Navigating the Road to Transplant in Pulmonary Arterial Hypertension: A Road Less Taken

Advances in Pulmonary Hypertension

Frost, A¹ 

DOI  :  

学科分类：医学（综合）

来源: Pulmonary Hypertension Association

【 摘 要 】

The lung allocation score (LAS) was developed and implemented in May 2005.1 Prior to that, lungs for transplant were allocated largely based on time on the waiting list; such a system is an obvious disadvantage for patients with rapidly progressive or unpredictably progressive disease. Parameters used to assess disease severity (risk of death without a transplant) were tailored to the majority of patients awaiting lung transplantation, individuals who had some form of parenchymal lung disease (idiopathic pulmonary fibrosis [IPF] [United Network for Organ Sharing (UNOS) Group D], cystic fibrosis [Group C], chronic obstructive pulmonary disease [COPD] [Group A]). It was soon recognized that the success of the LAS in optimizing utilization of organs for those who would most benefit excluded patients with pulmonary arterial hypertension (PAH). Analysis of patients in REVEAL (the Registry to Evaluate Early and Long-term PAH Disease Management) identified risk factors predictive of mortality in patients with PAH (functional class III/IV, impaired renal function, elevated b-type natriuretic peptide, reduced 6-minute walk distance [6MWD], elevated right atrial pressure [RAP], presence of a pericardial effusion, reduction in diffusion lung capacity for carbon monoxide). Unfortunately, the LAS did not reflect these risks for mortality. Another analysis from REVEAL in 2010 (5 years after introduction of the initial LAS) concluded that the LAS overestimated survival for patients who met criteria for listing for lung transplantation.2 The observed 1-year mortality exceeded that predicted by LAS in 2 subgroups of patients: those with mean RAP =14 mm Hg (14.8%±1.9% vs 12.2%) and those with a 6MWD =300 m (17.3%±1.7% vs 14.8%).

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