Survival in Paediatric Pulmonary Arterial Hypertension

ABSTRACT

Introduction: Outcome of paediatric PAH has not beenstudied in our population. Current study aimed to see theoutcome of children over a study period of one year who werediagnosed as pulmonary arterial hypertension.Material and Methods: Study was done on all children0-15 years age diagnosed with PAH on TransthoracicEchocardiography with systolic pulmonary artery pressure(sPAP) of >35 mmHg. Only Group 1 PAH (WHO) wereincluded and were followed for 1 year. Various clinical andechocardiographic variables affecting outcome were noted.Results: Total number of PAH cases were 40. Mean ageat the time of diagnosis was 7.3 months. 23 (57.5%) of thePAH patients were females whereas 17 (42.5%) were males.Idiopathic PAH constituted 42.5% of the study group, whereas50% of the PAH cases were associated with CHD. 7.5% caseswere diagnosed as PPHN Out of the total of 40 cases studied10 patients died representing 25% mortality over 1 year.Conclusion: Paediatric PAH is associated with high mortalityin our population.Those having Right heart failure and RightVentricular Dysfunction need close follow up.