, a bio/informatics shared resource is still "open for business" - Visit the CDS website


Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry.

Humpl T, Berger RMF, Austin ED, Fasnacht Boillat MS, Bonnet D, Ivy DD, Zuk M, Beghetti M, Schulze-Neick I
Cardiol Young. 2017 27 (6): 1123-1132

PMID: 27995825 · DOI:10.1017/S1047951116002493

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.

MeSH Terms (17)

Adolescent Antihypertensive Agents Calcium Channel Blockers Cardiac Catheterization Child Child, Preschool Female Humans Hypertension, Pulmonary Infant Male Phosphodiesterase 5 Inhibitors Prognosis Pulmonary Circulation Pulmonary Wedge Pressure Registries Vasodilator Agents

Connections (1)

This publication is referenced by other Labnodes entities:

Links