Bilateral Lung Transplant for a Connective Tissue Disorder: Esophageal Motility and 3-year Survival.

Connective tissue disorders (CTDs) are associated with esophageal dysmotility and gastroesophageal reflux disease, which may diminish survival after lung transplantation (LTx). We studied LTx outcomes in patients with a CTD stratified by esophageal motility. We identified patients who underwent bilateral LTx from 2012 to 2017. Patients with a CTD were classified by pre-LTx diagnosis: absent esophageal motility (AEM), ineffective esophageal motility (IEM), or preserved esophageal motility (PEM). The primary endpoint was 3-year survival. Sub-analysis compared survival between the AEM group and a propensity-matched (lung allocation score), non-CTD control group. Kaplan-Meier method and log-rank test were used. In total, 495 patients underwent LTx; 33 (6.7%) had a CTD. Median (IQR) age was 62 years (55.5-67.0); 24 (72.7%) were women. Survival trended lower for recipients with a CTD than without a CTD at 1-year (84.8% vs 91.8%; p = 0.2) and 3-years (66.7% vs 73.5%; p = 0.5). Within the CTD cohort, 1- and 3-year survival was significantly higher in the PEM (100%, 87.5%) and IEM (100%, 85.7%) groups than in the AEM group (50%, 20%; p < 0.001). The AEM group had significantly lower survival at 1-year (50% vs 92.5%) and 3-years (20% vs 65%) than a lung allocation score-matched cohort of patients without a CTD. LTx recipients with a CTD and AEM had significantly lower survival than those with PEM or IEM as well as significantly lower survival than that of a propensity-matched cohort of patients without a CTD. Patients with a CTD and AEM should be considered for LTx with extreme caution and counseled appropriately.