Does Etiology of Pulmonary Hypertension Impact Survival? An ISHLT Registry Analysis

Abstract

Purpose Various etiologies of pulmonary hypertension (PH) and their comorbidities may impact post-lung transplantation (LTx) outcomes. We hypothesized that primary PH, scleroderma PH, and other secondary PH differ in post-tx survival. Methods ISHLT registry was investigated (05/06/2005 -12/31/2016) for bilateral LTx patients > 18 yo excluding multi-organ tx, redo tx, or lobar tx. Primary diagnosis for tx divided as follows: Primary PH (PPH), other Secondary PH (SPH), scleroderma PH (sclPH), and non PH. Age and gender PPH against all 3 groups p Results Kaplan Meier survival (days, mean) PPH (n=937) 2677 (CI 2530, 2824); SPH (n=173) 1953 (CI 1695, 2212); non PH (n=27449) 2664.0 (CI 2633, 2694); SclPH (n=221) 2448.4 (CI 2175, 2721). Log rank tests show significant difference between PPH and SPH (11.8, p=.001), SPH and non PH (18.0, p Wilcoxon tests show statistical significant difference between PPH and SPH (5.1, p=.024) PPH and non PH (9.7, p=.002), SPH and non PH (16.9, p Conclusion PPH survival has worse early survival but better long-term survival. The etiology of scleroderma did not impact survival compared to non-PH. Survival after secondary PH and the outcomes observed may impact risk stratification in certain centers, especially in the setting of challenging regulatory realities.