Efficacy and safety of oral sildenafil in children with Down syndrome and pulmonary hypertension
| Autor: | Maurice Beghetti, Min Zhang, Andrzej Rudziński |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: |
Male
lcsh:Diseases of the circulatory (Cardiovascular) system Time Factors Vasodilator Agents Down syndrome Cardiac index Administration Oral 030204 cardiovascular system & hematology chemistry.chemical_compound 0302 clinical medicine 030212 general & internal medicine Pulmonary Artery/drug effects/physiopathology Child Children education.field_of_study ddc:618 Arterial Pressure/drug effects Cardiac surgery Treatment Outcome Vasodilator Agents/administration & dosage/adverse effects Adolescent Antihypertensive Agents/administration & dosage Antihypertensive Agents/adverse effects Child Preschool China Down Syndrome/complications Down Syndrome/diagnosis Female Humans Hypertension Pulmonary/diagnosis Hypertension Pulmonary/drug therapy Hypertension Pulmonary/etiology Hypertension Pulmonary/physiopathology Infant Pulmonary Artery/drug effects Pulmonary Artery/physiopathology Sildenafil Citrate/administration & dosage Sildenafil Citrate/adverse effects Vascular Resistance/drug effects Vasodilator Agents/administration & dosage Vasodilator Agents/adverse effects Pulmonary hypertension Sildenafil Administration Hypertension Cardiology cardiovascular system Cardiology and Cardiovascular Medicine Research Article Oral medicine.medical_specialty Hypertension Pulmonary Population Pulmonary Artery Placebo Sildenafil Citrate 03 medical and health sciences Internal medicine medicine Sildenafil Citrate/administration & dosage/adverse effects Arterial Pressure education Preschool Antihypertensive Agents Angiology business.industry Pulmonary/diagnosis/drug therapy/etiology/physiopathology medicine.disease Down Syndrome/complications/diagnosis respiratory tract diseases chemistry lcsh:RC666-701 Vascular Resistance Antihypertensive Agents/administration & dosage/adverse effects business |
| Zdroj: | BMC cardiovascular disorders, vol. 17, no. 1, pp. 177 BMC Cardiovascular Disorders, Vol. 17, No 1 (2017) P. 177 BMC Cardiovascular Disorders BMC Cardiovascular Disorders, Vol 17, Iss 1, Pp 1-9 (2017) |
| ISSN: | 0015-9913 1471-2261 |
| Popis: | Background Despite 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. Methods This 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. Results Of 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. Conclusion Sildenafil 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 registration Study received, September 8, 2005 (retrospectively registered); Study start, August 2003; ClinicalTrials.gov identifier, NCT00159913. Electronic supplementary material The online version of this article (doi:10.1186/s12872-017-0569-3) contains supplementary material, which is available to authorized users. |
| Databáze: | OpenAIRE |
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