Primary pulmonary hypertension in children: Clinical characterization and survival

Objectives. This study characterized mortality in a group of Mexican children (n = 18, mean [±SD] age 9.9 ± 3 years) with primary pulmonary hypertension and investigated the factors associated with their survival. Background. Primary pulmonary hypertension is a progressive, fatal disease of unknown cause. Establishing the diagnosis earlier in life may influence prognosis. Methods. A dynamic cohort of children with primary pulmonary hypertension were enrolled between December 1977 and May 1991 and followed up through September 1992. Measurements included hemodynamic and pulmonary function variables in addition to demographic data, medical history and response to vasodilator treatment. We also compared the survival estimates of these children with those of our adult patients with primary pulmonary hypertension (n = 42, mean age 27.9 ± 8.5 years). Results. Baseline mean (±SD) pulmonary artery pressure was similar in children and adults (66 ± 15 vs. 65 ± 18 mm Hg, p = NS), but a higher cardiac index resulted in a lower mean pulmonary vasclar resistance index in children (18 ± 7 vs. 26 ± 12 U/m2, p < 0.01). The proportion of patients who had a positive hemodynamic response to vasodilator treatment was higher in children than in adults (41% vs. 25%). Estimated median survival in children was 4.12 years (95% confidence interval [Cl], 0.75 to 8.66) and 3.12 years in adults (95% CI 0.5 to 13.25, chi-square log-rank 0.81, p = NS). Elevated right atrial pressure (rate ratio 10.2) and decreased stroke volume index (rate ratio 32.9) were the only significant predictors of mortality (Cox proportional hazards model). Conclusions. Children with primary pulmonary hypertension have a poor survival expectancy, which does not appear to differ from that in adults with primary pulmonary hypertension. Mortality in childhood primary pulmonary hypertension is also associated with variables that assess right ventricular dysfunction.