Evaluation of the Tolerability of Intermittent Intravenous Sildenafil in Pediatric Patients With Pulmonary Hypertension

Abstract

The primary purpose of this study was to determine the tolerability of intermittent intravenous (IV) sildenafil for the treatment of pulmonary hypertension in pediatric patients. Secondary objectives were to evaluate parameters related to efficacy. METHODS: This was a retrospective chart review from January 2013 to August 2014 of pediatric patients under age 18 years treated with intermittent doses of IV sildenafil for pulmonary hypertension. Patients were excluded if they were over age 18 years or received sildenafil for other indications. Measures collected to assess tolerability include blood pressure and heart rate before and after the administration of IV sildenafil, as well as adverse events. RESULTS: Thirty-seven patients (21 females and 16 males) were identified meeting inclusion criteria, and 21 (56.8%) were on oral sildenafil prior to the initial IV dose. The mean decrease in blood pressure after the first dose of IV sildenafil was 7.16/2.74 mmHg. The decrease in systolic blood pressure was statistically significant. During the study period, 5 patients experienced medication related adverse events, primarily hypotension. Despite this, none of the patients had the medication discontinued due to these events. For secondary objectives, a statistically significant difference was not found between other clinical measures before and after intermittent IV sildenafil dosing. CONCLUSIONS: Sildenafil, when administered as intermittent IV doses, was tolerated by the majority of patients evaluated in this study. For pediatric patients with pulmonary hypertension in whom enteral or continuous IV sildenafil cannot be administered, intermittent IV sildenafil may be considered as an alternative administration option.

Role of microRNA-130a in the pathogeneses of obstructive sleep apnea hypopnea syndrome-associated pulmonary hypertension by targeting the GAX gene

“…[ 28 ] And PHT is defined by an imbalance with an increase in pulmonary vascular resistance and pulmonary artery pressures. [ 29 ] This study explored the effect of miR-130a in OSAHS-associated PHT by targeting the GAX gene, our findings validate that miR-130a might limited the expression of GAX and induced OSAHS-associated PHT. A previous study has identified that many different pathogeneses, including hepatocellular carcinoma, cervical cancer, and ovarian cancer, were caused by miR-130a, and many genes which have a strong relationship with different cancer pathways have been identified and validated as targeted genes of miR-130a, such as the GAX gene.…”

Continuous and intermittent administration of intravenous sildenafil in critically ill infants with pulmonary hypertension

“…5 Intermittent IV sildenafil has demonstrated efficacy in various studies, 6 however hypotension is a reported adverse effect. 7 Studies investigating IV versus oral delivery have not demonstrated significant differences in safety outcomes. 8 Slower administration of IV sildenafil over longer infusion periods has been suggested as a way of avoiding the acute systemic vasodilatory effects of more rapid bolus dosing.…”

Treatment of pediatric pulmonary arterial hypertension: A focus on the NO‐sGC‐cGMP pathway

“…In 2014, however, the FDA clarified this warning, stating that there may be situations in which the risk‐benefit profile may be acceptable in individual children, although it is still not recommended (https://www.fda.gov/drugs/drugsafety/ucm390876.htm). Additional pediatric trials of sildenafil in PAH have been performed but are not discussed here because of low patient numbers and/or lack of placebo control …”