Survival in Paediatric Pulmonary Arterial Hypertension

Abstract

Introduction: Outcome of paediatric PAH has not been studied in our population. Current study aimed to see the outcome of children over a study period of one year who were diagnosed as pulmonary arterial hypertension. Material and Methods: Study was done on all children 0-15 years age diagnosed with PAH on Transthoracic Echocardiography with systolic pulmonary artery pressure (sPAP) of >35 mmHg. Only Group 1 PAH (WHO) were included and were followed for 1 year. Various clinical and echocardiographic variables affecting outcome were noted. Results: Total number of PAH cases were 40. Mean age at the time of diagnosis was 7.3 months. 23 (57.5%) of the PAH patients were females whereas 17 (42.5%) were males. Idiopathic PAH constituted 42.5% of the study group, whereas 50% of the PAH cases were associated with CHD. 7.5% cases were diagnosed as PPHN Out of the total of 40 cases studied 10 patients died representing 25% mortality over 1 year. Conclusion: Paediatric PAH is associated with high mortality in our population.Those having Right heart failure and Right Ventricular Dysfunction need close follow up.