Relation of Macrophage Migration Inhibitory Factor to Pulmonary Hemodynamics and Vascular Structure and Carbamyl-Phosphate Synthetase I Genetic Variations in Pediatric Patients with Congenital Cardiac Shunts

Figure 5

(a) Relation of medial hypertrophy of intra-acinar pulmonary arteries to the circulating (serum) levels of MIF chemokine. The data correspond to patients with high-PVR clinical profile and AC CPSI T1405N genotype for whom lung biopsy specimens could be collected intraoperatively (no significant association was seen in subjects with the CC genotype). These patients were characterized by high levels of serum MIF (i.e., above 7000 upi). The magnitude of wall thickness was similar between intra-acinar arteries () but varied across MIF levels (). The regression equations for arteries accompanying the respiratory bronchioles and alveolar ducts were  + 1.81E-3x (), and  + 1.48E-3x (), respectively. (b) Pulmonary vascular response to inhaled nitric oxide (NO) in the same patients. The level of pulmonary vascular resistance at 10 minutes of NO inhalation is shown as pulmonary-to-systemic vascular resistance ratio (PVR/SVR). Subjects with higher levels of MIF (the ones with higher degree of arteriolar hypertrophy) had a more pronounced response ( – 6.98E-5x (), ). No significant correlation was seen in patients with the CC genotype.

(a)

(b)