Abstract

Systemic Sclerosis (SSc) is an autoimmune connective tissue disease that can involve the heart and lung. Cardiac abnormality can stem from pulmonary hypertension or primary cardiac disease. While lung transplantation (LTx) has been effective for patients (pts) with pulmonary involvement, it is unknown how outcomes of heart-lung transplantation (HLTx) compare to LTx. The aim of this study is to evaluate HLTx utilizing a US national database. HLTx pts from 2000-2014 reported by the UNOS database were assessed and compared to both LTx pts with SSc and HLTx pts for all other indications (HLTx-OI). The primary outcome was all cause mortality. 1 and 4-year survival was determined using the Kaplan Meier method. Risk factors for survival (adjusted for recipient age/gender, donor age, ischemia time, heart transplant status) were analyzed using multi variable Cox proportional hazard regression. The secondary endpoint was freedom from 1st year rejection. 430 of 21,928 LTx (1.96%) and 8 of 466 HLTx (1.72%) pts had a primary indication of SSc. Demographics (recipient age/gender, donor age, time on the waiting list, CMV mismatch) between the 2 comparison groups were similar. Ischemic time was lower for HLTx-SSc (3.80 hrs) v. LTx-SSc (5.31 hrs) (p = 0.012). Status 1 was more common for HLTx-SSc (1A 25%, 1B 75%) v. HLTx-OI (1A 34%, 1B 20%) (p= 0.011). 1 and 4-year survival was lower but not significantly different between HLTx-SSc v. LTx-SSc as well as compared to HLTx-OI (Table). Freedom from 1-year rejection was not significantly different among the two comparison groups. SSx is a rare indication for HLTx as well as LTx (<2%). Review of national data confirms that, despite being somewhat sicker at the time of transplant as evidenced by a higher rate of Class 1 status, outcomes of HLTx-SSc pts are similar to that for LTx and HLTx-OI with respect to survival and freedom from rejection. HLTx appears to be a viable option for pts with SSc and significant cardiac involvement.

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