Transplantation for Idiopathic Pulmonary Arterial Hypertension

Abstract

Background— Lung transplantation and heart-lung transplantation represent surgical options for treatment of medically refractory idiopathic pulmonary arterial hypertension. The effect of the lung allocation score on wait-list and transplantation outcomes in patients with idiopathic pulmonary arterial hypertension is poorly described. Methods and Results— Adults diagnosed with idiopathic pulmonary arterial hypertension and listed for transplantation in the 80 months before and after the lung allocation score algorithm was implemented (n=1430) were identified in the United Network for Organ Sharing thoracic registry. Patients were stratified by organ listed and pre– and post–lung allocation score era. The cumulative incidences of transplantation and mortality for wait-listed patients in both eras were appraised with competing outcomes analysis. Posttransplantation survival was assessed with the Kaplan-Meier method. These analyses were repeated in propensity-matched subgroups. Cox proportional hazards analysis evaluated the effect of prelisting and pretransplantation characteristics on mortality. We found that patients in the post–lung allocation score era had significantly worse comorbidities; nevertheless, both lung transplantation and heart-lung transplantation candidates in this era enjoyed lower wait-list mortality and a higher incidence of transplantation in unmatched and propensity-matched analyses. On multivariable analysis, heart-lung transplantation and double-lung transplantation were associated with improved survival from the time of wait-listing, as was being listed at a medium- to high-volume institution. Donor/recipient sex matching predicted posttransplantation survival. Conclusions— The incidence of transplantation has increased while wait-list mortality has decreased in patients with idiopathic pulmonary arterial hypertension wait-listed for transplantation in the post–lung allocation score era. Both heart-lung transplantation and double-lung transplantation are predictive of survival in transplantation candidates with idiopathic pulmonary arterial hypertension, as is being listed at a medium- to high-volume institution. Donor/recipient sex matching is associated with better posttransplantation survival.

Pulmonary hypertension and its management in patients undergoing non‐cardiac surgery

“…Riociguat is a new drug that stimulates soluble guanylate cyclase, leading to an increase in cyclic GMP, and has proven to be efficacious in PAH and CTEPH patients [36]. The incidence of transplantation has increased and both heart-lung transplantation and double-lung transplantation improve survival in transplantation candidates with idiopathic PAH [37].…”

Advanced pulmonary arterial hypertension: mechanical support and lung transplantation

“…In the USA and Germany, the introduction of the LAS has shortened the time to transplantation and decreased the risk of waiting list mortality for many diseases [23,24], including IPAH [25,26]. Despite this observation, the LAS may not fully reflect disease severity in patients with IPAH [14,27].…”

A Comprehensive Review of Current Treatments and Therapies for Idiopathic Pulmonary Arterial Hypertension

“…Most deaths after transplantation occur within the first year. 24 Consideration of transplant is recommended early in disease progression in order to be evaluated for the transplant list and allow adequate survival time until a transplant becomes available. 12 Limitations of transplantation include the possibility of rejection, limited number of organs that become available, and also finding the perfect match.…”