Clinical features and prognostic factors in children with idiopathic pulmonary arterial hypertension

Abstract

Objective To investigate the clinical features and prognostic factors in children with idiopathic pulmonary arterial hypertension(IPAH). Methods The data of children with IPAH diagnosed in Beijing Children's Hospital from January 2006 to December 2015 were collected.The cases were divided into survival group and death group according to the prognosis.The risk factors influencing prognosis in children with IPAH were identified by Logistic regression analysis. Results Total 26 patients were enrolled in this study, and 17 of them survived and 9 of them were dead.IPAH was found to be more common in school-age children.The average age of the patients was(6.6±3.6)years and the male to female ratio was 1.17∶1.All of the children had the symptoms with decreased activity tolerance and fatigue.The incidence of syncope and dyspnea and edema of lower extremity were 34.6%, 23.1%.Accentuated pulmonic second sound(P2) was detected in 92.3% of patients during physical examination, which was also the most common sign.About 50.0% patients were functional class Ⅲ-Ⅳ.Echocardiography showed that all childrens' mean pulmonary artery systolic pressure(PASP) was(79.0±20.3) mmHg(1 mmHg=0.133 kPa) and 16 of them with right ventricular dilatation.Electrocardiogram with ST-T changes and right ventricular high voltage were common.The average plasma level of brain natriuretic peptide(BNP) in all cases was(870.4±720.9) pg/ml.The single factor analysis illustrated that syncope(OR=26.25, 95%CI 3.04-226.60, P=0.003), heart functional class Ⅲ-Ⅳ(OR=19.199, 95%CI 1.88-196.53, P=0.0128), PASP≥70 mmHg(OR=9.936, 95%CI 1.81-∞, P=0.005), BNP≥850 pg/ml(OR=59.991, 95%CI 4.69-767.62, P=0.002) indicated the worse outcome. Conclusion IPAH in children is rare and the prognosis is poor.Syncope, cardiac function grade, PASP and the levels of BNP are the main important factors affecting the prognosis of children with IPAH.BNP has important clinical significance for the evaluation of IPAH and the prognosis of the disease. Key words: Idiopathic pulmonary arterial hypertension; Clinical features; Prognosis; Children