Advertisement

Single- and Bilateral Lung Transplantation: Indications, Contraindications, Evaluation, and Requirements for Patients to Be Considered Eligible

  • Gerard J. Meachery
  • Paul A. Corris
Chapter

Abstract

Single- and double-lung transplantation epitomize the final therapeutic options available to selected patients with advanced, end-stage lung disease refractory to all methods of available medical management. The lack of evidence supporting effective lung transplant practices is widely acknowledged, necessitating the need for expert opinion practice guidelines. In this chapter we present the key recommendations for consideration when referring and listing an individual for lung transplantation. We critique seminal papers that have revised our selection process for single- and bilateral lung transplant recipients and outline the indications and contraindications unique to different lung pathologies. We define our current clinical practice based on the best available international expert opinions and review future developments of treatments and novel approaches to deal with the continued lack of suitable donor organs.

Keywords

Cystic fibrosis Chronic obstructive pulmonary disease Emphysema Interstitial lung disease Idiopathic pulmonary fibrosis Lung transplant Pulmonary arterial hypertension 

Abbreviations

BLT

Bilateral lung transplant

BMI

Body mass index

CF

Cystic fibrosis

CHDAPAH

Congenital heart disease-associated pulmonary arterial hypertension

COPD

Chronic obstructive pulmonary disease

CPB

Cardiopulmonary bypass

CT

Computed tomography

CTEPH

Chronic thromboembolic pulmonary hypertension

DLCO

Diffusing capacity for carbon monoxide

ECLS

Extracorporeal lung support

ECMO

Extracorporeal membrane oxygenation

FEV1

Forced expiratory volume in one second

FVC

Forced vital capacity

HLT

Heart-lung transplant

ILD

Interstitial lung disease

IPAH

Idiopathic pulmonary arterial hypertension

IPF

Idiopathic pulmonary fibrosis

ISHLT

International Society for Heart and Lung Transplantation

LAD

Lung assist device

LAS

Lung allocation score

LLLT

Living lobar lung transplantation

NSIP

Non-specific interstitial pneumonitis

NTM

Non-tuberculous mycobacteria

NYHA FC

New York Heart Association Functional Class

PAH

Pulmonary arterial hypertension

PH

Pulmonary hypertension

SLT

Single-lung transplant

SSc

Systemic sclerosis

UIP

Usual interstitial pneumonitis

UNOS

United Network for Organ Sharing

US

United States

References

  1. 1.
    Weill D, Benden C, Corris PA, Dark JH, Davis RD, Keshavjee S, et al. A consensus document for the selection of lung transplant candidates: 2014—an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34(1):1–15.PubMedCrossRefGoogle Scholar
  2. 2.
    Barrios JMV, Montero JR, Luna FS. Comorbidities impacting on prognosis after lung transplant. Arch Bronconeumol. 2014;50(1):25–33.Google Scholar
  3. 3.
    Smith PJ, Blumenthal JA, Trulock EP, Freedland KE, Carney RM, Davis RD, et al. Psychosocial predictors of mortality following lung transplantation. Am J Transplant. 2016;16(1):271–7.PubMedCrossRefPubMedCentralGoogle Scholar
  4. 4.
    Glanville AR, Estenne M. Indications, patient selection and timing of referral for lung transplantation. Eur Respir J. 2003;22(5):845–52.PubMedCrossRefPubMedCentralGoogle Scholar
  5. 5.
    Egan TM, Murray S, Bustami RT, Shearon TH, McCullough KP, Edwards LB, et al. Development of the new lung allocation system in the United States. Am J Transplant. 2006;6(5p2):1212–27.PubMedCrossRefPubMedCentralGoogle Scholar
  6. 6.
    Lingaraju R, Blumenthal NP, Kotloff RM, Christie J, Ahya VN, Sager JS, et al. Effects of lung allocation score on waiting list rankings and transplant procedures. J Heart Lung Transplant. 2006;25(9):1167–70.PubMedCrossRefPubMedCentralGoogle Scholar
  7. 7.
    Kozower BD, Meyers BF, Smith MA, De Oliveira NC, Cassivi SD, Guthrie TJ, et al. The impact of the lung allocation score on short-term transplantation outcomes: a multicenter study. J Thorac Cardiovasc Surg. 2008;135(1):166–71.PubMedCrossRefPubMedCentralGoogle Scholar
  8. 8.
    Egan TM, Kotloff RM. Pro/Con debate: lung allocation should be based on medical urgency and transplant survival and not on waiting time. Chest J. 2005;128(1):407–15.CrossRefGoogle Scholar
  9. 9.
    Kotloff RM, Thabut G. Lung transplantation. Am J Respir Crit Care Med. 2011;184(2):159–71.PubMedCrossRefPubMedCentralGoogle Scholar
  10. 10.
    Martinez FJ, Safrin S, Weycker D, Starko KM, Bradford WZ, King TE, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005;142(12_Part_1):963–7.PubMedCrossRefPubMedCentralGoogle Scholar
  11. 11.
    Lynch JP, Huynh RH, Fishbein MC, Saggar R, Belperio JA, Weigt SS. Idiopathic pulmonary fibrosis: epidemiology, clinical features, prognosis, and management. Semin Respir Crit Care Med. 2016;37(3):331–57.PubMedCrossRefPubMedCentralGoogle Scholar
  12. 12.
    Russo MJ, Iribarne A, Hong KN, Davies RR, Xydas S, Takayama H, et al. High lung allocation score is associated with increased morbidity and mortality following transplantation. Chest J. 2010;137(3):651–7.CrossRefGoogle Scholar
  13. 13.
    Liu V, Zamora MR, Dhillon GS, Weill D. Increasing lung allocation scores predict worsened survival among lung transplant recipients. Am J Transplant. 2010;10(4):915–20.PubMedPubMedCentralCrossRefGoogle Scholar
  14. 14.
    Egan TM, Edwards LB. Effect of the lung allocation score on lung transplantation in the United States. J Heart Lung Transplant. 2016;35(4):433–9.PubMedCrossRefPubMedCentralGoogle Scholar
  15. 15.
    Maxwell BG, Mooney JJ, Lee PHU, Levitt JE, Chhatwani L, Nicolls MR, et al. Increased resource use in lung transplant admissions in the lung allocation score era. Am J Respir Crit Care Med. 2015;191(3):302–8.PubMedPubMedCentralCrossRefGoogle Scholar
  16. 16.
    Fahim A, Crooks MG, Wilmot R, Campbell AP, Morice AH, Hart SP. Serum carcinoembryonic antigen correlates with severity of idiopathic pulmonary fibrosis. Respirology. 2012;17(8):1247–52.PubMedCrossRefPubMedCentralGoogle Scholar
  17. 17.
    Hadjiliadis D, Tapson VF, Davis RD, Palmer SM. Prognostic value of serum carcinoembryonic antigen levels in patients who undergo lung transplantation. J Heart Lung Transplant. 2001;20(12):1305–9.PubMedCrossRefPubMedCentralGoogle Scholar
  18. 18.
    Christie JD, Edwards LB, Aurora P, Dobbels F, Kirk R, Rahmel AO, et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-sixth official adult lung and heart-lung transplantation report—2009. J Heart Lung Transplant. 2009;28(10):1031–49.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    Tomaszek SC, Fibla JJ, Dierkhising RA, Scott JP, Shen K-HR, Wigle DA, et al. Outcome of lung transplantation in elderly recipients. Eur J Cardiothorac Surg. 2011;39(5):726–31.PubMedCrossRefPubMedCentralGoogle Scholar
  20. 20.
    Thabut G, Ravaud P, Christie JD, Castier Y, Fournier M, Mal H, et al. Determinants of the survival benefit of lung transplantation in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2008;177(10):1156–63.PubMedCrossRefPubMedCentralGoogle Scholar
  21. 21.
    Fischer S, Meyer K, Tessmann R, Meyer A, Gohrbandt B, Simon A, et al. Outcome following single vs bilateral lung transplantation in recipients 60 years of age and older. Transplant Proc. 2005;37(2):1369–70.PubMedCrossRefPubMedCentralGoogle Scholar
  22. 22.
    Aris RM, Neuringer IP, Weiner MA, Egan TM, Ontjes D. Severe osteoporosis before and after lung transplantation. Chest J. 1996;109(5):1176–83.CrossRefGoogle Scholar
  23. 23.
    Tschopp O, Boehler A, Speich R, Weder W, Seifert B, Russi EW, et al. Osteoporosis before lung transplantation: association with low body mass index, but not with underlying disease. Am J Transplant. 2002;2(2):167–72.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Aris RM, Lester GE, Renner JB, Winders A, Denene Blackwood A, Lark RK, et al. Efficacy of pamidronate for osteoporosis in patients with cystic fibrosis following lung transplantation. Am J Respir Crit Care Med. 2000;162(3):941–6.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Shane E, Silverberg SJ, Donovan D, Papadopoulos A, Staron RB, Addesso V, et al. Osteoporosis in lung transplantation candidates with end-stage pulmonary disease. Am J Med. 1996;101(3):262–9.PubMedCrossRefPubMedCentralGoogle Scholar
  26. 26.
    Braith RW, Conner JA, Fulton MN, Lisor CF, Casey DP, Howe KS, et al. Comparison of alendronate vs alendronate plus mechanical loading as prophylaxis for osteoporosis in lung transplant recipients: a pilot study. J Heart Lung Transplant. 2007;26(2):132–7.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Munro PE, Holland AE, Bailey M, Button BM, Snell GI. Pulmonary rehabilitation following lung transplantation. Transplant Proc. 2009;41(1):292–5.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    Caffarelli C, Gonnelli S, Pitinca MDT, Francolini V, Fui A, Bargagli E, et al. Idiopathic pulmonary fibrosis a rare disease with severe bone fragility. Intern Emerg Med. 2016;11(8):1087–94.PubMedCrossRefPubMedCentralGoogle Scholar
  29. 29.
    Tabarelli W, Bonatti H, Tabarelli D, Eller M, Müller L, Ruttmann E, et al. Long term complications following 54 consecutive lung transplants. J Thorac Dis. 2016;8(6):1234.PubMedPubMedCentralCrossRefGoogle Scholar
  30. 30.
    Culver DA, Mazzone PJ, Khandwala F, Blazey HC, DeCamp MM, Chapman JT, et al. Discordant utility of ideal body weight and body mass index as predictors of mortality in lung transplant recipients. J Heart Lung Transplant. 2005;24(2):137–44.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Carreno MC, Piedad UG, Maite L, Andrés V, Francisca P, Cesar B, et al. Hepatitis C virus infection after lung transplantation: dim prognosis. J Heart Lung Transplant. 2001;20(2):224.PubMedCrossRefPubMedCentralGoogle Scholar
  32. 32.
    Cotler SJ, Jensen DM, Kesten S. Hepatitis C virus infection and lung transplantation: a survey of practices. J Heart Lung Transplant. 1999;18(5):456–9.PubMedCrossRefPubMedCentralGoogle Scholar
  33. 33.
    Fong T-L, Cho YW, Hou L, Hutchinson IV, Barbers RG, Herrington CS. Outcomes after lung transplantation and practices of lung transplant programs in the United States regarding hepatitis C seropositive recipients. Transplantation. 2011;91(11):1293–6.PubMedCrossRefPubMedCentralGoogle Scholar
  34. 34.
    Doucette KE, Weinkauf J, Jackson K, Lein D. Survival following lung transplantation is not impacted by hepatitis C infection. Am J Transplant. 2012;12(S3):27–542.Google Scholar
  35. 35.
    Kern RM, Seethamraju H, Blanc PD, Sinha N, Loebe M, Golden J, et al. The feasibility of lung transplantation in HIV-seropositive patients. Ann Am Thorac Soc. 2014;11(6):882–9.PubMedPubMedCentralCrossRefGoogle Scholar
  36. 36.
    Morabito V, Grossi P, Lombardini L, Ricci A, Trapani S, Peritore D, et al. Solid organ transplantation in HIV+ recipients: Italian experience. Transplant Proc. 2016;48(2):424–30.PubMedCrossRefGoogle Scholar
  37. 37.
    De Soyza A, Meachery G, Hester KLM, Nicholson A, Parry G, Tocewicz K, et al. Lung transplantation for patients with cystic fibrosis and Burkholderia cepacia complex infection: a single-center experience. J Heart Lung Transplant. 2010;29(12):1395–404.PubMedCrossRefGoogle Scholar
  38. 38.
    Olland A, Falcoz P-E, Kessler R, Massard G. Should cystic fibrosis patients infected with Burkholderia cepacia complex be listed for lung transplantation? Interact Cardiovasc Thorac Surg. 2012;13(6):631–4.CrossRefGoogle Scholar
  39. 39.
    Bruckner BA, Motomura T, Kaleekal TS, Jyothula SS, Scheinin S, Bunge RR, et al. Clinical outcomes in elderly lung transplant recipients 70 years and older. J Heart Lung Transplant. 2013;32(4):S266–S7.CrossRefGoogle Scholar
  40. 40.
    Hayanga AJ, Aboagye JK, Hayanga HE, Morrell M, Huffman L, Shigemura N, et al. Contemporary analysis of early outcomes after lung transplantation in the elderly using a national registry. J Heart Lung Transplant. 2015;34(2):182–8.PubMedCrossRefPubMedCentralGoogle Scholar
  41. 41.
    Nathan SD, Albera C, Bradford WZ, Costabel U, Du Bois RM, Fagan EA, et al. Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis. Thorax. 2016;71(5):429–35.PubMedPubMedCentralCrossRefGoogle Scholar
  42. 42.
    Raghu G, Rochwerg B, Zhang Y, Garcia CAC, Azuma A, Behr J, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3–19.PubMedCrossRefPubMedCentralGoogle Scholar
  43. 43.
    Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82.PubMedCrossRefPubMedCentralGoogle Scholar
  44. 44.
    Kolb M, Richeldi L, Behr J, Maher TM, Tang W, Stowasser S, et al. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. Thorax. 2017;72(4):340–6.PubMedCrossRefPubMedCentralGoogle Scholar
  45. 45.
    Delanote I, Wuyts WA, Yserbyt J, Verbeken EK, Verleden GM, Vos R. Safety and efficacy of bridging to lung transplantation with antifibrotic drugs in idiopathic pulmonary fibrosis: a case series. BMC Pulm Med. 2016;16(1):156.PubMedPubMedCentralCrossRefGoogle Scholar
  46. 46.
    Stehlik J, Edwards LB, Kucheryavaya AY, Aurora P, Christie JD, Kirk R, et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-seventh official adult heart transplant report—2010. J Heart Lung Transplant. 2010;29(10):1089–103.PubMedCrossRefPubMedCentralGoogle Scholar
  47. 47.
    Thabut G, Christie JD, Ravaud P, Castier Y, Brugière O, Fournier M, et al. Survival after bilateral versus single lung transplantation for patients with chronic obstructive pulmonary disease: a retrospective analysis of registry data. Lancet. 2008;371(9614):744–51.PubMedCrossRefGoogle Scholar
  48. 48.
    Thabut G, Christie JD, Ravaud P, Castier Y, Dauriat G, Jebrak G, et al. Survival after bilateral versus single-lung transplantation for idiopathic pulmonary fibrosis. Ann Intern Med. 2009;151(11):767–74.PubMedCrossRefPubMedCentralGoogle Scholar
  49. 49.
    Kaiser LR, Pasque MK, Trulock EP, Low DE, Dresler CM, Cooper JD. Bilateral sequential lung transplantation: the procedure of choice for double-lung replacement. Ann Thorac Surg. 1991;52(3):438–46.PubMedCrossRefPubMedCentralGoogle Scholar
  50. 50.
    Groui TLT. Unilateral lung transplantation for pulmonary fibrosis. N Engl J Med. 1986;314:1140–5.CrossRefGoogle Scholar
  51. 51.
    Paoletti L, Whelan TPM. Lung transplantation for interstitial lung disease. Current Respiratory Care Reports. 2014;3(3):96–102.CrossRefGoogle Scholar
  52. 52.
    Mal H, Andreassian B, Pamela F, Duchatelle J-P, Rondeau E, Dubois F, et al. Unilateral lung transplantation in end-stage pulmonary emphysema1• 2. Am Rev Respir Dis. 1989;140:797.PubMedCrossRefGoogle Scholar
  53. 53.
    Schaffer JM, Singh SK, Reitz BA, Zamanian RT, Mallidi HR. Single-vs double-lung transplantation in patients with chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis since the implementation of lung allocation based on medical need. JAMA. 2015;313(9):936–48.PubMedCrossRefPubMedCentralGoogle Scholar
  54. 54.
    Keating D, Levvey B, Kotsimbos T, Whitford H, Westall G, Williams T, et al. Lung transplantation in pulmonary fibrosis: challenging early outcomes counterbalanced by surprisingly good outcomes beyond 15 years. Transplant Proc. 2009;41(1):289–91.PubMedCrossRefPubMedCentralGoogle Scholar
  55. 55.
    Mason DP, Brizzio ME, Alster JM, McNeill AM, Murthy SC, Budev MM, et al. Lung transplantation for idiopathic pulmonary fibrosis. Ann Thorac Surg. 2007;84(4):1121–8.PubMedCrossRefPubMedCentralGoogle Scholar
  56. 56.
    De Oliveira NC, Osaki S, Maloney J, Cornwell RD, Meyer KC. Lung transplant for interstitial lung disease: outcomes for single versus bilateral lung transplantation. Interact Cardiovasc Thorac Surg. 2012;14(3):263–7.PubMedCrossRefPubMedCentralGoogle Scholar
  57. 57.
    Nwakanma LU, Simpkins CE, Williams JA, Chang DC, Borja MC, Conte JV, et al. Impact of bilateral versus single lung transplantation on survival in recipients 60 years of age and older: analysis of United Network for Organ Sharing database. J Thorac Cardiovasc Surg. 2007;133(2):541–7.PubMedCrossRefPubMedCentralGoogle Scholar
  58. 58.
    Taylor DO, Edwards LB, Boucek MM, Trulock EP, Aurora P, Christie J, et al. Registry of the International Society for Heart and Lung Transplantation: twenty-fourth official adult heart transplant report—2007. J Heart Lung Transplant. 2007;26(8):769–81.PubMedCrossRefPubMedCentralGoogle Scholar
  59. 59.
    Titman A, Rogers CA, Bonser RS, Banner NR, Sharples LD. Disease-specific survival benefit of lung transplantation in adults: a national cohort study. Am J Transplant. 2009;9(7):1640–9.PubMedCrossRefPubMedCentralGoogle Scholar
  60. 60.
    Reitz BA, Wallwork JL, Hunt SA, Pennock JL, Billingham ME, Oyer PE, et al. Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med. 1982;306(10):557–64.PubMedCrossRefPubMedCentralGoogle Scholar
  61. 61.
    Christie JD, Edwards LB, Kucheryavaya AY, Benden C, Dobbels F, Kirk R, et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-eighth adult lung and heart-lung transplant report—2011. J Heart Lung Transplant. 2011;30(10):1104–22.PubMedCrossRefPubMedCentralGoogle Scholar
  62. 62.
    Aigner C, Mazhar S, Jaksch P, Seebacher G, Taghavi S, Marta G, et al. Lobar transplantation, split lung transplantation and peripheral segmental resection-reliable procedures for downsizing donor lungs. Eur J Cardiothorac Surg. 2004;25(2):179–83.PubMedCrossRefPubMedCentralGoogle Scholar
  63. 63.
    Inci I, Schuurmans MM, Kestenholz P, Schneiter D, Hillinger S, Opitz I, et al. Long-term outcomes of bilateral lobar lung transplantation. Eur J Cardiothorac Surg. 2013;43(6):1220–5.PubMedCrossRefPubMedCentralGoogle Scholar
  64. 64.
    Barr ML, Starnes VA. Living lobar lung transplantation. Adult chest surgery. 2nd ed. New York: McGraw Hill; 2013.Google Scholar
  65. 65.
    Spray TL, Mallory GB, Canter CB, Dulleston CB. Pediatric lung transplantation: indications, techniques and early results. J Thorac Cardiovasc Surg. 1994;107:990–1000.PubMedGoogle Scholar
  66. 66.
    Starnes VA, Barr ML, Cohen RG, Hagen JA, Wells WJ, Horn MV, et al. Living-donor lobar lung transplantation experience: intermediate results. J Thorac Cardiovasc Surg. 1996;112(5):1284–91.PubMedCrossRefGoogle Scholar
  67. 67.
    Couetil J-PA, Tolan MJ, Loulmet DF, Guinvarch A, Chevalier PG, Achkar A, et al. Pulmonary bipartitioning and lobar transplantation: a new approach to donor organ shortage. J Thorac Cardiovasc Surg. 1997;113(3):529–37.PubMedCrossRefGoogle Scholar
  68. 68.
    Artemiou O, Birsan T, Taghavi S, Eichler I, Wisser W, Wolner E, et al. Bilateral lobar transplantation with the split lung technique. J Thorac Cardiovasc Surg. 1999;118(2):369–70.PubMedCrossRefGoogle Scholar
  69. 69.
    Fischer S, Hoeper MM, Tomaszek S, Simon A, Gottlieb J, Welte T, et al. Bridge to lung transplantation with the extracorporeal membrane ventilator Novalung in the veno-venous mode: the initial Hannover experience. ASAIO J. 2007;53(2):168–70.PubMedCrossRefGoogle Scholar
  70. 70.
    Strueber M. Bridges to lung transplantation. Curr Opin Organ Transplant. 2011;16(5):458–61.PubMedCrossRefPubMedCentralGoogle Scholar
  71. 71.
    Diaz-Guzman E, Hoopes CW, Zwischenberger JB. The evolution of extracorporeal life support as a bridge to lung transplantation. ASAIO J. 2013;59(1):3–10.PubMedCrossRefPubMedCentralGoogle Scholar
  72. 72.
    Strueber M. Extracorporeal support as a bridge to lung transplantation. Curr Opin Crit Care. 2010;16(1):69–73.PubMedCrossRefPubMedCentralGoogle Scholar
  73. 73.
    Cypel M, Yeung JC, Machuca T, Chen M, Singer LG, Yasufuku K, et al. Experience with the first 50 ex vivo lung perfusions in clinical transplantation. J Thorac Cardiovasc Surg. 2012;144(5):1200–7.PubMedCrossRefPubMedCentralGoogle Scholar
  74. 74.
    de Perrot M, Granton JT, McRae K, Pierre AF, Singer LG, Waddell TK, et al. Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single-center experience. J Thorac Cardiovasc Surg. 2012;143(4):910–8.PubMedCrossRefPubMedCentralGoogle Scholar
  75. 75.
    de Perrot M, Granton JT, McRae K, Cypel M, Pierre A, Waddell TK, 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(9):997–1002.PubMedCrossRefPubMedCentralGoogle Scholar
  76. 76.
    Javidfar J, Bacchetta M. Bridge to lung transplantation with extracorporeal membrane oxygenation support. Curr Opin Organ Transplant. 2012;17(5):496–502.PubMedCrossRefPubMedCentralGoogle Scholar
  77. 77.
    Toyoda Y, Bhama JK, Shigemura N, Zaldonis D, Pilewski J, Crespo M, et al. Efficacy of extracorporeal membrane oxygenation as a bridge to lung transplantation. J Thorac Cardiovasc Surg. 2013;145(4):1065–71.PubMedCrossRefPubMedCentralGoogle Scholar
  78. 78.
    Shafii AE, Mason DP, Brown CR, Vakil N, Johnston DR, McCurry KR, et al. Growing experience with extracorporeal membrane oxygenation as a bridge to lung transplantation. ASAIO J. 2012;58(5):526–9.PubMedCrossRefPubMedCentralGoogle Scholar
  79. 79.
    Hoopes CW, Kukreja J, Golden J, Davenport DL, Diaz-Guzman E, Zwischenberger JB. Extracorporeal membrane oxygenation as a bridge to pulmonary transplantation. J Thorac Cardiovasc Surg. 2013;145(3):862–8.PubMedCrossRefPubMedCentralGoogle Scholar
  80. 80.
    Bittner HB, Lehmann S, Rastan A, Garbade J, Binner C, Mohr FW, et al. Outcome of extracorporeal membrane oxygenation as a bridge to lung transplantation and graft recovery. Ann Thorac Surg. 2012;94(3):942–50.PubMedCrossRefPubMedCentralGoogle Scholar
  81. 81.
    Fuehner T, Kuehn C, Hadem J, Wiesner O, Gottlieb J, Tudorache I, et al. Extracorporeal membrane oxygenation in awake patients as bridge to lung transplantation. Am J Respir Crit Care Med. 2012;185(7):763–8.PubMedCrossRefPubMedCentralGoogle Scholar
  82. 82.
    MacLaren G, Combes A, Bartlett RH. Contemporary extracorporeal membrane oxygenation for adult respiratory failure: life support in the new era. Intensive Care Med. 2012;38(2):210–20.PubMedCrossRefPubMedCentralGoogle Scholar
  83. 83.
    Gottlieb J, Warnecke G, Hadem J, Dierich M, Wiesner O, Fühner T, et al. Outcome of critically ill lung transplant candidates on invasive respiratory support. Intensive Care Med. 2012;38(6):968–75.PubMedCrossRefPubMedCentralGoogle Scholar
  84. 84.
    Vermeijden JW, Zijlstra JG, Erasmus ME, van der Bij W, Verschuuren EA. Lung transplantation for ventilator-dependent respiratory failure. J Heart Lung Transplant. 2009;28(4):347–51.PubMedCrossRefPubMedCentralGoogle Scholar
  85. 85.
    Strueber M, Hoeper MM, Fischer S, Cypel M, Warnecke G, Gottlieb J, et al. Bridge to thoracic organ transplantation in patients with pulmonary arterial hypertension using a pumpless lung assist device. Am J Transplant. 2009;9(4):853–7.PubMedCrossRefPubMedCentralGoogle Scholar
  86. 86.
    Taylor K, Holtby H. Emergency interventional lung assist for pulmonary hypertension. Anesth Analg. 2009;109(2):382–5.PubMedCrossRefPubMedCentralGoogle Scholar
  87. 87.
    Hook JL, Lederer DJ. Selecting lung transplant candidates: where do current guidelines fall short? Expert Rev Respir Med. 2012;6(1):51–61.PubMedPubMedCentralCrossRefGoogle Scholar
  88. 88.
    Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326(18):1187–91.PubMedCrossRefPubMedCentralGoogle Scholar
  89. 89.
    Mayer-Hamblett N, Rosenfeld M, Emerson J, Goss CH, Aitken ML. Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality. Am J Respir Crit Care Med. 2002;166(12):1550–5.PubMedCrossRefPubMedCentralGoogle Scholar
  90. 90.
    Augarten A, Akons H, Aviram M, Bentur L, Blau H, Picard E, et al. Prediction of mortality and timing of referral for lung transplantation in cystic fibrosis patients. Pediatr Transplant. 2001;5(5):339–42.PubMedCrossRefPubMedCentralGoogle Scholar
  91. 91.
    Milla CE, Warwick WJ. Risk of death in cystic fibrosis patients with severely compromised lung function. Chest J. 1998;113(5):1230–4.CrossRefGoogle Scholar
  92. 92.
    Aurora P, Wade A, Whitmore P, Whitehead B. A model for predicting life expectancy of children with cystic fibrosis. Eur Respir J. 2000;16(6):1056–60.PubMedCrossRefPubMedCentralGoogle Scholar
  93. 93.
    Vizza CD, Yusen RD, Lynch JP, Fedele F, Alexander Patterson G, Trulock EP. Outcome of patients with cystic fibrosis awaiting lung transplantation. Am J Respir Crit Care Med. 2000;162(3):819–25.PubMedCrossRefPubMedCentralGoogle Scholar
  94. 94.
    Sharma R, Florea VG, Bolger AP, Doehner W, Florea ND, Coats AJS, et al. Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax. 2001;56(10):746–50.PubMedPubMedCentralCrossRefGoogle Scholar
  95. 95.
    Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson RL. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol. 2002;34(2):91–100.PubMedCrossRefPubMedCentralGoogle Scholar
  96. 96.
    Liou TG, Adler FR, FitzSimmons SC, Cahill BC, Hibbs JR, Marshall BC. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol. 2001;153(4):345–52.PubMedPubMedCentralCrossRefGoogle Scholar
  97. 97.
    Fauroux B, Hart N, Belfar S, Boulé M, Tillous-Borde I, Bonnet D, et al. Burkholderia cepacia is associated with pulmonary hypertension and increased mortality among cystic fibrosis patients. J Clin Microbiol. 2004;42(12):5537–41.PubMedPubMedCentralCrossRefGoogle Scholar
  98. 98.
    Alexander BD, Petzold EW, Reller LB, Palmer SM, Davis RD, Woods CW, et al. Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia cepacia complex. Am J Transplant. 2008;8(5):1025–30.PubMedCrossRefPubMedCentralGoogle Scholar
  99. 99.
    Tonelli AR, Fernandez-Bussy S, Lodhi S, Akindipe OA, Carrie RD, Hamilton K, et al. Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival. J Heart Lung Transplant. 2010;29(8):865–72.PubMedCrossRefPubMedCentralGoogle Scholar
  100. 100.
    Andersen KH, Schultz HHL, Nyholm B, Iversen MP, Gustafsson F, Carlsen J. Pulmonary hypertension as a risk factor of mortality after lung transplantation. Clin Transplant. 2016;30(4):357–64.PubMedCrossRefPubMedCentralGoogle Scholar
  101. 101.
    Scarsini R, Prioli MA, Milano EG, Castellani C, Pesarini G, Assael BM, et al. Hemodynamic predictors of long term survival in end stage cystic fibrosis. Int J Cardiol. 2016;202:221–5.PubMedCrossRefPubMedCentralGoogle Scholar
  102. 102.
    Galiè N, Humbert M, Vachiery J-L, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67–119.PubMedCrossRefPubMedCentralGoogle Scholar
  103. 103.
    Manika K, Pitsiou GG, Boutou AK, Tsaoussis V, Chavouzis N, Antoniou M, et al. The impact of pulmonary arterial pressure on exercise capacity in mild-to-moderate cystic fibrosis: a case control study. Pulm Med. 2012;2012:252345.PubMedPubMedCentralCrossRefGoogle Scholar
  104. 104.
    Mourani PM, Sontag MK, Younoszai A, Ivy DD, Abman SH. Clinical utility of echocardiography for the diagnosis and management of pulmonary vascular disease in young children with chronic lung disease. Pediatrics. 2008;121(2):317–25.PubMedPubMedCentralCrossRefGoogle Scholar
  105. 105.
    Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H, Clinical Practice Guidelines on Growth and Nutrition Subcommittee, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108(5):832–9.PubMedCrossRefPubMedCentralGoogle Scholar
  106. 106.
    Efrati O, Mei-Zahav M, Rivlin J, Kerem E, Blau H, Barak A, et al. Long term nutritional rehabilitation by gastrostomy in Israeli patients with cystic fibrosis: clinical outcome in advanced pulmonary disease. J Pediatr Gastroenterol Nutr. 2006;42(2):222–8.PubMedCrossRefPubMedCentralGoogle Scholar
  107. 107.
    Floto RA, Olivier KN, Saiman L, Daley CL, Herrmann J-L, Nick JA, et al. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis. Thorax. 2016;71(Suppl 1):i1–22.PubMedCrossRefPubMedCentralGoogle Scholar
  108. 108.
    Leitão JH, Sousa SA, Ferreira AS, Ramos CG, Silva IN, Moreira LM. Pathogenicity, virulence factors, and strategies to fight against Burkholderia cepacia complex pathogens and related species. Appl Microbiol Biotechnol. 2010;87(1):31–40.PubMedCrossRefPubMedCentralGoogle Scholar
  109. 109.
    Coenye T, Vandamme P, Govan JRW, LiPuma JJ. Taxonomy and identification of the Burkholderia cepacia complex. J Clin Microbiol. 2001;39(10):3427–36.PubMedPubMedCentralCrossRefGoogle Scholar
  110. 110.
    Mahenthiralingam E, Baldwin A, Vandamme P. Burkholderia cepacia complex infection in patients with cystic fibrosis. J Med Microbiol. 2002;51(7):533–8.PubMedCrossRefPubMedCentralGoogle Scholar
  111. 111.
    Parke JL, Gurian-Sherman D. Diversity of the Burkholderia cepacia complex and implications for risk assessment of biological control strains. Annu Rev Phytopathol. 2001;39(1):225–58.PubMedCrossRefPubMedCentralGoogle Scholar
  112. 112.
    Mahenthiralingam E, Urban TA, Goldberg JB. The multifarious, multireplicon Burkholderia cepacia complex. Nat Rev Microbiol. 2005;3(2):144–56.PubMedCrossRefPubMedCentralGoogle Scholar
  113. 113.
    Mahenthiralingam E, Vandamme P. Taxonomy and pathogenesis of the Burkholderia cepacia complex. Chron Respir Dis. 2005;2(4):209–17.PubMedCrossRefPubMedCentralGoogle Scholar
  114. 114.
    Schaedel C, De Monestrol I, Hjelte L, Johannesson M, Kornfält R, Lindblad A, et al. Predictors of deterioration of lung function in cystic fibrosis. Pediatr Pulmonol. 2002;33(6):483–91.PubMedCrossRefPubMedCentralGoogle Scholar
  115. 115.
    Egan TM, Detterbeck FC, Mill MR, Bleiweis MS, Aris R, Paradowski L, et al. Long term results of lung transplantation for cystic fibrosis. Eur J Cardiothorac Surg. 2002;22(4):602–9.PubMedCrossRefPubMedCentralGoogle Scholar
  116. 116.
    Meachery G, De Soyza A, Nicholson A, Parry G, Hasan A, Tocewicz K, et al. Outcomes of lung transplantation for cystic fibrosis in a large UK cohort. Thorax. 2008;63(8):725–31.PubMedCrossRefPubMedCentralGoogle Scholar
  117. 117.
    De Soyza A, Morris K, McDowell A, Doherty C, Archer L, Perry J, et al. Prevalence and clonality of Burkholderia cepacia complex genomovars in UK patients with cystic fibrosis referred for lung transplantation. Thorax. 2004;59(6):526–8.PubMedPubMedCentralCrossRefGoogle Scholar
  118. 118.
    Nash EF, Coonar A, Kremer R, Tullis E, Hutcheon M, Singer LG, et al. Survival of Burkholderia cepacia sepsis following lung transplantation in recipients with cystic fibrosis. Transpl Infect Dis. 2010;12(6):551–4.PubMedCrossRefPubMedCentralGoogle Scholar
  119. 119.
    Qvist T, Pressler T, Høiby N, Katzenstein TL. Shifting paradigms of nontuberculous mycobacteria in cystic fibrosis. Respir Res. 2014;15(1):41.PubMedPubMedCentralCrossRefGoogle Scholar
  120. 120.
    Lobo LJ, Chang LC, Esther CR, Gilligan PH, Tulu Z, Noone PG. Lung transplant outcomes in cystic fibrosis patients with pre-operative Mycobacterium abscessus respiratory infections. Clin Transplant. 2013;27(4):523–9.PubMedCrossRefPubMedCentralGoogle Scholar
  121. 121.
    Guirguis-Blake JM, Senger CA, Webber EM, Mularski RA, Whitlock EP. Screening for chronic obstructive pulmonary disease: evidence report and systematic review for the US Preventive Services Task Force. JAMA. 2016;315(13):1378–93.PubMedCrossRefPubMedCentralGoogle Scholar
  122. 122.
    Mannino DM, Kiri VA. Changing the burden of COPD mortality. Int J Chron Obstruct Pulmon Dis. 2006;1(3):219–33.PubMedPubMedCentralGoogle Scholar
  123. 123.
    Lane CR, Tonelli AR. Lung transplantation in chronic obstructive pulmonary disease: patient selection and special considerations. Int J Chron Obstruct Pulmon Dis. 2015;10:2137.PubMedPubMedCentralGoogle Scholar
  124. 124.
    Celli BR, Cote CG, Marin JM, Casanova C, Montes de Oca M, Mendez RA, et al. The body-mass index, airflow obstruction, dyspnea, and exercise capacity index in chronic obstructive pulmonary disease. N Engl J Med. 2004;350(10):1005–12.PubMedCrossRefPubMedCentralGoogle Scholar
  125. 125.
    D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med. 1991;115(5):343–9.PubMedCrossRefPubMedCentralGoogle Scholar
  126. 126.
    Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Hervé P, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol. 2002;40(4):780–8.PubMedCrossRefPubMedCentralGoogle Scholar
  127. 127.
    McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension the impact of epoprostenol therapy. Circulation. 2002;106(12):1477–82.PubMedCrossRefPubMedCentralGoogle Scholar
  128. 128.
    Conte JV, Gaine SP, Orens JB, Harris T, Rubin LJ. The influence of continuous intravenous prostacyclin therapy for primary pulmonary hypertension on the timing and outcome of transplantation. J Heart Lung Transplant. 1998;17(7):679–85.PubMedPubMedCentralGoogle Scholar
  129. 129.
    Robbins IM, Christman BW, Newman JH, Matlock R, Loyd JE. A survey of diagnostic practices and the use of epoprostenol in patients with primary pulmonary hypertension. Chest J. 1998;114(5):1269–75.CrossRefGoogle Scholar
  130. 130.
    Hoeper MM, Barberà JA, Channick RN, Hassoun PM, Lang IM, Manes A, et al. Diagnosis, assessment, and treatment of non-pulmonary arterial hypertension pulmonary hypertension. J Am Coll Cardiol. 2009;54(1s1):S85–96.PubMedCrossRefPubMedCentralGoogle Scholar
  131. 131.
    Condliffe R, Kiely DG, Gibbs JSR, Corris PA, Peacock AJ, Jenkins DP, et al. Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2008;177(10):1122–7.PubMedCrossRefGoogle Scholar
  132. 132.
    Trulock EP. Lung transplantation and atrial septostomy for pulmonary arterial hypertension. In: Hill NS, Farber HW, editors. Contemporary cardiology: pulmonary hypertension. New York: Springer; 2008. p. 383–403.CrossRefGoogle Scholar
  133. 133.
    Galiè N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353(20):2148–57.PubMedCrossRefGoogle Scholar
  134. 134.
    Ghofrani HA, Seeger W, Grimminger F. Imatinib for the treatment of pulmonary arterial hypertension. N Engl J Med. 2005;353(13):1412–3.PubMedCrossRefGoogle Scholar
  135. 135.
    Galiè N, Rubin LJ, Hoeper MM, Jansa P, Al-Hiti H, Meyer GMB, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet. 2008;371(9630):2093–100.PubMedCrossRefGoogle Scholar
  136. 136.
    Oudiz RJ, Galiè N, Olschewski H, Torres F, Frost A, Ghofrani HA, et al. Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(21):1971–81.PubMedCrossRefGoogle Scholar
  137. 137.
    Rosenkranz S. Pulmonary hypertension: current diagnosis and treatment. Clin Res Cardiol. 2007;96:527–41.PubMedCrossRefPubMedCentralGoogle Scholar
  138. 138.
    Macchia A, Marchioli R, Tognoni G, Scarano M, Marfisi R, Tavazzi L, et al. Systematic review of trials using vasodilators in pulmonary arterial hypertension: why a new approach is needed. Am Heart J. 2010;159(2):245–57.PubMedCrossRefPubMedCentralGoogle Scholar
  139. 139.
    Barst RJ, Gibbs JSR, Ghofrani HA, Hoeper MM, McLaughlin VV, Rubin LJ, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(1s1):S78–84.PubMedPubMedCentralCrossRefGoogle Scholar
  140. 140.
    Galiè N, Manes A, Negro L, Palazzini M, Reggiani MLB, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. 2009;30(4):394–403.PubMedPubMedCentralCrossRefGoogle Scholar
  141. 141.
    Ghofrani H-A, Galiè N, Grimminger F, Grünig E, Humbert M, Jing Z-C, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369(4):330–40.PubMedCrossRefPubMedCentralGoogle Scholar
  142. 142.
    Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani H-A, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809–18.PubMedCrossRefPubMedCentralGoogle Scholar
  143. 143.
    Hoeper MM, Leuchte H, Halank M, Wilkens H, Meyer FJ, Seyfarth HJ, et al. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2006;28(4):691–4.PubMedCrossRefPubMedCentralGoogle Scholar
  144. 144.
    Hoeper MM, Markevych I, Spiekerkoetter E, Welte T, Niedermeyer J. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J. 2005;26(5):858–63.PubMedCrossRefPubMedCentralGoogle Scholar
  145. 145.
    Simonneau G, Barst RJ, Galie N, Naeije R, Rich S, Bourge RC, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002;165(6):800–4.PubMedCrossRefPubMedCentralGoogle Scholar
  146. 146.
    Galié N, Badesch D, Oudiz R, Simonneau G, McGoon MD, Keogh AM, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2005;46(3):529–35.PubMedCrossRefPubMedCentralGoogle Scholar
  147. 147.
    Rubin LJ, Badesch DB, Barst RJ, Galiè N, Black CM, Keogh A, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346(12):896–903.PubMedCrossRefPubMedCentralGoogle Scholar
  148. 148.
    Sitbon O, Humbert M, Jaïs X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111(23):3105–11.PubMedCrossRefGoogle Scholar
  149. 149.
    Galiè N, Brundage BH, Ghofrani HA, Oudiz RJ, Simonneau G, Safdar Z, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009;119(22):2894–903.PubMedCrossRefGoogle Scholar
  150. 150.
    Tapson VF, Gomberg-Maitland M, McLaughlin VV, Benza RL, Widlitz AC, Krichman A, et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest J. 2006;129(3):683–8.CrossRefGoogle Scholar
  151. 151.
    Galiè N, Corris PA, Frost A, Girgis RE, Granton J, Jing ZC, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D60–72.PubMedCrossRefGoogle Scholar
  152. 152.
    Baldi F, Fuso L, Arrighi E, Valente S. Optimal management of pulmonary arterial hypertension: prognostic indicators to determine treatment course. Ther Clin Risk Manag. 2014;10:825–39.PubMedPubMedCentralGoogle Scholar
  153. 153.
    Launay D, Sitbon O, Hachulla E, Mouthon L, Gressin V, Rottat L, et al. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Ann Rheum Dis. 2013;72(12):1940–6.  https://doi.org/10.1136/annrheumdis-2012-202489.CrossRefPubMedPubMedCentralGoogle Scholar
  154. 154.
    Miyamoto S, Nagaya N, Satoh T, Kyotani S, Sakamaki F, Fujita M, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension: comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2000;161(2):487–92.PubMedCrossRefPubMedCentralGoogle Scholar
  155. 155.
    Lordan JL, Corris PA. Pulmonary arterial hypertension and lung transplantation. Expert Rev Respir Med. 2011;5(3):441–54.PubMedCrossRefPubMedCentralGoogle Scholar
  156. 156.
    Woods PR, Taylor BJ, Frantz RP, Johnson BD. A pulmonary hypertension gas exchange severity (PH-GXS) score to assist with the assessment and monitoring of pulmonary arterial hypertension. Am J Cardiol. 2012;109(7):1066–72.PubMedPubMedCentralCrossRefGoogle Scholar
  157. 157.
    Chen H, Shiboski SC, Golden JA, Gould MK, Hays SR, Hoopes CW, et al. Impact of the lung allocation score on lung transplantation for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2009;180(5):468–74.PubMedPubMedCentralCrossRefGoogle Scholar
  158. 158.
    Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest J. 2012;142(2):448–56.CrossRefGoogle Scholar
  159. 159.
    Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest J. 2010;137(2):376–87.CrossRefGoogle Scholar
  160. 160.
    Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, 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(2):164–72.PubMedCrossRefGoogle Scholar
  161. 161.
    Benza RL, Gomberg-Maitland M, Miller DP, Frost A, Frantz RP, Foreman AJ, et al. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest J. 2012;141(2):354–62.CrossRefGoogle Scholar
  162. 162.
    Valapour M, Skeans MA, Heubner BM, Smith JM, Hertz MI, Edwards LB, et al. OPTN/SRTR 2013 annual data report: lung. Am J Transplant. 2015;15(S2):1–28.PubMedCrossRefGoogle Scholar
  163. 163.
    Orens JB, Estenne M, Arcasoy S, Conte JV, Corris P, Egan JJ, et al. 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(7):745–55.PubMedCrossRefPubMedCentralGoogle Scholar
  164. 164.
    Kreider M, Highland K. Pulmonary involvement in Sjögren syndrome. Semin Respir Crit Care Med. 2014;35(2):255–64.PubMedCrossRefPubMedCentralGoogle Scholar
  165. 165.
    Kreider M, Hadjiliadis D, Kotloff RM. Candidate selection, timing of listing, and choice of procedure for lung transplantation. Clin Chest Med. 2011;32(2):199–211.PubMedCrossRefPubMedCentralGoogle Scholar
  166. 166.
    Gottlieb J. Lung transplantation for interstitial lung diseases and pulmonary hypertension. Semin Respir Crit Care Med. 2013;34(3):281–7.PubMedCrossRefPubMedCentralGoogle Scholar
  167. 167.
    Christie JD, Edwards LB, Kucheryavaya AY, Benden C, Dipchand AI, Dobbels F, et al. The Registry of the International Society for Heart and Lung Transplantation: 29th adult lung and heart-lung transplant report—2012. J Heart Lung Transplant. 2012;31(10):1073–86.PubMedCrossRefPubMedCentralGoogle Scholar
  168. 168.
    Toyoda Y, Thacker J, Santos R, Nguyen D, Bhama J, Bermudez C, et al. Long-term outcome of lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension. Ann Thorac Surg. 2008;86(4):1116–22.PubMedCrossRefPubMedCentralGoogle Scholar
  169. 169.
    Fadel E, Mercier O, Mussot S, Leroy-Ladurie F, Cerrina J, Chapelier A, et al. Long-term outcome of double-lung and heart–lung transplantation for pulmonary hypertension: a comparative retrospective study of 219 patients. Eur J Cardiothorac Surg. 2010;38(3):277–84.PubMedCrossRefPubMedCentralGoogle Scholar
  170. 170.
    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):100–5.PubMedPubMedCentralGoogle Scholar
  171. 171.
    Charman SC, Sharples LD, McNeil KD, Wallwork J. Assessment of survival benefit after lung transplantation by patient diagnosis. J Heart Lung Transplant. 2002;21(2):226–32.PubMedCrossRefPubMedCentralGoogle Scholar
  172. 172.
    D’Armini AM, Cattadori B, Monterosso C, Klersy C, Emmi V, Piovella F, et al. Pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension: hemodynamic characteristics and changes. Eur J Cardiothorac Surg. 2000;18(6):696–702.PubMedCrossRefPubMedCentralGoogle Scholar
  173. 173.
    McNeil K, Dunning J. Chronic thromboembolic pulmonary hypertension (CTEPH). Heart. 2007;93(9):1152–8.PubMedPubMedCentralCrossRefGoogle Scholar
  174. 174.
    McMahan ZH, Hummers LK. Systemic sclerosis—challenges for clinical practice. Nat Rev Rheumatol. 2013;9(2):90–100.PubMedCrossRefPubMedCentralGoogle Scholar
  175. 175.
    Savarino E, Bazzica M, Zentilin P, Pohl D, Parodi A, Cittadini G, et al. Gastroesophageal reflux and pulmonary fibrosis in scleroderma: a study using pH-impedance monitoring. Am J Respir Crit Care Med. 2009;179(5):408–13.PubMedCrossRefPubMedCentralGoogle Scholar
  176. 176.
    Shitrit D, Amital A, Peled N, Raviv Y, Medalion B, Saute M, et al. Lung transplantation in patients with scleroderma: case series, review of the literature, and criteria for transplantation. Clin Transplant. 2009;23(2):178–83.PubMedCrossRefPubMedCentralGoogle Scholar
  177. 177.
    Schachna L, Medsger TA, Dauber JH, Wigley FM, Braunstein NA, White B, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2006;54(12):3954–61.PubMedCrossRefPubMedCentralGoogle Scholar
  178. 178.
    Mertens V, Blondeau K, Van Oudenhove L, Vanaudenaerde B, Vos R, Farre R, et al. Bile acids aspiration reduces survival in lung transplant recipients with BOS despite azithromycin. Am J Transplant. 2011;11(2):329–35.PubMedCrossRefPubMedCentralGoogle Scholar
  179. 179.
    Robertson AGN, Ward C, Pearson JP, Corris PA, Dark JH, Griffin SM. Lung transplantation, gastroesophageal reflux, and fundoplication. Ann Thorac Surg. 2010;89(2):653–60.PubMedCrossRefPubMedCentralGoogle Scholar
  180. 180.
    D’Ovidio F, Mura M, Tsang M, Waddell TK, Hutcheon MA, Singer LG, et al. Bile acid aspiration and the development of bronchiolitis obliterans after lung transplantation. J Thorac Cardiovasc Surg. 2005;129(5):1144–52.PubMedCrossRefPubMedCentralGoogle Scholar
  181. 181.
    D’Ovidio F, Singer LG, Hadjiliadis D, Pierre A, Waddell TK, de Perrot M, et al. Prevalence of gastroesophageal reflux in end-stage lung disease candidates for lung transplant. Ann Thorac Surg. 2005;80(4):1254–60.PubMedCrossRefPubMedCentralGoogle Scholar
  182. 182.
    Saggar R, Khanna D, Furst DE, Belperio JA, Park GS, Weigt SS, et al. Systemic sclerosis and bilateral lung transplantation: a single centre experience. Eur Respir J. 2010;36(4):893–900.PubMedPubMedCentralCrossRefGoogle Scholar
  183. 183.
    Sottile PD, Iturbe D, Katsumoto TR, Connolly MK, Collard HR, Leard LA, et al. Outcomes in systemic sclerosis-related lung disease following lung transplantation. Transplantation. 2013;95(7):975.PubMedPubMedCentralCrossRefGoogle Scholar
  184. 184.
    Diamond JM, Lee JC, Kawut SM, Shah RJ, Localio AR, Bellamy SL, et al. Clinical risk factors for primary graft dysfunction after lung transplantation. Am J Respir Crit Care Med. 2013;187(5):527–34.PubMedPubMedCentralCrossRefGoogle Scholar
  185. 185.
    Kawut SM. Lung retransplantation. Clin Chest Med. 2011;32(2):367–77.PubMedPubMedCentralCrossRefGoogle Scholar
  186. 186.
    Novick RJ, Stitt LW, Al-Kattan K, Klepetko W, Schäfers H-J, Duchatelle J-P, et al. Pulmonary retransplantation: predictors of graft function and survival in 230 patients. Ann Thorac Surg. 1998;65(1):227–34.PubMedCrossRefPubMedCentralGoogle Scholar
  187. 187.
    Brugiere O, Thabut G, Castier Y, Mal H, Dauriat G, Marceau A, et al. Lung retransplantation for bronchiolitis obliterans syndrome: long-term follow-up in a series of 15 recipients. Chest J. 2003;123(6):1832–7.CrossRefGoogle Scholar
  188. 188.
    Strueber M, Fischer S, Gottlieb J, Simon AR, Goerler H, Gohrbandt B, et al. Long-term outcome after pulmonary retransplantation. J Thorac Cardiovasc Surg. 2006;132(2):407–12.PubMedCrossRefPubMedCentralGoogle Scholar

Copyright information

© The Author(s) 2018

Authors and Affiliations

  1. 1.Department of Respiratory MedicineFreeman HospitalNewcastle Upon TyneUK
  2. 2.Department of Respiratory MedicineFreeman Hospital and Institute of Cellular Medicine, Newcastle UniversityNewcastle Upon TyneUK

Personalised recommendations