Atrial Septostomy and Transplantation for Patients with Pulmonary Arterial Hypertension

ABSTRACT

The development of specific disease-targeted therapy for pulmonary arterial hypertension has offered a major advancement in the management of patients, and these medical treatments have been shown to improve both quality and quantity of life. Nevertheless, many patients deteriorate with time and will require consideration of other approaches. This article focuses on the roles of atrial septostomy and lung transplantation.

Pulmonary arterial hypertension - atrial septostomy - right ventricular failure - lung transplantation - selection criteria - outcomes

REFERENCES

• 1 D'Alonzo G E, Barst R J, Ayres S M et al.. Survival in patients with primary pulmonary hypertension: results from a national prospective registry.  Ann Intern Med. 1991;  115 343-349
• 2 Nihill M R, O'Laughlin M P, Mullins C E. Effects of atrial septostomy in patients with terminal cor pulmonale due to pulmonary vascular disease.  Cathet Cardiovasc Diagn. 1991;  24 166-172
• 3 Kerstein D, Levy P S, Hsu D T, Hordof A J, Gersony W M, Barst R J. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension.  Circulation. 1995;  91 2028-2035
• 4 Austen W G, Morrow A G, Berry W B. Experimental studies of the surgical treatment of primary pulmonary hypertension.  J Thorac Cardiovasc Surg. 1964;  48 448-455
• 5 Hopkins W E. The remarkable right ventricle of patients with Eisenmenger syndrome.  Coron Artery Dis. 2005;  16 19-25
• 6 Rich S, Lam W. Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension.  Am J Cardiol. 1983;  51 1560-1561
• 7 Hausknecht M J, Sims R E, Nihill M R, Cashion W R. Successful palliation of primary pulmonary hypertension by atrial septostomy.  Am J Cardiol. 1990;  65 1045-1046
• 8 Bristow M R, Zisman L S, Lowes B D et al.. The pressure-overloaded right ventricle in pulmonary hypertension.  Chest. 1998;  114(1, Suppl) 101S-106S
• 9 Micheletti A, Hislop A A, Lammers A et al.. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension.  Heart. 2006;  92 969-972
• 10 Rosenzweig E B, Kerstein D, Barst R J. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.  Circulation. 1999;  99 1858-1865
• 11 Simonneau G, Barst R J, Galie N Treprostinil Study Group 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;  165 800-804
• 12 Galiè N, Beghetti M, Gatzoulis M A Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study.  Circulation. 2006;  114 48-54
• 13 Sandoval J, Gaspar J. Atrial septostomy. In: Peacock AJ, Rubin LJ Pulmonary Circulation. 2nd ed. London; Edward Arnold 2004: 319-333
• 14 Rich S, Dodin E, McLaughlin V V. Usefulness of atrial septostomy as a treatment for primary pulmonary hypertension and guidelines for its application.  Am J Cardiol. 1997;  80 369-371
• 15 Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension.  Clin Chest Med. 2001;  22 547-560
• 16 Rothman A, Sklansky M S, Lucas V W et al.. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension.  Am J Cardiol. 1999;  84 682-686
• 17 Klepetko W, Mayer E, Sandoval J et al.. Interventional and surgical modalities of treatment for pulmonary arterial hypertension.  J Am Coll Cardiol. 2004;  43(12, Suppl S) 73S-80S
• 18 Reichenberger F, Pepke-Zaba J, McNeil K, Parameshwar J, Shapiro L M. Atrial septostomy in the treatment of severe pulmonary arterial hypertension.  Thorax. 2003;  58 797-800
• 19 Velez-Roa S, Ciarka A, Najem B, Vachiery J L, Naeije R, van de Borne P. Increased sympathetic nerve activity in pulmonary artery hypertension.  Circulation. 2004;  110 1308-1312
• 20 Kurzyna M, Dabrowski M, Bielecki D et al.. Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension.  Chest. 2007;  131 977-983
• 21 Law M A, Grifka R G, Mullins C E, Nihill M R. Atrial septostomy improves survival in select patients with pulmonary hypertension.  Am Heart J. 2007;  153 779-784
• 22 Trulock E P, Edwards L B, Taylor D O, Boucek M M, Keck B M, Hertz M I. Registry of the International Society for Heart and Lung Transplantation: twenty-second official adult lung and heart-lung transplant report—2005.  J Heart Lung Transplant. 2005;  24 956-967
• 23 Charman S C, Sharples L D, McNeil K D, Wallwork J. Assessment of survival benefit after lung transplantation by patient diagnosis.  J Heart Lung Transplant. 2002;  21 226-232
• 24 De Meester J, Smits J M, Persijn G G, Haverich A. Listing for lung transplantation: life expectancy and transplant effect, stratified by type of end-stage lung disease, the Eurotransplant experience.  J Heart Lung Transplant. 2001;  20 518-524
• 25 Thabut G, Mal H, Castier Y et al.. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis.  J Thorac Cardiovasc Surg. 2003;  126 469-475
• 26 Geertsma A, Ten Vergert E M, Bonsel G J, de Boer W J, van der Bij W. Does lung transplantation prolong life? A comparison of survival with and without transplantation.  J Heart Lung Transplant. 1998;  17 511-516
• 27 Hosenpud J D, Bennett L E, Keck B M, Edwards E B, Novick R J. Effect of diagnosis on survival benefit of lung transplantation for end-stage lung disease.  Lancet. 1998;  351 24-27
• 28 Gerbase M W, Spiliopoulos A, Rochat T, Archinard M, Nicod L P. Health-related quality of life following single or bilateral lung transplantation: a 7-year comparison to functional outcome.  Chest. 2005;  128 1371-1378
• 29 Gross C R, Savik K, Bolman III R M, Hertz M I. Long-term health status and quality of life outcomes of lung transplant recipients.  Chest. 1995;  108 1587-1593
• 30 Maish A B. Priorities for lung transplantation among patients with cystic fibrosis.  JAMA. 2002;  287 1524-1525 author reply 1524-1525
• 31 Kanasky Jr W F, Anton S D, Rodrigue J R, Perri M G, Szwed T, Baz M A. Impact of body weight on long-term survival after lung transplantation.  Chest. 2002;  121 401-406
• 32 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;  40 780-788
• 33 McLaughlin V V, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy.  Circulation. 2002;  106 1477-1482
• 34 Orens J B, Estenne M, Arcasoy S Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation 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;  25 745-755
• 35 Bando K, Keenan R J, Paradis I L et al.. Impact of pulmonary hypertension on outcome after single-lung transplantation.  Ann Thorac Surg. 1994;  58 1336-1342
• 36 McCarthy P M, Rosenkranz E R, White R D et al.. Single-lung transplantation with atrial septal defect repair for Eisenmenger's syndrome.  Ann Thorac Surg. 1991;  52 300-303
• 37 Pasque M K, Trulock E P, Kaiser L R, Cooper J D. Single-lung transplantation for pulmonary hypertension: three-month hemodynamic follow-up.  Circulation. 1991;  84 2275-2279
• 38 Pasque M K, Trulock E P, Cooper J D et al.. Single lung transplantation for pulmonary hypertension: single institution experience in 34 patients.  Circulation. 1995;  92 2252-2258
• 39 Starnes V A, Stinson E B, Oyer P E et al.. Single lung transplantation: a new therapeutic option for patients with pulmonary hypertension.  Transplant Proc. 1991;  23(1 Pt 2) 1209-1210
• 40 Ueno T, Smith J A, Snell G I et al.. Bilateral sequential single lung transplantation for pulmonary hypertension and Eisenmenger's syndrome.  Ann Thorac Surg. 2000;  69 381-387
• 41 Birsan T, Zuckermann Z, Artermiou O et al.. Bilateral lung transplantation for pulmonary hypertension.  Transplant Proc. 1997;  29 2892-2894
• 42 Whyte R I, Robbins R C, Altinger J et al.. Heart–lung transplantation for primary pulmonary hypertension.  Ann Thorac Surg. 1999;  67 937-941 discussion 941-942
• 43 Mikhail G, al-Kattan K, Banner N et al.. Long-term results of heart–lung transplantation for pulmonary hypertension.  Transplant Proc. 1997;  29 633
• 44 Stoica S C, McNeil K D, Perreas K et al.. Heart–lung transplantation for Eisenmenger syndrome: early and long-term results.  Ann Thorac Surg. 2001;  72 1887-1891
• 45 Boujoukos A J, Martich G D, Vega J D, Keenan R J, Griffith B P. Reperfusion injury in single lung transplant recipients with pulmonary hypertension and emphysema.  J Heart Lung Transplant. 1997;  16 439-448
• 46 Kramer M R, Marshall S E, McDougall I R et al.. The distribution of ventilation and perfusion after single-lung transplantation in patients with pulmonary fibrosis and pulmonary hypertension.  Transplant Proc. 1991;  23(1 Pt 2) 1215-1216
• 47 Levine S M, Jenkinson S G, Bryan C L et al.. Ventilation-perfusion inequalities during graft rejection in patients undergoing single lung transplantation for primary pulmonary hypertension.  Chest. 1992;  101 401-405
• 48 Huerd S S, Hodges T N, Grover F L et al.. Secondary pulmonary hypertension does not adversely affect outcome after single lung transplantation.  J Thorac Cardiovasc Surg. 2000;  119 458-465
• 49 Kramer M R, Valantine H A, Marshall S E, Starnes V A, Theodore J. Recovery of the right ventricle after single-lung transplantation in pulmonary hypertension.  Am J Cardiol. 1994;  73 494-500
• 50 Schulman L L, Leibowitz D W, Anandarangam T et al.. Variability of right ventricular functional recovery after lung transplantation.  Transplantation. 1996;  62 622-625
• 51 Lupinetti F M, Bolling S F, Bove E L et al.. Selective lung or heart–lung transplantation for pulmonary hypertension associated with congenital cardiac anomalies.  Ann Thorac Surg. 1994;  57 1545-1548, 1549
• 52 Waddell T K, Bennett L W, Kennedy R et al.. Lung or heart lung transplantation for Eisenmenger's syndrome: analysis of the ISHLT/UNOS joint thoracic registry [abstract].  J Heart Lung Transplant. 2000;  19 57
• 53 Chapelier A, Vouhé P, Macchiarini P et al.. Comparative outcome of heart–lung and lung transplantation for pulmonary hypertension.  J Thorac Cardiovasc Surg. 1993;  106 299-307
• 54 Bando K, Armitage J M, Paradis I L et al.. Indications for and results of single, bilateral, and heart–lung transplantation for pulmonary hypertension.  J Thorac Cardiovasc Surg. 1994;  108 1056-1065
• 55 Gammie J S, Keenan R J, Pham S M et al.. Single- versus double-lung transplantation for pulmonary hypertension.  J Thorac Cardiovasc Surg. 1998;  115 397-402 discussion 402-403
• 56 Conte J V, Borja M J, Patel C B, Yang S C, Jhaveri R M, Orens J B. Lung transplantation for primary and secondary pulmonary hypertension.  Ann Thorac Surg. 2001;  72 1673-1679 discussion 1679-1680
• 57 D'Alonzo G E, Barst R J, Ayres S M et al.. Survival in patients with primary pulmonary hypertension: results from a national prospective registry.  Ann Intern Med. 1991;  115 343-349
• 58 Barst R J, Rubin L J, Long W A The Primary Pulmonary Hypertension Study Group et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension.  N Engl J Med. 1996;  334 296-302
• 59 Robbins I M, Christman B W, Newman J H, Matlock R, Loyd J E. A survey of diagnostic practices and the use of epoprostenol in patients with primary pulmonary hypertension.  Chest. 1998;  114 1269-1275
• 60 Conte J V, Gaine S P, Orens J B, Harris T, Rubin L J. The influence of continuous intravenous prostacyclin therapy for primary pulmonary hypertension on the timing and outcome of transplantation.  J Heart Lung Transplant. 1998;  17 679-685
• 61 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.  J Heart Lung Transplant. 2000;  19 909-931
• 62 Pielsticker E J, Martinez F J, Rubenfire M. Lung and heart–lung transplant practice patterns in pulmonary hypertension centers.  J Heart Lung Transplant. 2001;  20 1297-1304
• 63 Rich S, Levy P S. Characteristics of surviving and nonsurviving patients with primary pulmonary hypertension.  Am J Med. 1984;  76 573-578
• 64 Glanville A R, Burke C M, Theodore J, Robin E D. Primary pulmonary hypertension. Length of survival in patients referred for heart–lung transplantation.  Chest. 1987;  91 675-681
• 65 Eysmann S B, Palevsky H I, Reichek N, Hackney K, Douglas P S. Two dimensional echocardiography and cardiac catheterisation correlates of survival in primary pulmonary hypertension.  Circulation. 1989;  80 353-360