Clinical profile and outcome of paediatric pulmonary artery hypertension patients

Introduction: Patterns, prevalence & outcome of Paediatric PAH have not been characterized in our local population. Aims & Objectives: To study the clinico-echocardiographic profile and outcome of children diagnosed as pulmonary arterial hypertension (PAH). Material & 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. All patients were followed for one year 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. PPHN Out of the total of 40 cases studied 10 patients died representing 25% mortality over 1 year. 5 had IPAH, 4 had PAH with CHD and 1 had PPHN. Mortality for different types of PAH was as follows: IPAH: 5 out of 17 (29.41%) PAH associated with CHD: 4 out of 20 (20%) PPHN: 1 out of 3 (33%) .Overall 1 year survival rate for PAH was 75%. For different types of PAH the survival rates were as follows: IPAH: 70.59% PAH associated with CHD: 80% PPHN: 66.7%. Right heart failure (p value .0001) and evidence of right ventricular systolic dysfunction (p value .04) were found significantly associated with Increased mortality 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. Paediatric PAH is associated with high mortality in our population. Those having Right heart failure and Right Ventricular Dysfunction need close follow up.