Parameters for the Assessment of Pulmonary Vasodilator Therapy in Pulmonary Arterial Hypertension in Children

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