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.