Introduction: Pulmonary arterial hypertension (PAH) is a rare pathology with different etiologies, representing a cause of morbidity and mortality in the pediatric age group. Most cases of PAH in children are secondary to congenital heart diseases (CHD), followed closely by idiopathic PAH and familial forms. Our objective was to evaluate children with pulmonary arterial hypertension in order to establish which parameter is more useful for the assessment of pulmonary arterial hypertension.
Method: Twenty pediatric patients diagnosed with pulmonary arterial hypertension undergoing pulmonary vasodilator therapy were evaluated between March 2008 and January 2012 in the Pediatric Cardiology Department from Tirgu Mures County Emergency Clinical Hospital. Patients were assessed clinically, the exercise capacity was assessed using the 6-minute walk test, and echocardiography was performed. The initial assessment was considered at the time of initiation of the pulmonary vasodilator therapy, further evaluations being performed at intervals of 3 months.
Results: This study demonstrates the improvement of the functional class and 6-minute walk test, without compromising peripheral oxygen saturation whereas echocardiographic evaluation of patients did not show any echocardiographic parameter to correlate with improved exercise capacity and functional class.
Conclusion: The 6-minute walk test and NYHA functional class represent useful parameters for evaluating the efficiency of pulmonary vasodilator therapy

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 shunt- flow 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.