Lung transplantation for idiopathic pulmonary arterial hypertension: perioperational features

Lung transplantation (LT) for idiopathic pulmonary arterial hypertension (IPAH) now is the only radical treatment of this disease.

Aim: to analyze own experience of performing LT in patients with IPAH.

Materials and methods. 8 adult IPAH patients, who underwent LT between 2014 and october 2018, were included. In 7 of 8 patients undergoing bilateral lung transplantation on intraoperative venoarterial extracorporeal membrane oxygenation (VA ECMO) with prolongation into the postoperative period.

Results. VA ECMO support was prolonged into postoperative period 6 and 7 days respectively in 2 (25,0%) patients and 3 days in 6 (75,0%) patients. Hospital mortality in IPAH patients was 1.

Conclusions. Own experience demonstrates that LT is an effective method of treatment in patients with IPAH. Hospital, 1- and 3-year survival rates for the patient collective were 87.5, 75.0 and 75.0% respectively.

1. Corris P, Degano B. Severe pulmonary arterial hypertension treatment option sand the bridge to transplantation. Eur Respir Rev. 2014; 23: 488–497.

2. McLaughlin VV, Shah SJ, Souza R, Humbert M. Management of pulmonary arterial hypertension. J Am Coll Cardiol. 2015; 65: 1976–1997.

3. Sultan S, Tseng S, Stanziola AA et al. Pulmonary Hypertension: The Role of Lung Transplantation. Heart Fail Cin. 2018; 14: 327–331.

4. Machuca TN, Collaud S, Mercier O et al. Outcomes of intraoperative extracorporeal membrane oxygenation versus cardiopulmonary bypass for lung transplantation. J Thorac Cardiovasc Surg. 2015; 149: 1152–1157.

5. Merlo CA, Weiss ES, Orens JB et al. Impact of U. S. Lung Allocation Score on survival after lung transplantation. J Heart Lung Transplant. 2009; 28: 769–775.

6. Orens JB, Boehler A, de Perrot M et al. A review of lung transplant donor acceptability criteria. J Heart Lung Transplant. 2003; 22: 1183–2000.

7. Stocks J, Quanjer PH. Reference values for residual volume, functional residual capacity and total lung capacity. ATS Workshop on Lung Volume Measurements. Official Statement of The European Respiratory Society. Eur Respir J. 1995; 8: 492–506.

8. Van Raemdonck D, Neyrinck A, Verleden GM et al. Lung donor selection and management. Proc Am Thorac Soc. 2009; 6: 28–38.

9. Oto T, Levvey BJ, Snell G. Potential refinements of the International Society for Heart and Lung Transplantation primary graft dysfunction grading system. J Heart Lung Transplant. 2007; 26: 431–436.

10. McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006; 114: 1417–1431.

11. Humbert M, Sitbon O, Chaouat A et al. Survival in patients with idiopathic, familial and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010; 122: 156–163.

12. Kamler M, Pizanis N, Aleksic I et al. Pulmonary hypertension and lung transplantation. Herz. 2005; 30: 281– 285.

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

14. Christie JD, Edwards LB, Autora P et al. The registry of the International Society for Heart and Lung Transplantation: twenty-sixth official adult lung and heart-lung transplantation report. J Heart Lung Transplant. 2009; 28: 1031–1049.

15. Katz WE, Gasior TA, Quinlan JJ et al. Immediate effects of lung transplantation on right ventricular morphology and function in patients with variable degrees of pulmonary hypertension. J Am Coll Cardiol. 1996; 27: 384– 391.

16. Birsan T, Zuckermann Z, Artermiou O et al. Bilateral lung transplantation for pulmonary hypertension. Transplant Proc. 1997; 29: 2892–2894.

17. Julliard WA, Meyer KC, De Oliveira NC et al. The presence or severity of pulmonary hypertension does not affect for single-lung transplantation. Thorax. 2016; 71: 478–480.

18. Yusen RD, Edwards LB, Kucheryavaya AY et al. The registry of International Society for Heart and Lung Transplantation: thirty-second official adult lung and heart-lung transplantation report – 2015. J Heart Lung Transplant. 2015; 34: 1264–1277.

19. Hill C, Maxwell B, Boulate D et al. Heart-lung vsdouble-lung transplantation for idiopathic pulmonary arterial hypertension. Clin Transplant. 2015; 29: 1067–1075.

20. Fang A, Studer S, Kawut SM et al. Elevated pulmonary artery pressure is a risk factor for primary graft dysfunction following lung transplantation for idiopathic pulmonary fibrosis. Chest. 2011; 139: 782–787.

21. Wisser W, Marta G, Senbaklavica O et al. BLTX with intra- and postoperatively prolonged ECMO in patients with pulmonary hypertension: beneficial effect on initial organ function. J Heart Lung Transplant. 2001; 20: 224–225.

22. Pereszlenyi A, Lang G, Steltzer H et al. Bilateral lung transplantation with intra- and postoperatively prolonged ECMO support in patients with pulmonary hypertension. Eur J Cardiothorac Surg. 2002; 21: 858–863.

23. Mozer B, Jaksch P, Taghavi S et al. Lung transplantation for idiopathic pulmonary arterial hypertension on intraoperative and postoperatively prolonged extracorporeal membrane oxygenation provides optimally controlled reperfusion and excellent outcome. Eur J Cardiothorac Surg. 2018; 53: 178–185.