1. Treatment initiation in paediatric pulmonary hypertension
- Author
-
D. Dunbar Ivy, Rolf M. F. Berger, Malgorzata Zuk, Margrit S Fasnacht Boillat, Eric D. Austin, Ingram Schulze-Neick, Maurice Beghetti, Tilman Humpl, Damien Bonnet, Cardiovascular Centre (CVC), and Vascular Ageing Programme (VAP)
- Subjects
Male ,Cardiac Catheterization ,Pulmonary Circulation ,Vasodilator Agents ,medicine.medical_treatment ,CHILDREN ,030204 cardiovascular system & hematology ,DISEASE ,Targeted therapy ,0302 clinical medicine ,Quality of life ,SILDENAFIL CITRATE ,Registries ,Child ,ddc:618 ,General Medicine ,Calcium Channel Blockers ,Prognosis ,Child, Preschool ,SAFETY ,SURVIVAL ,Female ,Cardiology and Cardiovascular Medicine ,EPOPROSTENOL ,medicine.drug ,medicine.medical_specialty ,Adolescent ,Combination therapy ,Hypertension, Pulmonary ,BOSENTAN ,03 medical and health sciences ,Internal medicine ,medicine ,Humans ,Pulmonary Wedge Pressure ,Intensive care medicine ,Pulmonary wedge pressure ,Antihypertensive Agents ,business.industry ,Vascular disease ,Infant ,Phosphodiesterase 5 Inhibitors ,medicine.disease ,Pulmonary hypertension ,Bosentan ,COMBINATION THERAPY ,030228 respiratory system ,Supportive psychotherapy ,Pediatrics, Perinatology and Child Health ,ARTERIAL-HYPERTENSION ,business - 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 2, 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.
- Published
- 2017