Introduction: Pulmonary arterial hypertension (PAH) is a progressive and ultimately fatal disease of the pulmonary circulation. There has never been an investigation of the end-of-life symptoms in patients with PAH. In this investigation, we surveyed surrogates of recent decedents with PAH. We evaluate their responses to better understand the end-of-life experience of patients with PAH. Methods: The survey instrument includes demographic information and the Edmonton Symptoms Assessment Scale. Accredo Therapeutics mailed the survey to surrogates of recent decedents with PAH, and responses were anonymously returned to investigators at Virginia Commonwealth University and used in our descriptive analysis. Results: Of 100 surveys distributed over 24 months (February 2009 to February 2011), we obtained 36 responses (response rate 36%). We found that most patient deaths (90%) were related to PAH, that the majority of patients died in the hospital (67%), with the majority of in-hospital deaths (83%) occurring in intensive care. Palliative care was infrequently involved in patients' care, and many surrogates were unaware of palliative care and hospice services available to the decedents. Patients died with a high symptom burden, especially dyspnea. Conclusion: In this cohort, patients with PAH usually died from their disease, often in the hospital setting with a high symptom burden. Further study will be needed to confirm the findings from this study and to better understand the forces leading to the trends uncovered in this investigation. © Copyright 2012, Mary Ann Liebert, Inc.