Improving Safety of Intravenous Prostacyclin Administration to Pediatric Patients With Pulmonary Hypertension.

BACKGROUND: Pulmonary hypertension is a rare, life-threatening disease with limited therapeutic options and no definitive cure. Continuous intravenous prostacyclin therapy is indicated for treatment of severe disease. These medications have a narrow therapeutic index and a brief half-life; therefore, administration errors can be lethal. OBJECTIVE: To reduce medication errors through an inpatient program to improve, standardize, and disseminate continuous intravenous prostacyclin therapy practice guidelines. METHODS: Data were collected from the electronic safety reporting system of a single hospital to determine the number and types of continuous intravenous prostacyclin therapy errors that were reported over an 8-year period. A clinical database and hospital pharmacy records were used to determine the number of days on which hospitalized pediatric patients received the therapy. INTERVENTIONS: A nursing-directed quality improvement initiative to enhance the safety of continuous intravenous prostacyclin therapy for pediatric patients was begun in January 2009. Efforts to improve safety fell into 4 domains: policy, process, education, and hospital-wide safety initiatives. RESULTS: The number of therapy errors per 1000 patient days fell from 19.28 in 2009 to 5.95 in 2016. Chi-square analysis was used to compare the result for 2009 with that for each subsequent year, with P values of .66, .35, .16, .09, .03, .12, and .25 found for 2010 through 2016, respectively. CONCLUSIONS: The trend in reduction of continuous intravenous prostacyclin therapy errors suggests that proactive processes to standardize its administration, emphasizing both policy and education, reduce medication errors and increase patient safety.

The impact of right ventricular pressure and function on survival in patients with pulmonary vein stenosis.

Pulmonary vein stenosis (PVS) is associated with pulmonary hypertension (PH), but there is little information regarding the impact of PH on right ventricular (RV) systolic function and survival. We conducted a retrospective cohort study of our patients to explore this and other aspects of pulmonary hemodynamics with PVS. RV function was assessed using qualitative two-dimensional echocardiography. The ratio of systolic pulmonary artery (PA) and aortic pressures (PA:Ao) at cardiac catheterization reflected pulmonary hemodynamics. Reactivity testing employed inhaled nitric oxide + 100% fiO2, or 100% fiO2 only; "reactivity" was a ≥ 20% decrease in PA:Ao. There were 105 PVS patients, although not all had data at every time point. (1) The mean PA:Ao at first cardiac catheterization (n = 77) was 0.79 ± 0.36; at last catheterization (n = 54), PA:Ao = 0.69 ± 0.30; 90% had systolic PAP > one-half systemic. Survival was shorter with PA:Ao > 0.5. (2) Differences in survival relative to RV dysfunction on the first echocardiogram were not significant, although they were using the last echocardiogram. (3) The magnitude of RV dysfunction was positively correlated with PA:Ao. (4) Balloon dilation of PV acutely decreased PA:Ao (-0.13 ± 0.37, P = 0.03 [n = 40 patients]). 5. Of 20 patients tested, 13 were acutely reactive to vasodilators. PH is a major feature of PVS. Reduced RV function and PA:Ao appear to be predictors of survival. Given the importance of PH in this disease, clinical studies of PVS treatments should include measures of PAP and RV function as important variables of interest.