Clinicoechocardiographic Profile of Paediatric Pulmonary Artery Hypertension Patients

Patterns and prevalence of Paediatric PAH have not been characterized in our local population. Aims & Objectives: To study the clinico-echocardiographic profile of children diagnosed as pulmonary arterial hypertension (PAH). Methods: The study was a prospective non-randomized study conducted .The study group included all the children in the age group of 0-15 years who were diagnosed as pulmonary arterial hypertension on Transthoracic Doppler Echocardiography. Detailed history, examination besides other investigations including Chest X ray, complete blood counts, ABG analysis, ECG, screening for connective tissue disorders and HIV, PBF, LFT, KFT were done as per standard guidelines. Results: The total number of admissions during the study period were 22150.Total number of PAH cases were 40. PAH case represented 0.18% of the total admissions. 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. The most common clinical features were irritability (82.5%), tachypnea (75%), cyanosis (70%) followed by poor feeding (65%), features of right heart failure (35%) and syncope (5%). 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. The mean systolic Pulmonary Artery Pressure in the study group was 63.17 mmHg. Most of the cases had severe PAH (65%), whereas moderate and mild PAH cases were 25% and 10% respectively. Conclusion: In view of relatively higher incidence of idiopathic PAH observed in this study in children of Kashmir, further studies are needed to identify the role of possible genetic and familial factors.

Survival in Paediatric Pulmonary Arterial Hypertension

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.