Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry.
Details
Serval ID
serval:BIB_CFB00EE527D2
Type
Article: article from journal or magazin.
Publication sub-type
Editorial
Collection
Publications
Institution
Title
Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry.
Journal
Cardiology in the young
ISSN
1467-1107 (Electronic)
ISSN-L
1047-9511
Publication state
Published
Issued date
08/2017
Peer-reviewed
Oui
Volume
27
Number
6
Pages
1123-1132
Language
english
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
Publication Status: ppublish
Abstract
Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry. Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected. Inclusion criteria were >3 months and <18 years of age and diagnostic cardiac catheterisation consistent with pulmonary hypertension (mean pulmonary arterial pressure ⩾25 mmHg, pulmonary vascular resistance index ⩾3 Wood units×m2, and mean pulmonary capillary wedge pressure ⩽12 mmHg). At diagnostic catheterisation, 217/244 patients (88.9%) were treatment naïve for pulmonary hypertension-targeted therapy. Targeted therapy was initiated after catheterisation in 170 (78.3%) treatment-naïve patients. A total of 19 patients received supportive therapy, 28 patients were not started on therapy, and 26 patients (10.7%) were on targeted treatment before catheterisation. Among treatment-naïve subjects, treatment was initiated with one targeted drug (n=112, 51.6%), dual therapy (n=39, 18%) or triple-therapy (n=5, 2.3%), and calcium channel blockers with one targeted medication in one patient (0.5%). Phosphodiesterase inhibitors type 5 were used frequently; some patients with pulmonary hypertension related to lung disease received targeted therapy. There is a diverse therapeutic approach for children with pulmonary hypertension with a need of better-defined treatment algorithms based on paediatric consensus for different aetiologies including the best possible diagnostic workup.
Keywords
Adolescent, Antihypertensive Agents/therapeutic use, Calcium Channel Blockers/therapeutic use, Cardiac Catheterization/methods, Child, Child, Preschool, Female, Humans, Hypertension, Pulmonary/diagnosis, Hypertension, Pulmonary/drug therapy, Hypertension, Pulmonary/physiopathology, Infant, Male, Phosphodiesterase 5 Inhibitors/therapeutic use, Prognosis, Pulmonary Circulation, Pulmonary Wedge Pressure/physiology, Registries, Vasodilator Agents/therapeutic use, Pulmonary hypertension, children, treatment
Pubmed
Web of science
Create date
10/01/2019 18:15
Last modification date
11/10/2019 6:26