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To evaluate survival and prognostic factors in pediatric idiopathic pulmonary arterial hypertension.
We analyzed data from 119 patients <18 years of age at diagnosis who were enrolled in the pulmonary hypertension center in Beijing An Zhen Hospital, the demographics, hemodynamic Parameters and follow-up data were collected.
Median age at diagnosis was 5.90±4.23 years, decreased activity was the most frequent symptom(77.5%),At the time of diagnosis,56.3% patients were in NYHA functional classIII-IV, mean pulmonary artery pressure and pulmonary vascular resistance index were 66.41±19.20mmHg and 16.95±9.19 Wood/m2,respectively. 99 cases(83.2%)patients received targeted therapy. Mean survival time from the diagnosis was 37.0±17.9 month. with 1-,3-,5-year survival rates of 86.3,51.4 and 37.8%, respectively. The analysis of the survival curves revealed a better prognosis in patients with baseline BNP level < 605 pg/mL and baseline I/II NYHA functional class.44 cases(36.9%)died, with a worse NYHA functional class, high level of BNP and total bilirubin, higher probability of edema, the right atrial pressure is higher, and less acute vasodilator testing responders. Variables that were significantly associated with decreased survival in univariate analysis included edema(HR=2.723,P=0.005),NYHA function classification (HR=2.310,P=0.028),troponin (HR=16964.8,P=0.024),total bilirubin(HR=1.066,P=0.001),PA/AO diameter ratio(HR=3.719,P=0.030),Additional variables at diagnosis that were associated with increased survival included the existence of PFO/ASD (HR=0.563,P=0.169). In multivariate analysis only edema (HR=2.398,P=0.025) associated with decreased survival.
we identified key predictors of survival in pediatric IPAH. Refining these prognostic parameters should help clinicians improve outcomes.