Amy L. Kiskaddon

and 3 more

Background: Intravenous (IV) Sildenafil form may be given intermittently or continuously. This study evaluated if IV continuous sildenafil titration is well tolerated and looked at adverse events and outcomes when used for management of pulmonary hypertension (PH). Methods: Retrospective study (January 1, 2016 - March 31, 2019). Patients ≤18 years of age on IV continuous sildenafil with diagnosis of secondary PH. Change in dose (mg/kg/hr.) and frequency of dose change were collected. Heart rate, blood pressure, and oxygen saturation immediately following the change of dose and echocardiographic data were collected. Primary objective evaluated tolerability. Secondary outcomes: describe titration regimens and clinical outcomes. Clinical characteristics were summarized with descriptive statistics. Continuous variables were reported with medians and categorical variables with frequencies and percentages. Results: The median age and weight were 156 (5-890) days and 4.2 (1.9-12.1) kg, respectively. Median initial and maximum doses of continuous IV sildenafil were 1 mg/k/day (0.4-2.5) and 4 mg/k/day (2-4). The median (range) time of continuous IV sildenafil titration was every 8 (5.7-89.3) hours. Median number of times IV continuous sildenafil was upward titrated was 5 (2-8) times. Hemodynamically important adverse events noted was decrease in blood pressure in 3 subjects (23.1%). A decrease in the estimated right ventricular pressure was noted in 9/11 subjects (81%) Conclusion: According to these data, continuous IV sildenafil appears to be tolerated, but may be associated with hypotension. Further studies are necessary to compare intermittent to continuous IV sildenafil therapy.