Continuous intravenous sildenafil use, dose titration, tolerability and
clinical outcomes in children with pulmonary hypertension.
Abstract
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.