholls M, Shaw M, Dhital K, Weintraub R, Winlaw DS. Modified Potts shunt in an adult with pulmonary arterial hypertension and recurrent syncope - three-year follow-up. Int J Cardiol 2015; 182: 36-37
- 67 Esch JJ, Shah PB, Cockrill BA. , et al. Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant 2013; 32 (04) 381-387
- 68 Salna M, van Boxtel B, Rosenzweig EB, Bacchetta M. Modified Potts shunt in an adult with idiopathic pulmonary arterial hypertension. Ann Am Thorac Soc 2017; 14 (04) 607-609
- 69 Xu Z, Owens G, Gordon D, Cain C, Ludomirsky A. Noninvasive creation of an atrial septal defect by histotripsy in a canine model. Circulation 2010; 121 (06) 742-749
- 70 Kula S, Atasayan V. Surgical and transcatheter management alternatives in refractory pulmonary hypertension: Potts shunt. Anatol J Cardiol 2015; 15 (10) 843-847
- 71 Punnoose L, Burkhoff D, Rich S, Horn EM. Right ventricular assist device in end-stage pulmonary arterial hypertension: insights from a computational model of the cardiovascular system. Prog Cardiovasc Dis 2012; 55 (02) 234-243.e2
- 72 Mauritz GJ, Marcus JT, Westerhof N, Postmus PE, Vonk-Noordegraaf A. Prolonged right ventricular post-systolic isovolumic period in pulmonary arterial hypertension is not a reflection of diastolic dysfunction. Heart 2011; 97 (06) 473-478
- 73 Rasmussen JT, Thenappan T, Benditt DG, Weir EK, Pritzker MR. Is cardiac resynchronization therapy for right ventricular failure in pulmonary arterial hypertension of benefit?. Pulm Circ 2014; 4 (04) 552-559
- 74 Hardziyenka M, Surie S, de Groot JR. , et al. Right ventricular pacing improves haemodynamics in right ventricular failure from pressure overload: an open observational proof-of-principle study in patients with chronic thromboembolic pulmonary hypertension. Europace 2011; 13 (12) 1753-1759
- 75 Berruezo A, De Potter T, Sitges M, Mansour F, Mont L, Brugada J. Bifocal right ventricular resynchronization for the failing right ventricle. Pacing Clin Electrophysiol 2011; 34 (08) e78-e81
- 76 Bando K, Armitage JM, Paradis IL. , et al. Indications for and results of single, bilateral, and heart-lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1994; 108 (06) 1056-1065
- 77 Gammie JS, Keenan RJ, Pham SM. , et al. Single- versus double-lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1998; 115 (02) 397-402 , discussion 402–403
- 78 Conte JV, Borja MJ, Patel CB, Yang SC, Jhaveri RM, Orens JB. Lung transplantation for primary and secondary pulmonary hypertension. Ann Thorac Surg 2001; 72 (05) 1673-1679 , discussion 1679–1680
- 79 Christie JD, Kotloff RM, Pochettino A. , et al. Clinical risk factors for primary graft failure following lung transplantation. Chest 2003; 124 (04) 1232-1241
- 80 King RC, Binns OA, Rodriguez F. , et al. Reperfusion injury significantly impacts clinical outcome after pulmonary transplantation. Ann Thorac Surg 2000; 69 (06) 1681-1685
- 81 Kuntz CL, Hadjiliadis D, Ahya VN. , et al. Risk factors for early primary graft dysfunction after lung transplantation: a registry study. Clin Transplant 2009; 23 (06) 819-830
- 82 Shah RJ, Diamond JM, Cantu E. , et al. Objective estimates improve risk stratification for primary graft dysfunction after lung transplantation. Am J Transplant 2015; 15 (08) 2188-2196
- 83 Pérez-Terán P, Roca O, Rodríguez-Palomares J. , et al. Influence of right ventricular function on the development of primary graft dysfunction after lung transplantation. J Heart Lung Transplant 2015; 34 (11) 1423-1429
- 84 Armstrong HF, Schulze PC, Kato TS, Bacchetta M, Thirapatarapong W, Bartels MN. Right ventricular stroke work index as a negative predictor of mortality and initial hospital stay after lung transplantation. J Heart Lung Transplant 2013; 32 (06) 603-608
- 85 Pérez-Terán P, Roca O, Rodríguez-Palomares J. , et al. Prospective validation of right ventricular role in primary graft dysfunction after lung transplantation. Eur Respir J 2016; 48 (06) 1732-1742
- 86 Liu Y, Liu Y, Su L, Jiang SJ. Recipient-related clinical risk factors for primary graft dysfunction after lung transplantation: a systematic review and meta-analysis. PLoS One 2014; 9 (03) e92773
- 87 Herve P, Humbert M, Sitbon O. , et al. Pathobiology of pulmonary hypertension. The role of platelets and thrombosis. Clin Chest Med 2001; 22 (03) 451-458
- 88 Hoeper MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12 (06) 1446-1449
- 89 Ritchie ME, Dávila-Román VG, Barzilai B. Dynamic right ventricular outflow obstruction after single-lung transplantation. Biplane transesophageal echocardiographic findings. Chest 1994; 105 (02) 610-611
- 90 Porteous MK, Ky B, Kirkpatrick JN. , et al. Diastolic dysfunction increases the risk of primary graft dysfunction after lung transplant. Am J Respir Crit Care Med 2016; 193 (12) 1392-1400
- 91 Yusen RD, Edwards LB, Kucheryavaya AY. , et al. The Registry of the International Society for Heart and Lung Transplantation: Thirty-second Official Adult Lung and Heart-Lung Transplantation Report--2015; Focus Theme: Early Graft Failure. J Heart Lung Transplant 2015; 34 (10) 1264-1277
- 92 Sato M, Waddell TK, Wagnetz U. , et al. Restrictive allograft syndrome (RAS): a novel form of chronic lung allograft dysfunction. J Heart Lung Transplant 2011; 30 (07) 735-742
- 93 Lau EM, Manes A, Celermajer DS, Galiè N. Early detection of pulmonary vascular disease in pulmonary arterial hypertension: time to move forward. Eur Heart J 2011; 32 (20) 2489-2498