Sildenafil Use in Children with Pulmonary Hypertension - 24/01/19
Abstract |
Objective |
To assess the demographics, treatment algorithm, and outcomes in a large cohort of children treated with sildenafil.
Study design |
A retrospective cohort study of children with pulmonary hypertension (PH) treated with sildenafil at a single institution between 2004 and 2015. Baseline and follow-up data collected by chart review.
Results |
There were 269 children included in this study: 47 with idiopathic pulmonary arterial hypertension, 53 with congenital heart disease, 135 with bronchopulmonary dysplasia, 24 with congenital diaphragmatic hernia, and 7 with other causes. Sildenafil was initial monotherapy in 84.8% and add-on therapy in 15.2%. Median follow-up time was 3.1 years (2 weeks-12.4 years). On follow-up, 99 (37%) remained on sildenafil or transitioned to tadalafil, 93 (35%) stopped sildenafil for improvement in PH, 54 (20%) died, and 20 (7%) were lost to follow-up. PH was most likely to improve in those with bronchopulmonary dysplasia, allowing for the discontinuation of sildenafil in 45%. Eighteen deaths were related to PH and 36 from other systemic causes. Two patients stopped sildenafil owing to airway spasm with desaturation. Overall survival was significantly lower in World Health Organization group 3 PH (bronchopulmonary dysplasia and congenital diaphragmatic hernia) vs group 1 (idiopathic pulmonary arterial hypertension and congenital heart disease), P = .02.
Conclusions |
In this retrospective experience in children with mainly World Health Organization groups 1 and 3 PH, low-dose sildenafil was well-tolerated, safe, and had an acceptable side effect profile. Although patients with group 3 PH have high mortality, survivors have a high likelihood of PH improving.
Le texte complet de cet article est disponible en PDF.Keywords : Sildenafil, bronchopulmonary dysplasia, congenital diaphragmatic hernia, congenital heart disease
Abbreviations : BPD, CDH, CHD, FDA, IPAH, PAH, PDE, PH, WHO
Plan
S.N. receives salary support through a Ruth L. Kirschstein National Research Service Award of the National Institutes of Health (5T32HL007854-22). The authors declare no conflicts of interest. |
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Portions of this study were presented as an oral presentation at the Pediatric Academic Societies annual meeting, May 6-9, 2017, San Francisco, California. |
Vol 205
P. 29 - février 2019 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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