ABSTRACT
Objective To investigate the etiology and clinical characteristics of pediatric pulmonary arterial hypertension(PAH) and improve its early diagnosis and treatment.Methods The clinical and echocardiogram data of all inpatients with PAH in Pediatric Department of Peking University First Hospital between May 1995 and May 2007 were retrospectively analyzed for age,sex,etiology,symptoms and echocardiographic measurement of pulmonary artery pressure.Data were divided into groups according to different etiology and statistics.Pulmonary arterial systolic pressure(sPAP) values estimated from the tricuspid regurgitant velocity by Doppler echocardiography were compared among different groups.Cases who were not belonged to the first category of the Venice Clinical Classification of pulmonary hypertension were not included.Results Totally 276 cases,168 boys and 108 girls were diagnosed to have PAH.Age ranged from 1 month to 17 years,median age was 9 months.Most of pediatric PAH was associated-PAH(267 cases,96.7%),while idiopathic PAH took a small part(9 cases,3.3%).Congenital heart disease-associated PAH(CHD-PAH) was predominant(245 cases,88.7%) and left to right shunt was the main lesion (217 cases,88.6%),while complex lesion-associated PAH comprised 28 cases(11.4%).Connective tissue disease associated PAH(CTD-PAH) was the second common among this group of pediatric PAH patients(19 cases,6.9 %).The incidence of PAH in systemic lupus erythematosus(SLE),juvenile rheumatoid arteritis and takayasu arteritis were 10.3 %(13/126),8.7%(4/46),15.4%(2/13),respectively.The other 3 cases of PAH were associated with portal hypertension(2 cases) and thalassanemia(1 case).The estimated sPAP from tricuspid regurgitant velocity in 8 cases with idiopathic PAH[(74.6?23.9) mmHg(1 mmHg=0.133 kPa)]was higher significantly compared with those of 33 cases of CHD-PAH [(58.0?19.7) mmHg ] and 12 cases of CTD-PAH [(49.6?18.9) mmHg] respectively(t=-2.052,-2.609 Pa