ABSTRACT

Pulmonary arterial hypertension (PAH) is a progressive and fatal disease for which there is no cure. The current pharmacological treatment has improved the quality of life (QOL) and possibly the survival (SV) of PAH patients. However, not all patients respond and there is no worldwide availability for this treatment. For patients who are refractory to pharmacological treatment there are two more options available: atrial septostomy (AS) and lung transplantation (LT).
Atrial septostomy is an interventional palliative procedure that improves cardiac output (CO) and decompresses right heart chambers through the creation of an Interatrial shunt. It causes a drop in arterial oxygen saturation which is compensated by the increase in CO. Although there are no controlled studies, the procedure has shown to improve QOL and possibly the SV of PAH patients with right ventricular failure. AS has also been used as a bridge to transplantation. AS should be only performed in specialized centers.
At present, LT represent the last option for treatment of PAH. Based on the relative success of medical treatment, indications and optimal timing for the procedure are now more complicated decisions. Long term SV after LT is limited mainly by chronic rejection, however, in those patients who survive the improvement in QOL could be extraordinary and superior to any other medical intervention.

KEYWORDS

Pulmonary arterial hypertension, atrial septostomy, lung transplantation.

REFERENCES

1. Badesch DB, Abman SH, Ahearn GS, Barst RJ, McCrory DC, Simonneau G, McLaughlin VV. Medical therapy for pulmonary arterial hypertension. ACCP Evidence-Based Clinical Practice Guidelines. Chest 2004; 126: 35S-62S.

2. Galie N, Seeger W, Naeije R, et al. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43: 81S-88S.

3. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106: 1477-1482.

4. D’Alonso GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results of a national prospective study. Ann Intern Med 1991; 115: 343-9.

5. Sandoval J, Gaspar J. Atrial Septostomy. In: Pulmonary Circulation 2nd Edition. Peacock AJ, Rubin LJ (Eds). Edward Arnold Publishers Ltd. London UK, 2004: 319-333.

6. Torbicki A, Kurzyna M, Kuca P, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation 2003; 108: 844-848.

7. Austen WG, Morrow AG, Berry WB. Experimental studies of the surgical treatment of primary pulmonary hypertension. J Thorac Cardiovasc Surg 1964; 48: 448-55.

8. Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries. JAMA 1966; 196: 173.

9. Park SC, Neches WH, Zuberbuhler JR, et al. Clinical Use of Blade Atrial Septostomy. Circulation 1978; 58: 600.

10. Park SC, Neches WH, Mullins CE, et al. Blade Atrial Septostomy: Collaborative Study. Circulation 1982; 66: 258

11. Rich S, Lam W. Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension. Am J Cardiol 1983; 51: 1560-61.

12. Rozkovec A, Montanes P, Oakley CM. Factors that influence the outcome of primary pulmonary hypertension. Br Heart J 1986; 55: 449-58.

13. Hopkins WE, Ochoa LL, Richardson GW, et al. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996; 15: 100-5.

14. Hopkins WE. The remarkable right ventricle of patients with Eisenmenger syndrome. Coronary Artery Disease 2005; 16: 19-25.

15. Nihill MR, O’Laughlin MP, Mullins CE. Effects of atrial septostomy in patients with terminal cor pulmonale due to pulmonary vascular disease. Cathet Cardiovasc Diagn 1991; 24: 166-72.

16. Kerstein D, Levy PS, Hsu DT, et al. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 1995; 91: 2028-35.

17. Rich S, Dodin E, McLaughlin VV. Usefulness of atrial septostomy as a treatment for primary pulmonary hypertension and guidelines for its application. Am J Cardiol 1997; 80: 369-71.

18. Hausknecht MJ, Sims RE, Nihill MR, et al. Successful palliation of primary pulmonary hypertension by atrial septostomy. Am J Cardiol 1990; 65: 1045-46.

19. Rothman A, Beltran D, Kriett JM, et al. Graded balloon dilation atrial septostomy as a bridge to transplantation in primary pulmonary hypertension. Am Heart J 1993; 125: 1763-66.

20. Sandoval J, Gaspar J, Pulido T, et al. Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients non-responsive to vasodilator treatment. J Am Coll Cardiol 1998; 32: 297-304.

21. Rothman A, Slansky MS, Lucas VW, et al. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol 1999; 84: 682-686.

22. Reichenberger F, Pepke-Zaba J, McNeil K, et al. Atrial septostomy in the treatment of severe pulmonary arterial hypertension. Thorax 2003; 58: 797-800.

23. Vachiery JL, Stoupel E, Boonstra A, Naeije R. Balloon atrial septostomy for pulmonary hypertension in the prostacyclin era. Am J Respir Crit Care Med 2003; 167: A692.

24. Moscussi M, Dairywala IT, Chetcuti S, et al. Balloon atrial septostomy in end-stage pulmonary hypertension guided a novel intracardiac echocardiographic transducer. Catheter Cardiovasc Interv 2001; 52: 530-4.

25. Kothari SS, Yusuf A, Juneja R, et al. Graded balloon atrial septostomy in severe pulmonary hypertension. Indian Heart J 2002; 54: 164-9

26. Allcock RJ, O’Sullivan JJ, Corris PA. Atrial septostomy for pulmonary hypertension. Heart 2003; 89: 1344-1347.

27. Kurzyna M, Dabrowsky M, Torbicki A, et al. Atrial septostomy for severe primary pulmonary hypertension. Report of two cases. Kardiol Pol 2003; 58: 27-33.

28. Chau EMC, Fan KYY, Chow WH. Combined atrial septostomy and oral sildenafil for severe right ventricular failure due to primary pulmonary hypertension. Hong Kong Med J 2004; 10: 281-4.

29. Micheletti A, Hislop A, Lammers A, et al. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension. Heart published online 8 Nov 2005. doi: 10.1136/hrt.2005.077669.

30. Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med 2001; 22: 547-560.

31. Barst RJ. Role of atrial septostomy in the treatment of pulmonary vascular disease. Thorax 2000; 55: 95-96.

32. 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; 126: 63S-71S.

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

34. Rubin LJ. Pulmonary Arterial Hypertension. Proc Am Thorac Soc 2006; 3: 111-115.

35. Hayden AM. Balloon atrial septostomy increases cardiac index and may reduce mortality among pulmonary hypertension patients awaiting lung transplantation. J Transpl Coord 1997; 7: 131-133.

36. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99: 1858-1865.

37. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of Treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 2002; 165: 800-804.

38. Bristow MR, Zisman LS, Lances BD, et al. The pressure-overloaded right ventricle in pulmonary hypertension. Chest 1998; 114 (Suppl): 101S-106S.

39. Rosas M, Sandoval J, Granados N, Attie F, Pulido T, Miranda T. Effect of sodium Treprostinil on circadian regulation of heart rate variability in primary pulmonary hypertension. J Am Coll Cardiol

40. Ciarka A, Vachiery JL, Stoupel E, Velez-Roa S, Degaute JP, Naeije R, van de Borne P. Atrial septostomy and muscle sympathetic nerve activity in pulmonary arterial hypertension. Kardiol Pol 2006; 64: 5 (Suppl 1). P B29.

41. Espínola-Zavaleta N, Vargas-Barrón J, Tazar JI, et al. Echocardiographic evaluation of patients with pulmonary hypertension before and after atrial septostomy. Echocardiography 1999; 16: 625.

42. Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Herve P, et al. Long term intravenous epoprostenol infusion in primary pulmonary hypertension. Prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780-88.

43. BandoK, Armitage JM, Paradis IL, Keenan RJ, Hardesty RL, Konishi H, et al. Indications for and results of single, bilateral and heart-lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1994; 108: 1056-65.

44. Pasque MK, Trulock EP, Cooper JD, Triantafillou AN, Huddleston CB, Rosenbloom M, et al. Single lung transplantation for pulmonary hypertension: single institution experience in 34 patients. Circulation 1995; 92: 2252-8.

45. 2001 Annual Report of the U.S. Organ Procurment and Transplantation Network and the Scientific Registry of Transplant Recipients: Transplant Data 1991-2000: 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.

46. Hertz MI, Taylor DO, Trulock EP, Boucek MM, Mohacsi PJ, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: Nineteenth Official Report 2002. J Heart Lung Transplant 2002; 21: 950-70.

47. 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.

48. 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. Am J Respir Crit Care Med 2000; 161: 487-92.

49. Wensel R, Opitz CF, Anker SD, Winkler J, Höffken G, Kleber FX, et al. Assessment of survival in patients with primary pulmonary hypertension. Importance of cardiopulmonary exercise testing. Circulation 2002; 106: 319-24.

50. Gammie JS, Keenan RJ, Pham SM, McGrath MF, Hattler BG, Khosbin E, et al. Single versus double lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1998; 115: 397-403.

51. Mendeloff EN, Meyers BF, Sundt TM, Guthrie TJ, Sweet SC, de la Morena M, et al. Lung transplantation for pulmonary vascular disease. Ann Thorac Surg 2002; 73: 209-19.

52. 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.

53. Franke U, Wiebe K, Harringer W, Franke T, Wittwer T, Wahlers T, 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.

54. 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.

55. Pereszlenyi A, Lang G, Steltzer H, Hetz H, Kocher A, Neuhauser P, Wisser W, Klepetko W. Bilateral lung transplantation with intra- and postoperatively prolonged ECMO support in patients with pulmonary hypertension. Eur J Cardiothorac Surg 2002; 21(5): 858-63.