Perioperative management of lung transplantations in patients with pulmonary arterial hypertension compared with that in patients with lymphangiomyomatosis

Background : 'The number of lung transplantation has tended to increase as a treatment for patients with pulmonary arterial hypertension (PAH) and lymphangiomyomatosis (LAM) in Japan. However, we have little evidence about the comparison of perioperative management in patients with PAH and that in patients with LAM. Methods : In this retrospective study, ten patients with PAH and seventeen patients with LAM who underwent the lung transplantations between 2006 and 2011 were enrolled. Results : PAH patients received double lung transplantation with intraoperative cardiopulmonary bypass (CPB) support. Before anesthesia induction, percutaneous cardiopulmonary support (PCPS) was begun. Most of LAM patients received single lung transplantation without using CPB support and PCPS support before anesthesia induction. But sometimes during an operation PCPS support was necessary. Postoperative PCPS support showed no significant differences between PAH and LAM. The four year survival rate was 80% (PAH) and 87.8% (LAM). Pa_O2/F_IO2 in patients with PAH and in those with LAM, %FEV_1.0 in those with LAM after surgery improved when compared to those before surgery. Conclusions : The large difference in perioperative management between patients with PAH and those with LAM is an important knowledge for anesthesist.