Download PDF
Semin Respir Crit Care Med 2001; 22(5): 533-540
DOI: 10.1055/s-2001-18425
Copyright © 2001 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Lung Transplantation for Pulmonary Hypertension

Sean P. Gaine1 , Jonathan B. Orens2
  • 1Mater Private Hospital, Dublin, Ireland
  • 2Division of Pulmonary and Critical Care Medicine, Johns Hopkins Medical Institutions, Baltimore, Maryland
Further Information

Publication History

Publication Date:
14 November 2001 (online)

    Permissions and Reprints

ABSTRACT

Although lung transplantation is a viable option for patients with end-stage pulmonary hypertension, it is associated with numerous problems including infection, rejection, and limited long-term survival. Because of these limitations, transplantation should only be considered for patients who are failing maximal medical therapy. Treatment options for patients with pulmonary hypertension that may serve to prolong or obviate the need for transplantation include anticoagulation with warfarin, diuretics, and vasodilators such as calcium channel blockers or continuous intravenous epoprostenol (prostacyclin). The response to medical therapy should be assessed at regular intervals by evaluating exercise tolerance and hemodynamic parameters. Because waiting periods for transplantation now exceed 1.5 to 2 years in the United States, and the response to medications is unpredictable, referral for transplantation should occur when patients become symptomatic. Those who are responding well to medical therapy should be removed from the active transplant waiting list, whereas those who fail therapy should go on to transplant. Utilizing medical therapy and transplantation as complementary treatments will achieve the best potential to improve quality of life and prolong survival.

KEYWORD

Lung transplantation - prostacyclin - pulmonary hypertension

REFERENCES

  • 1 Reitz B A, Wallwork J L, Hunt S A. Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease.  N Engl J Med . 1982;  306 557-564
    CrossrefPubMedSearch in Google Scholar
  • 2 Rubin, L J, Nicod P, Hillis L D. Treatment of primary pulmonary hypertension with nifedipine.  Ann Intern Med . 1983;  99 433-438
    PubMedSearch in Google Scholar
  • 3 Rich S, Brundage B H. High dose calcium channel blocking therapy for primary pulmonary hypertension: evidence for long-term reduction in pulmonary artery pressure and regression of right ventricular hypertrophy.  Circulation . 1987;  76 135-141
    CrossrefPubMedSearch in Google Scholar
  • 4 Rich S, Kaufmann E, Levy P S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension.  N Engl J Med . 1992;  327(2) 76-81
    CrossrefPubMedSearch in Google Scholar
  • 5 Rubin L J, Mendoza J, Hood M. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol): results of a randomized trial.  Ann Intern Med . 1990;  112 485-491
    CrossrefPubMedSearch in Google Scholar
  • 6 Higenbottam T W, Wells F, Wheeldon D. Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin).  Lancet . 1984;  1 1046-1047
    PubMedSearch in Google Scholar
  • 7 Conte J V, Gaine S P, Orens J B. Continuous intravenous prostacyclin as a bridge to lung transplantation.  J Heart Lung Transplant . 1998;  17 679-685
    PubMedSearch in Google Scholar
  • 8 Rich S. Medical treatment of primary pulmonary hypertension: a bridge to transplantation?.  Am J Cardiol . 1995;  75(3) 63A-66A
    CrossrefPubMedSearch in Google Scholar
  • 9 Gaine S, Rubin L. Medical and surgical treatment options for pulmonary hypertension.  Am J Med Sci . 1998;  315(3) 179-184
    PubMedSearch in Google Scholar
  • 10 Rich S. Executive summary of the World Symposium on PPH. Available at: http:www.who.int/ncd/cvd/pph.html. Accessed December 2000
  • 11 Holcomb B W, Loyd J E, Ely E W. Pulmonary veno-occlusive disease: a case series and new observations.  Chest . 2000;  118(6) 1671-1679
    CrossrefPubMedSearch in Google Scholar
  • 12 Moser K M, Daily P O, Peterson K. Thromboendarterectomy for chronic, major vessel thromboembolic pulmonary hypertension: immediate and long-term results in 42 patients.  Ann Intern Med . 1987;  107 560-565
    PubMedSearch in Google Scholar
  • 13 Rubin L J. Primary pulmonary hypertension.  N Engl J Med . 1997;  336(2) 111-117
    CrossrefPubMedSearch in Google Scholar
  • 14 Hopkins W E, Ochoa L L, Richardson. 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
    PubMedSearch in Google Scholar
  • 15 D'Alonzo G E, Barst R J, Ayres S M. Survival in patients with primary pulmonary hypertension: results from a national prospective registry.  Ann Intern Med . 1991;  115(5) 343-349
    PubMedSearch in Google Scholar
  • 16 Rich S, Levy P S. Characteristics of surviving and nonsurviving patients with primary pulmonary hypertension.  Am J Med . 1984;  76 573-578
    CrossrefPubMedSearch in Google Scholar
  • 17 Rich S, Dantzker D R, Ayres S M. Primary pulmonary hypertension: a national prospective study.  Ann Intern Med . 1987;  107(2) 216-223
    CrossrefPubMedSearch in Google Scholar
  • 18 Kadikar A, Maurer J, Kesten S. The six-minute walk test: a guide to assessment for lung transplantation.  J Heart Lung Transplant . 1997;  16(3) 313-319
    PubMedSearch in Google Scholar
  • 19 Fuster V, Steele P M, Edwards W D. Primary pulmonary hypertension: natural history and the importance of thrombosis.  Circulation . 1984;  70 580-587
    CrossrefPubMedSearch in Google Scholar
  • 20 Barst R J, Rubin L J, McGoon M D. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin.  Ann Intern Med . 1994;  121(6) 409-415
    CrossrefPubMedSearch in Google Scholar
  • 21 Shapiro S M, Oudiz R J, Cao T. Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoerostenol infusion.  JACC . 1997;  30(2) 343-349
    CrossrefPubMedSearch in Google Scholar
  • 22 Gaine S. Pulmonary Hypertension.  JAMA . 2000;  284 3160-3168
    CrossrefPubMedSearch in Google Scholar
  • 23 Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive pulmonary disease: a clinical trial.  Ann Intern Med . 1980;  93 391-398
    PubMedSearch in Google Scholar
  • 24 Barst R J, Rubin L J, Long W A. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group.  N Engl J Med . 1996;  334(5) 296-302
    CrossrefPubMedSearch in Google Scholar
  • 25 Badesch D B, Tapson V F, Mcgoon M D. A comparison of continuous intravenous epoprostenol with conventional therapy for pulmonary hypertension secondary to the scleroderma spectrum of diseases.  Ann Intern Med . 2000;  132(6) 425-434
    PubMedSearch in Google Scholar
  • 26 Kuo P C, Johnson L B, Plotkin J S. Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension.  Transplantation . 1997;  63(4) 604-606
    CrossrefPubMedSearch in Google Scholar
  • 27 Robbins I M, Gaine S P, Schilz R. Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus.  Chest . 2000;  117(1) 14-18
    CrossrefPubMedSearch in Google Scholar
  • 28 McLaughlin V V, Genthner D E, Panella M M. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series.  Ann Intern Med . 1999;  4(130[9]) 740-743
    PubMedSearch in Google Scholar
  • 29 Rosenzweig E, Kerstein D, Barst R. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.  Circulation . 1999;  13(99[14]) 1858-1865
    PubMedSearch in Google Scholar
  • 30 Sandoval J, Gaspar J, Pulido T. Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension: a therapeutic alternative for patients nonresponsive to vasodilator treatment.  J Am Coll Cardiol . 1998;  32(2) 297-304
    CrossrefPubMedSearch in Google Scholar
  • 31 Rich S, Lam W. Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension.  Am J Coll Cardiol . 1983;  51 1560-1561
    CrossrefPubMedSearch in Google Scholar
  • 32 Kerstein D, Levy P S, Hsu D T. Blade balloon atrial septostomy improves survival in patients with severe primary pulmonary hypertension.  Circulation . 1995;  91(7) 2028-2035
    PubMedSearch in Google Scholar
  • 33 Rothman A, Sklansky M S, Lucas V W. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension.  Am J Cardiol . 1999;  15(84(6)) 682-686
    PubMedSearch in Google Scholar
  • 34 Rothman A, Beltran D, Kriett J M. Graded balloon dilation atrial septostomy as a bridge to lung transplantation in pulmonary hypertension [see comments].  Am Heart J . 1993;  125(6) 1763-1766
    CrossrefPubMedSearch in Google Scholar
  • 35 Tarver J H, Rosas I, Diette G B. Chronic ambulatory dopamine infusion therapy for refractory right heart failure due to pulmonary hypertension.  ARRCM . 2000;  161(3) A459
    PubMedSearch in Google Scholar
  • 36 UNOS data. Available at: http://www.unos.org/frame_default.asp. Accessed October 2001
  • 37 Lilienfeld D, Rubin L. Mortality from primary pulmonary hypertension in the United States, 1979-1996.  Chest . 2000;  117(3) 796-800
    CrossrefPubMedSearch in Google Scholar
  • 38 Maurer J R, Frost A E, Estenne M. International guidelines for the selection of lung transplant candidates.  J Heart Lung Transplant . 1998;  17(7) 703-709
    PubMedSearch in Google Scholar
  • 39 Whyte R I, Robbins R C, Altinger J. Heart-lung transplantation for primary pulmonary hypertension.  Ann Thorac Surg . 1999;  67(4) 937-942
    CrossrefPubMedSearch in Google Scholar
  • 40 Bando K, Keenan R J, Paradis I L. Impact of pulmonary hypertension on outcome after single-lung transplantation.  Ann Thorac Surg . 1994;  58(5) 1336-1342
    CrossrefPubMedSearch in Google Scholar
  • 41 Bando K, Armitage J M, Paradis I L. Indications for and results of single, bilateral, and heart-lung transplantation for pulmonary hypertension.  J Thorac Cardiovasc Surg . 1994;  108(6) 1056-1065
    PubMedSearch in Google Scholar
  • 42 Pasque M K, Trulock E P, Cooper J D. Single lung transplantation for pulmonary hypertension: single institution experience in 34 patients.  Circulation . 1995;  92 2252-2258
    CrossrefPubMedSearch in Google Scholar
  • 43 Hosenpud J D, Bennett L E, Keck B M, Boucek M M, Novick R J. The Registry of the International Society for Heart and Lung Transplantation: seventeenth official report-2000.  J Heart Lung Transplant . 2000;  19 909-931
    CrossrefPubMedSearch in Google Scholar
  • 44 Levine S MJ, Erikinson S G, Bryan C L. Ventilation-perfusion inequalities during graft rejection in patients undergoing single lung transplantation for primary pulmonary hypertension.  Chest . 1992;  101 401-405
    CrossrefPubMedSearch in Google Scholar
  • 45 1999 Annual Report of the U.S. Scientific Registry for Transplant Recipients and the Organ Procurement and Transplantation Network: Transplant Data: 1989-1998. Rockville, MD: U.S. Department of Health and Human Services, Health Resources and Services Administration, Office of Special Programs, Division of Transplantation; Richmond, VA: UNOS
  • 46 Hosenpud J D, Bennett L E, Keck B M. The Registry of the International Society for Heart and Lung Transplantation: fourteenth official report-1997.  J Heart Lung Transplant . 1997;  16 691-712
    PubMedSearch in Google Scholar