Clinical Features Of Pediatric Pulmonary Hypertension In 716 Patients

Objectives: to get a good understanding of clinical characteristics in pediatric pulmonary hypertension(PH),and to provide with information for earlier detection and treatment.Methods: 716 children diagnosed with PH in the children’s Hospital of Chongqing Medical University were enrolled in this study from 2016.5.1 to 2017.5.1.Characteristics including demographic data,presenting symptoms,world health organization functional classifications(WHO-FC),and auxiliary examination data were recorded for statistical analysis.Results: A total of 716 children with PH were enrolled,including 360 males and 356 females.Median age at diagnosis was 0.58 years(0.08 years~15.5 years).Median time from onset to diagnosis was 2 months(0.02 months~180 months).661 cases(92.3%)had pulmonary arterial hypertension associated with congenital heart disease(aCHD-PAH).21 cases(2.9%)had PH due to left heart diseases.16 cases(2.3%)had PH due to chronic lung diseases,and only 18 cases(2.5%)had idiopathic pulmonary hypertension(iPAH).Dyspnea(482 cases,67.3%),growth retardation(396 cases,55.3%),and fatigue(350 cases,48.9%)were the most frequently clinical symptoms.According to WHO-FC,452 cases(63.1%)were in functional class I or II,and 264 cases(36.9%)were in III or IV.Children in PH due to left heart diseases,which were classed with class III or IV,were more than those in aCHD-PAH and PH due to chronic lung diseases(all P<0.01).Pulmonary artery systolic pressure(PASP)of 716 patients was(61.2±18.5)mmHg.According to the classification of PASP,mild group accounted for 32%(229 cases),moderate(214 cases,29.9%),and severe(273 cases,30.1%).The cases of severe PH in iPAH were more than other groups of PH(all P<0.01).270 cases(37.7%)had abnormal electrophysiological conditions in pediatric PH,including ST-T segment or T wave(135 cases,18.9%)and Heart-block(107 cases,14.9%).In aCHD-PAH,33 cases(4.6%)were diagnosed as 21-trisomy syndrome,124 cases(18.8%)had ventricular septal defect(VSD),and 125 cases(18.9%)had VSD combined with atrial septal defect(ASD).Moreover,59 cases(8.9%)had coarctation of aorta,and 46 cases(7%)had anomalous pulmonary venous connection.The critical value of the ratio of main pulmonary artery diameter to main ascending aorta diameter(MPAD/MAD)to evaluate the occurrence of PH was 1.22(sensitivity 83.7%,specificity 60.2%)by ROC curve.Conclusion: The aCHD-PAH is the most common classification of pediatric PH,and iPAH is rare.A ratio of MPAD to MAD of 1.22 should raise the suspicion of PH in children.