Circulating Immunoreactive Endothelin-1 in Children with Pulmonary Hypertension: Association with Acute Hypoxic Pulmonary Vasoreactivity

摘要: To determine whether circulating levels of endothelin-1 (ET-1), a potent vasoconstrictor peptide, are elevated in children with pulmonary hypertension and related to the degree hypoxic vasoconstriction, we measured arterial mixed venous plasma concentrations immunoreactive ET-1 (irET-1) 13 during cardiac catheterization. Clinical diagnoses seven (PH) included chronic lung disease (four children), congenital heart after surgical repair (two primary ("reactive") (one child). Blood samples were simultaneously obtained from artery (venous) systemic sites baseline conditions. Plasma irET-1 was PH (12.3 +/- 3.4 versus 3.6 0.7 pg/ml, non-PH; p < 0.01). Arterial/venous ratios group (1.1 0.2) not different those non-PH group. During acute hypoxia, mean Ppa increased 27 3 40 5 mm Hg. Basal correlated strongly elevation hypoxia (r = 0.69; 0.02). We conclude that often PH, they vasoreactivity hypoxia. Whether contribute directly or markers altered vascular tone reactivity remains speculative.