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

Humpl, Tilman
Berger, Rolf M F
Austin, Eric D
Fasnacht Boillat, Margrit S
Bonnet, Damien
Ivy, Dunbar D
Zuk, Malgorzata
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Published in Cardiology in the Young. 2017, vol. 27, no. 6, p. 1123-1132
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.
PMID: 27995825
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Research group L'hypertension pulmonaire (pédiatrie) (228)
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HUMPL, Tilman et al. Treatment initiation in paediatric pulmonary hypertension: insights from a multinational registry. In: Cardiology in the Young, 2017, vol. 27, n° 6, p. 1123-1132. doi: 10.1017/S1047951116002493 https://archive-ouverte.unige.ch/unige:96726

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Deposited on : 2017-09-14

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