Trial reportBMC cardiovascular disorders2017
Efficacy and safety of oral sildenafil in children with Down syndrome and pulmonary hypertension.
Trial report in BMC cardiovascular disorders, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00159913 (A Randomized, Double-Blind, Placebo Controlled, Dose Ranging, Parallel Group Study of Oral Sildenafil in the Treatment of Children, Aged 1-17 Years, With Pulmonary Arterial Hypertension.), which is not on this map. Cited by 22 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
A Randomized, Double-Blind, Placebo Controlled, Dose Ranging, Parallel Group Study of Oral Sildenafil in the Treatment of Children, Aged 1-17 Years, With Pulmonary Arterial Hypertension.
Who cites it
22 citing papers in PubMed, 36 citations in OpenAlex.
- Association between upper airway obstruction and pulmonary hypertension in children with Down syndrome.Current opinion in pulmonary medicine · 2026Review
- How Is Pulmonary Hypertension Characterised and Treated in Children With Trisomy 21? Observations From the TOPP Registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension).Pulmonary circulation · 2025Article
- Article
- Effects of sildenafil and metformin on endothelin-1 in the progression of pulmonary hypertension.Open veterinary journal · 2025Article
- Tailoring of Bilosomal Nanogel for Augmenting the Off-Label Use of Sildenafil Citrate in Pediatric Pulmonary Hypertension.ACS omega · 2024Article
- Development of pulmonary hypertension remains a major hurdle to corrective surgery in Down syndrome.World journal of cardiology · 2024Article
- Advances in targeted therapy for pulmonary arterial hypertension in children.European journal of pediatrics · 2023Review
- Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus.Circulation · 2023Article
- Pulmonary Hypertension in the Population with Down Syndrome.Cardiology and therapy · 2022Review
- Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome.Pediatric cardiology · 2022Article
- Long-Term Survival and Causes of Death in Children with Trisomy 21 After Congenital Heart Surgery.The Journal of pediatrics · 2021Article
- Clinical Efficacy and Safety of Different Doses of Sildenafil in the Treatment of Persistent Pulmonary Hypertension of the Newborn: A Network Meta-analysis.Frontiers in pharmacology · 2021Article
- Down syndrome and congenital heart disease: perioperative planning and management.Journal of congenital cardiology · 2021Review
- Review
- Review
- Treatment of pediatric pulmonary arterial hypertension: A focus on the NO-sGC-cGMP pathway.Pediatric pulmonology · 2019Review
- Postoperative and long-term outcomes in children with Trisomy 21 and single ventricle palliation.Congenital heart disease · 2019Article
- Pulmonary vascular resistance index: Getting the units right and why it matters.Clinical cardiology · 2019Review
- Sildenafil Use in Children with Pulmonary Hypertension.The Journal of pediatrics · 2019Article
- Tale of 2 Endothelin Receptor Antagonists in Eisenmenger Syndrome.Circulation · 2019Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDespite the increased risk for pulmonary hypertension in children with Down syndrome, the response to treatment with targeted therapies for pulmonary hypertension in these patients is not well characterized. The Sildenafil in Treatment-naive children, Aged 1-17 years, with pulmonary arterial hypertension (STARTS-1) trial was a dose-ranging study of the short-term efficacy and safety of oral sildenafil in children with pulmonary arterial hypertension. We assessed the safety and efficacy of oral sildenafil in children with Down syndrome and pulmonary arterial hypertension.
methodsThis was a post-hoc analysis of children with Down syndrome and pulmonary arterial hypertension enrolled in the STARTS-1 trial. Mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance index (PVRI), and cardiac index (CI) were assessed at baseline and following 16 weeks of treatment with sildenafil.
resultsOf 234 patients randomized and treated in the STARTS-1 trial, 48 (20.5%) had Down syndrome. Although sildenafil produced dose-related reductions in PVRI and mPAP, compared with placebo, in non-Down syndrome patients and children developmentally able to exercise, this was not satisfactorily marked in patients with Down syndrome. The dose-related reductions in PVRI, compared with placebo, occurred in all subgroups, with the exception of the Down syndrome subgroup. Sildenafil appeared to be well tolerated in the Down syndrome subpopulation and the most frequently reported AEs were similar to those reported for the entire STARTS-1 population.
conclusionSildenafil treatment for 16 weeks had no effect on PVRI or mPAP in children with Down syndrome and pulmonary arterial hypertension. The results suggest that children with Down syndrome may be less responsive to sildenafil for pulmonary arterial hypertension, but the incomplete work-up for the etiology of pulmonary arterial hypertension may have introduced a potential bias.
trial registrationStudy received, September 8, 2005 (retrospectively registered); Study start, August 2003; ClinicalTrials.gov identifier, NCT00159913 .
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.