Korean J Transplant.  2021 Oct;35(Supple 1):S13. 10.4285/ATW2021.PO-1069.

Lung transplantation in six patients with idiopathic pulmonary artery hypertension

• Kim H
• Kim D
• Choi S
• Lee G
• Oh D
• Lee J
• Jung S
• Park S
• Hong S

• KMID: 2521185
• DOI: http://doi.org/10.4285/ATW2021.PO-1069

Abstract

Background
Idiopathic pulmonary artery hypertension (IPAH) is an incurable and invariably fatal disease. Lung transplantation is a useful therapeutic option in patients who are unresponsive to medical treatment; however, lung transplantation performed for pulmonary hypertension is associated with significantly high perioperative mortality rates.
Methods
We report case series of six patients who underwent lung transplantation for IPAH between October 2008 and June 2021.
Results
Patients’ median age was 28.5 years, and the study included 5 of 6 female (83%). Pre-transplantation hemodynamic parameters showed mean right atrial pressure of 12.0±7.1 mmHg and mean pulmonary artery pressure of 62.2±29.5 mmHg. Two of six patients received extracorporeal membrane oxygenation (ECMO) therapy as a bridge to transplantation over 14 and 17 days, and four patients underwent elective transplantation. Two patients required prolonged postoperative venoarterial (VA) ECMO support. Grade 3 primary graft dysfunction occurred in one patient; however, the clinical course improved following prolonged VA ECMO therapy. Five patients (83.3%) required intervention for postoperative bleeding control; one of these patients died of uncontrolled bleeding concomitant with disseminated intravascular coagulation, on the 14th postoperative day, and we observed no other perioperative deaths. One patient died of carbapenem-resistant Acinetobacter baumannii bacteremia, a year postoperatively. The 1-month, 6-month, and 1-year survival rates were 83.3%, 83.3%, and 66.7%, respectively.
Conclusions
In view of the poor prognosis of IPAH, lung transplantation (1-year mortality rates <40%) merits consideration as a useful therapeutic option in this patient population. However, postoperative bleeding tends to occur in most patients; therefore, close monitoring is important during post-transplantation management.