Risk Stratification in Patients with Pulmonary Arterial Hypertension and Candidates for Lung or Heart-Lung Transplantation

Abstract

<h3>Purpose</h3> During the 2018 6^th PH World Symposium, it was stated that risk stratification scores may be used for the decision of lung (LT) or heart-lung transplantation (HLT) listing in patients with pulmonary arterial hypertension (PAH). The objective of our study was to evaluate risk assessment tools in a cohort of PAH patients listed for LT or HLT. <h3>Methods</h3> All PAH patients listed for LT or HLT between 01/2006 and 12/2018 were included excepted patients with pulmonary veno-occlusive disease or congenital heart diseases because of the specific indications for transplantation in these subtypes of PAH. At the time of listing, risk stratification was assessed according to the ESC-ERS criteria (invasive French and COMPERA methods) and the REVEAL Lite score. The primary endpoint was overall survival after listing. Secondary outcome measures were mortality on waiting list, post-transplant survival, and cumulative incidence of chronic graft dysfunction. <h3>Results</h3> One hundred and six PAH patients were listed for LT or HLT between 01/2006 and 12/2018. The mean age was 37±12 years (73% females). Proportion of idiopathic/heritable PAH was 88%. Overall survival after listing was 72%, 62% and 58% at 1, 3 and 5 years respectively. It was significantly lower in "high risk" patients according to the French method (p=0.0004), the COMPERA method (p=0.005), and the REVEAL Lite score (p=0.01). The mortality rate on list was higher in the "high risk" patients (from 17% with the French method to 19% with the COMPERA method) and urgent transplantation was more frequently performed. Post-transplant survival was 81%, 71% and 66% at 1, 3 and 5 years respectively. It was significantly lower in "high risk" patients without difference in cumulative incidence of chronic graft dysfunction. <h3>Conclusion</h3> PAH patients classified as "high risk" at the time of listing for LT or HLT have poorer outcomes. Time for listing should be anticipated and "high risk" patients should be prioritized to reduce waiting list mortality and improve post-transplant outcomes.