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BACKGROUND: Pulmonary arterial hypertension (PAH) is a life-threatening disease that appears to cause an increase in perioperative risk. The present study compared patients with pulmonary hypertension to a control group to determine predictors of poor outcome. METHODS: An IRB approved retrospective review of all patients undergoing non-cardiac surgery from January 2003 through August 2009 was analyzed. They were compared to a cohort group of patients, without PAH undergoing similar operations during the study period. The two groups were statistically analyzed. RESULTS: 106 patients underwent surgery of which 36 cases (23 females and 13 males) were non-cardiac. These patients were compared. They were compared to a cohort group of 72 patients. The two groups were demographically similar. The two groups were also similar based length and type of operation, anesthetic technique, history of asthma, deep venous thrombosis, prior coronary artery or cardiac valve surgery, underlying renal disease, and liver disease. However, patients in the PAH had a higher incidence in treatment for congestive heart failure (p < 0.01). The average length of stay in the hospital for the PAH group was 19.3 days compared to control group of 7.8 days (p < 0.05). The PAH group had a greater incidence of morbid events compared to their matched cohorts (1.8 vs. .57 per patient, p < 0.05). 53% patients in the PAH group experienced had at least one or more morbid event after their surgery compared to 6% of cohorts. The mortality was 22% in patients with PAH and none in the cohort group (p<0.05). CONCLUSIONS: Patients with PAH are at a much greater risk for complications and death when undergoing non-cardiac surgery. The effect of PAH should be carefully considered when planning elective surgery.