摘要：Introduction : Pulmonary arterial hypertension (PAH) is a rare disease associated with significant morbidity and mortality. Pediatric patients often present with mixted aetiologies. Objectives : To characterize the epidemiology, management and outcome of pediatric PAH. Methods : Children with PAH were included and followed prospectively for six months. WHO functional class, 6-minute walk test, biomarkers, electrocardiogram, spirometers and echocardiographic parameters were evaluated in progressive PAH group. Results : Two hundred and four children were included in the study from July 2012 until July 2013, with a mean age of 6.13 years. Transient PAH patients (n=170, 83.33%) included newborns with persistent pulmonary hypertension (n=8, 3.92%) and children with congenital heart defects with systemic-to-pulmonary shuntflow PAH (n=162, 79.41%) in whom PAH resolved after successful surgery correction. Progressive PAH (n=34, 16.66%) included patients with idiopathic PAH (n=5, 2.45%), Eisenmenger syndrome (n=17, 8.33%) and post-operative PAH (n= 6, 2.94%). Patients with progressive PAH remained stable in regards to clinical status, WHO functional class, 6-minute walk distance, biomarkers, spirometers parameters and echocardiographic parameters with prognostic value. Conclusions : Pediatric PAH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Pediatric PAH associated with congenital heart defects represents a heterogeneous group with highly variable clinical courses. PAH specific therapies may have contributed to disease stability and favorable outcomes.
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