Pulmonary arterial endothelial and smooth muscle cell homeostasis is regulated through the bone morphogenetic protein (BMP) and transforming growth factor beta (TGF-β) receptor pathways. Pathway imbalance results in pulmonary hypertension (PH). Each pathway has ligands and modulators influencing this balance. How these pathways differ in pediatric PH patients is unknown. Ten PH and 20 control subjects (ages 2-17 years) were prospectively enrolled. Pulmonary artery serum BMP 2, 4, 6, 7, 9, 10, activin A, TGF-β1, carboxyl terminus of Hsc70-interating protein (CHIP), NT Pro BNP, and CRP were measured by ELISA. Analyses were made using the Fisher's exact test, the Mann-Whitney test, ROC analysis, and Pearson and Spearman correlations as appropriate. PH subjects were group 1 (four with simple shunts) or group 3 PH. Control subjects had shunts scheduled for catheter closure but no PH. Only BMP 7 and CHIP levels were statistically elevated in PH patients versus controls
(BMP 7 0.081(0.076-0.084) vs. 0.074(0.069-0.08) OD,