Clinicoechocardiographic Profile of Paediatric Pulmonary Artery Hypertension Patients

ABSTRACT

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.