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Lung Transplantation and Atrial Septostomy for Pulmonary Arterial Hypertension

  • Chapter
Pulmonary Hypertension

Part of the book series: Contemporary Cardiology™ ((CONCARD))

Abstract

Improvements in medical therapy for PAH patients have led to dramatic changes in the way PAH is managed, with much less emphasis on surgical techniques than in the past. Nevertheless, the current drug regimens are still ineffective or lose effectiveness in many patients, related to the progression of the underlying disease. For these patients, lung transplantation and atrial septostomy offer the hope of improved function, prolonged survival, and, most important to some patients, enhanced quality of life. However, both procedures are risky, with the potential for substantial morbidity and mortality, and patients must be selected carefully. Most lung transplant recipients now undergo double-lung transplants and experience higher perioperative mortality rates than those undergoing transplants for other diagnoses. Infection and acute rejection are early causes of morbidity, and most patients later suffer from bronchiolitis obliterans, thought to be related to chronic rejection. This is the main reason for a survival rate that approaches only 50% at five years’ posttransplant. Septostomies are reserved for patients with high-rate atrial pressures who are suffering from low cardiac output syndromes or syncope. They have been associated with mortality rates of 16% in the past, but more recent studies using graded balloon septostomy report rates approaching 5%.

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References

  1. Rubin LJ. Primary pulmonary hypertension. N Engl J Med 1997; 336: 111–7.

    Article  PubMed  CAS  Google Scholar 

  2. Barst RJ. Role of atrial septostomy in the treatment of pulmonary vascular disease. Thorax 2000; 55:95–6.

    Article  PubMed  CAS  Google Scholar 

  3. Hoeper MM, Galiè N, Simonneau G, Rubin LJ. New treatments for pulmonary arterial hypertension. Am J Respir Crit Care Med 2002; 165:1209–16.

    Article  PubMed  Google Scholar 

  4. Galiè N, Seeger W, Naeije R, Simonneau G, Rubin LJ. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43(Suppl):81S–88S.

    Article  PubMed  Google Scholar 

  5. Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension. ACCP evidence-based guidelines. Chest 2004; 126:35S–62S.

    Article  PubMed  Google Scholar 

  6. Doyle RL, McCrory D, Channick RN, Simonneau G, Conte J. Surgical treatments/interventions for pulmonary arterial hypertension. ACCP evidence-based clinical practice guidelines. Chest 2004; 26:63S–71S.

    Article  Google Scholar 

  7. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension. The impact of epoprostenol therapy. Circulation 2002;106:1477–82.

    Article  PubMed  CAS  Google Scholar 

  8. Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension. Prognostic factors and survival. J Am Coll Cardiol 2002; 40:780–8.

    Article  PubMed  CAS  Google Scholar 

  9. Rich S, Kaufman E, Levy PS. The effect of high doses of calcium channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327:76–81.

    PubMed  CAS  Google Scholar 

  10. International guidelines for the selection of lung transplant candidates. Joint statement of the American Society for Transplant Physicians (ASTP)/American Thoracic Society (ATS)/European Respiratory Society (ERS)/International Society for Heart and Lung Transplantation (ISHLT). Am J Respir Crit Care Med 1998; 158:335–9.

    Google Scholar 

  11. Pasque MK, Trulock EP, Kaiser LR, Cooper JD. Single-lung transplantation for pulmonary hypertension: Three-month hemodynamic follow-up. Circulation 1991; 84:2275–9.

    PubMed  CAS  Google Scholar 

  12. Ritchie M, Waggoner AD, Dávila-Román VG, Barzilai B, Trulock EP, Eisenberg PR. Echocardiographic characterization of the improvement in right ventricular function in patients with severe pulmonary hypertension after single-lung transplantation. J Am Coll Cardiol 1993; 22:1170–4.

    Article  PubMed  CAS  Google Scholar 

  13. Kramer MR, Valantine HA, Marshall SE, Starnes VA, Theodore J. Recovery of the right ventricle after single-lung transplantation in pulmonary hypertension. Am J Cardiol 1994; 73:494–500.

    Article  PubMed  CAS  Google Scholar 

  14. 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; 108:1056–65.

    PubMed  CAS  Google Scholar 

  15. Pasque MK, Trulock EP, Cooper JD, et al. Single lung transplantation for pulmonary hypertension: Single institution experience in 34 patients. Circulation 1995; 92:2252–8.

    PubMed  CAS  Google Scholar 

  16. Gammie JS, Keenan RJ, Pham SM, et al. Single- versus double-lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1998; 115:397–403.

    Article  PubMed  CAS  Google Scholar 

  17. Mendeloff EN, Meyers BF, Sundt TM, et al. Lung transplantation for pulmonary vascular disease. Ann Thorac Surg 2002; 73:209–19.

    Article  PubMed  Google Scholar 

  18. Waddell TK, Bennett L, Kennedy R, Todd TRJ, Keshavjee SH. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002; 21:731–7.

    Article  PubMed  Google Scholar 

  19. Trulock EP, Edwards LB, Taylor DO, Boucek MM, Keck BM, Hertz MI. The Registry of the International Society for Heart and Lung Transplantation: Twenty-first official adult lung and heart-lung transplant report—2004. J Heart Lung Transplant 2004; 23:804–15.

    Article  PubMed  Google Scholar 

  20. Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Am J Respir Crit Care Med 2000; 161:487–92.

    PubMed  CAS  Google Scholar 

  21. Wensel R, Opitz CF, Anker SD, et al. Assessment of survival in patients with primary pulmonary hypertension. Importance of cardiopulmonary exercise testing. Circulation 2002; 106:319–24.

    Article  PubMed  Google Scholar 

  22. Nagaya N, Nishikimi T, Okano Y, et al. Plasma brain natriuretic peptide levels increase in proportion to the extent of right ventricular dysfunction in pulmonary hypertension. J Am Coll Cardiol 1998; 31:202–8.

    Article  PubMed  CAS  Google Scholar 

  23. Nagaya N, Nishikimi T, Uematsu M, et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 2000; 102:865–70.

    PubMed  CAS  Google Scholar 

  24. Registry of the International Society for Heart and Lung Transplantation, http://www.ishlt.org/registries/slides.asp; accessed July 18, 2004.

    Google Scholar 

  25. Reitz BA, Wallwork JL, Hunt SA, et al. Heart-lung transplantation. Successful therapy for patients with pulmonary vascular disease. N Engl J Med 1982; 306:557–64.

    PubMed  CAS  Google Scholar 

  26. 2003 Annual Report of the U.S. Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients: Transplant Data 1993–2002. Department of Health and Human Services, Health Resources and Services Administration, Office of Special Programs, Division of Transplantation, Rockville, MD; United Network for Organ Sharing, Richmond, VA; University Renal Research and Education Association, Ann Arbor, MI.

    Google Scholar 

  27. Whyte RI, Robbins RC, Atlinger J, et al. Heart-lung transplantation for primary pulmonary hypertension. Ann Thorac Surg 1999; 67:937–42.

    Article  PubMed  CAS  Google Scholar 

  28. Ueno T, Smith JA, Snell GI, et al. Bilateral sequential single lung transplantation for pulmonary hypertension and Eisenmenger’s syndrome. Ann Thorac Surg 2000; 69:381–7.

    Article  PubMed  CAS  Google Scholar 

  29. Franke U, Wiebe K, Harringer W, et al. Ten years experience with lung and heart-lung transplantation in primary and secondary pulmonary hypertension. Eur J Cardiothorac Surg 2000; 18:447–52.

    Article  PubMed  CAS  Google Scholar 

  30. Stoica SC, McNeil KD, Perreas K, et al. Heart-lung transplantation for Eisenmenger syndrome: Early and long-term results. Ann Thorac Surg 2001; 72:1887–91.

    Article  PubMed  CAS  Google Scholar 

  31. Levine SM, Gibbons WJ, Bryan CL, et al. Single lung transplantation for primary pulmonary hypertension. Chest 1990; 98:1107–15.

    Article  PubMed  CAS  Google Scholar 

  32. Conte JV, Borja MJ, Patgel CB, Yang SC, Jhaveri RM, Orens JB. Lung transplantation for primary and secondary pulmonary hypertension. Ann Thorac Surg 2001; 72:1673–80.

    Article  PubMed  CAS  Google Scholar 

  33. Dawkins KD, Jamieson SW, Hunt SA, et al. Long-term results, hemodynamics, and complications after combined heart and lung transplantation. Circulation 1985; 71:919–26.

    PubMed  CAS  Google Scholar 

  34. Howard DA, Iademarco E, Trulock EP. The role of cardiopulmonary exercise testing in lung and heart-lung transplantation. Clin Chest Med 1994; 15:405–20.

    PubMed  CAS  Google Scholar 

  35. Parekh PI, Blumenthal JA, Babyak MA, et al. Psychiatric disorder and quality of life in patients awaiting lung transplantation. Chest 2003; 124:1682–8.

    Article  PubMed  Google Scholar 

  36. Ramsey SD, Patrick DL, Lewis S, Albert RK, Raghu G. Improvement in quality of life after lung transplantation: A preliminary study. J Heart Lung Transplant 1995; 14:870–7.

    PubMed  CAS  Google Scholar 

  37. Gross C, Savik K, Bolman RM, Hertz MI. Long-term health status and quality of life outcomes of lung transplant recipients. Chest 1995; 108:1587–93.

    Article  PubMed  CAS  Google Scholar 

  38. TenVergert EM, Essink-Bot M-L, Geertsma A, van Enckevort PJ, de Boer WJ, van der Bij W. The effect of lung transplantation on health-related quality of life: A longitudinal study. Chest 1998; 113:358–64.

    Article  PubMed  CAS  Google Scholar 

  39. Limbos MM, Joyce DP, Chan CKN, Kesten S. Psychological functioning and quality of life in lung transplant candidates and recipients. Chest 2000; 118:408–16.

    Article  PubMed  CAS  Google Scholar 

  40. Lanuza DM, Lefavier C, McCabe M, Farcas GA, Garrity E, Jr. Prospective study of functional status and quality of life before and after lung transplantation. Chest 2000; 118:115–22.

    Article  PubMed  CAS  Google Scholar 

  41. Vermeulen KM, Ouwens J-P, van der Bij W, de Boer WJ, Koëter GH, TenVergert EM. Long-term quality of life in patients surviving at least 55 months after lung transplantation. Gen Hosp Psychiatry 2003; 25:95–102.

    Article  PubMed  Google Scholar 

  42. Ramsey SD, Patrick DL, Albert RK, et al. The cost-effectiveness of lung transplantation: A pilot study. Chest 1995; 108:1594–601.

    Article  PubMed  CAS  Google Scholar 

  43. Gartner SH, Sevick MA, Keenan RJ, Chen GJ. Cost-utility of lung transplantation: A pilot study. J Heart Lung Transplant 1997; 16:1129–34.

    PubMed  CAS  Google Scholar 

  44. Maiwenn J. AI, Koopmanschap MA, van Enckevort PJ, et al. Cost-effectiveness of lung transplantation in the Netherlands. Chest 1998; 113:124–30.

    Article  Google Scholar 

  45. Sharples LD, Taylor GJ, Karnon J, et al. A model for analyzing the cost of the main clinical events after lung transplantation. J Heart Lung Transplant 2001; 20:474–82.

    Article  PubMed  CAS  Google Scholar 

  46. Anyanwu AC, McGuire A, Rogers CA, Murday AJ. An economic evaluation of lung transplantation. J Thorac Cardiovasc Surg 2002; 123:411–20.

    Article  PubMed  Google Scholar 

  47. Christie JD, Carby M, Bag R, et al. Report of the ISHLT Working Group on primary lung graft dysfunction, Part II: Definition. A consensus statement of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2005; 24:1454–9.

    Article  PubMed  Google Scholar 

  48. de Perrot M, Bonser RS, Dark J, et al. Report of the ISHLT Working Group on primary lung graft dysfunction, Part III: Donor-related risk factors and markers. J Heart Lung Transplant 2005; 24:1460–7.

    Article  PubMed  Google Scholar 

  49. Barr ML, Kawut SM, Whelan TP, et al. Report of the ISHLT Working Group on primary lung graft dysfunction, Part IV: Recipient-related risk factors and markers. J Heart Lung Transplant 2005; 24:1468–82.

    Article  PubMed  Google Scholar 

  50. Arcasoy SM, Fisher A, Hachem RR, et al. Report of the ISHLT Working Group on primary lung graft dysfunction, Part V: Predictors and outcomes. J Heart Lung Transplant 2005; 24:1483–8.

    Article  PubMed  Google Scholar 

  51. Shargall Y, Guenther G, Ahya VN, et al. Report of the ISHLT Working Group on primary lung graft dysfunction, Part VI: Treatment. J Heart Lung Transplant 2005; 24:1489–500.

    Article  PubMed  Google Scholar 

  52. Christie JD, Kotloff RM, Pochettino A, et al. Clinical risk factors for primary graft failure following lung transplantation. Chest 2003; 124:1232–41.

    Article  PubMed  Google Scholar 

  53. Boehler A, Kesten S, Weder W, Speich R. Bronchiolitis obliterans after lung transplantation. A review. Chest 1998; 114:1411–26.

    Article  PubMed  CAS  Google Scholar 

  54. Estenne M, Hertz MI. Bronchiolitis obliterans after human lung transplantation. Am J Respir Crit Care Med 2002; 166:440–4.

    Article  PubMed  Google Scholar 

  55. Cooper JD, Billingham M, Egan T, et al. A working formulation for the standardization of nomenclature and for clinical staging of chronic dysfunction in lung allografts. J Heart Lung Transplant 1993; 12:713–6.

    PubMed  CAS  Google Scholar 

  56. Estenne M, Maurer JR, Boehler A, et al. Bronchiolitis obliterans syndrome 2001: An update of the diagnostic criteria. J Heart Lung Transplant 2002; 21:297–310.

    Article  PubMed  Google Scholar 

  57. Sharples LD, McNeil K, Stewart S, Wallwork J. Risk factors for bronchiolitis obliterans: A systematic review of recent publications. J Heart Lung Transplant 2002; 21:271–81.

    Article  PubMed  Google Scholar 

  58. Kroshus TJ, Kshettry VR, Savik K, John R, Hertz MI, Bolman RM III. Risk factors for the development of bronchiolitis obliterans syndrome after lung transplantation. J Thorac Cardiovasc Surg 1997; 114:195–202.

    Article  PubMed  CAS  Google Scholar 

  59. Novick RJ, Stitt LW, Al-Kattan K, et al. Pulmonary retransplantation: Predictors of graft function and survival in 230 patients. Ann Thorac Surg 1998; 65:227–34.

    Article  PubMed  CAS  Google Scholar 

  60. Klepetko W, Mayer E, Sandoval J, et al. Interventional and surgical modalities of treatment for pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43 (Suppl):73S–80S.

    Article  PubMed  Google Scholar 

  61. Kerstein D, Levy PS, Hsu DT, Hordof AJ, Gersony WM, Barst RJ. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 1995; 91:2028–35.

    PubMed  CAS  Google Scholar 

  62. Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med 2001; 22:547–60.

    Article  PubMed  CAS  Google Scholar 

  63. Sandoval J, Pepke Zaba J, Vachiery JL. Atrial septostomy for primary pulmonary hypertension. Task force paper for Third World Symposium on Pulmonary Arterial Hypertension; Venice; June 23–25, 2003.

    Google Scholar 

  64. 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; 115:343–9.

    PubMed  CAS  Google Scholar 

  65. Sandoval J, Barst RJ, Rich S, Rothman A. Atrial septostomy for pulmonary hypertension, World Symposium on Primary Pulmonary Hypertension 1998, Evian, France, 1998. Vol. Executive summary. World Health Organization, 1998.

    Google Scholar 

  66. Rothman A, Sklansky MS, Lucas VW, et al. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol 1999; 84:682–6.

    Article  PubMed  CAS  Google Scholar 

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Trulock, E.P. (2008). Lung Transplantation and Atrial Septostomy for Pulmonary Arterial Hypertension. In: Hill, N.S., Farber, H.W. (eds) Pulmonary Hypertension. Contemporary Cardiology™. Humana Press. https://doi.org/10.1007/978-1-60327-075-5_18

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  • DOI: https://doi.org/10.1007/978-1-60327-075-5_18

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