Abstract

Context: Pediatric Primary pulmonary Arterial hypertension is a rare disease in infants and an important cause of mortality and morbidity. Timely detection and appropriate treatment strategies are challenges in pediatric age group. Treatable causes need to be identified earlier in the course.

Aim: To study the clinical presentation, treatment and outcome at discharge in children with Primary Pulmonary Arterial Hypertension.

Setting and Design: It is a Time bound retrospective descriptive chart based observational study. Data was collected by reviewing case records in a predesigned, structured proforma of children above 1 month of age with primary pulmonary hypertension from Jan 2017 to July 2018.

Results: There weretotal 12 cases of primary pulmonary arterial hypertension. Mean age of presentation of primary pulmonary hypertension varied between 1½ month to 1½ years. Common symptoms at presentation were hurried breathing, cough, poor feeding. Primary Pulmonary hypertension was not suspected in majority of children at initial assessment because symptoms were variable. 11(92%) presented with signs of cardiac failure. Chest X Rays showed Cardiomegaly in 50% cases and prominent pulmonary conusin 16%. On ECHO, 100% had RA and RV dilatation and severe PAH. 58% children showed Right Ventricular dysfunction. All the cases were treated with Digoxin, Sildenafil, Milrinone. 4(33%) of them were treated with Thiamine in addition. Mortality rate was high (58%), thoughall children who were administered thiamine have survived.

Conclusion: Primary pulmonary hypertension is a rare disease of infancy with atypical presentation. Mortality rate is high. Administration of Thiamine has proved to be beneficial.

Keywords: Primary Pulmonary Arterial Hypertension, Right Ventricular dysfunction