OBJECTIVETo observe the expression of urotensin II (UII) on the lung of patients with pulmonary hypertension (PH) with congenital heart disease and investigate the meaning of this phenomenon.

METHODThirty eight patients with CHD were divided into three groups according to pulmonary arterial systolic pressure (PASP) measured in cardiac catheterization and surgery: normal pulmonary pressure group (N group, PASP < 30 mm Hg, n = 10), mild PH group (M group, PASP ≥ 30 mm Hg, n = 15), severe or moderate PH group (S group, PASP ≥ 50 mm Hg, n = 13). The expression of UII protein and UII mRNA in pulmonary arterioles were measured separately by immunohistochemical (IHC) analysis and in situ hybridization (ISH) analysis.

RESULT(1) The results of UIIIHC staining: The UII protein expression of group M was higher than that of group N (20.22 ± 3.58 vs. 14.34 ± 2.18, P < 0.01), but less than group S (20.22 ± 3.58 vs. 28.92 ± 3.22, P < 0.05). (2) The results of UIIISH mRNA staining were similar to IHC staining, the A value of group M was higher than group N (12.51 ± 2.02 vs. 8.85 ± 1.41, P < 0.05), less than that of group S(12.51 ± 2.02 vs. 25.35 ± 4.33, P < 0.01). (3) Correlation study: there was a positive correlation between the A values of UIIIHC and pulmonary hypertension (r = 0.64, P < 0.01, n = 38), a positive correlation between the A values of UIIISH and pulmonary hypertension (r = 0.58, P < 0.01, n = 38).

CONCLUSIONThere was the expression of Urotensin II protein and mRNA in the lung of pulmonary hypertension patients with congenital heart disease, and these expression may involve the formation of pulmonary hypertension of congenital heart disease.

Adolescent ; Blood Pressure ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; complications ; metabolism ; physiopathology ; Humans ; Hypertension, Pulmonary ; etiology ; metabolism ; physiopathology ; Immunohistochemistry ; In Situ Hybridization ; Infant ; Lung ; metabolism ; physiopathology ; Male ; Pulmonary Artery ; metabolism ; physiopathology ; RNA, Messenger ; genetics ; metabolism ; Severity of Illness Index ; Urotensins ; genetics ; metabolism

OBJECTIVETo study the expression and pathological implication of transforming growth factor-1 (TGF-1) and endothelin-1 (ET-1) in intraacinar pulmonary arterioles of children with congenital heart disease and pulmonary hypertension (HP).

METHODSForty-one children with left-to-right shunt congenital heart disease were studied including 25 cases of HP (group A), 16 cases without HP (group B) and 10 children without congenital heart disease as the contols (group C). Expression of TGF-beta1 mRNA and ET-1 mRNA in intraacinar pulmonary arteriolar (IAPA) was studied using in-situ hybridization and image pattern analysis of their absorption values (A value). Changes of the intraacinar arterioles and lung tissue were studied by elastic fiber staining and electronic microscopy respectively.

RESULTS(1) There was a significant difference in the amount of intraacinar pulmonary arterioles (partial-muscular and muscular) counted in either group A or B in comparing with that of group C (F values 149.96 and 142.01 respectively, P < 0.01); (2) Electronic microscopy demonstrated endothelial proliferation of the small arteries, thickening of arteriolar wall, increased density of collagen fibers at adventitia and increased thickness of the capillary basal membrane; (3) The A value of TGF-beta1 mRNA expressed in the pulmonary arterioles of groups A and B by in-situ hybridization were 0.1988 +/- 0.0498 and 0.1098 +/- 0.0428 respectively, however, the expression was weak in group C (A value: 0.0578 +/- 0.0096). There were all significant between each two groups (F = 45.95, P < 0.01). The expression of ET-1 mRNA was markedly increased as well in the endothelial cells of pulmonary arterioles in both groups A and B, with A values of 0.1692 +/- 0.0205 and 0.1004 +/- 0.0140 respectively, whereas the expression was weak in group C (A value of 0.0746 +/- 0.0119). There were all significant between each two groups (F = 139.996, P < 0.01).

CONCLUSIONSThe number of intraacinar pulmonary partial-muscular and muscular arterioles in patients with left-to-right shunt congenital heart defect is drastically increased, along with marked restructuring of the pulmonary vasculatures. In addition, there seems a correlation present between the overexpression of TGF-beta1 mRNA and ET-1 mRNA in intraacinar pulmonary arterioles and the occurrence of pulmonary hypertension in patients with congenital heart disease.

Child ; Child, Preschool ; Endothelin-1 ; biosynthesis ; genetics ; Female ; Heart Defects, Congenital ; complications ; metabolism ; pathology ; Humans ; Hypertension, Pulmonary ; complications ; metabolism ; pathology ; Infant ; Lung ; pathology ; ultrastructure ; Male ; Pulmonary Artery ; metabolism ; pathology ; RNA, Messenger ; biosynthesis ; genetics ; Transforming Growth Factor beta1 ; biosynthesis ; genetics

OBJECTIVETo study the changes of serum leptin (LEP) and vascular endothelial growth factor (VEGF) in children with congenital heart disease(CHD) and their roles in CHD.

METHODSForty-eight children with acyanotic CHD (ACHD group), 20 age-matched children with cyanotic CHD (CCHD group) and 20 healthy children (control group) were enrolled. The ACHD group was subdivided into two groups with (n=20) or without concurrent heart failure (n=28). Serum LEP, VEGF, total protein and albumin levels and body mass index (BMI) were measured.

RESULTSSerum total protein and albumin levels were not apparently different in all CHD children from healthy controls, but there was a significant difference in the BMI between them (p<0.01). Serum LEP and VEGF levels and the ratio of LEP/BMI in all CHD children were significantly higher than those in healthy controls (p<0.01). Compared with the ACHD group without heart failure, the serum LEP and VEGF levels and the ratio of LEP/BMI in the CCHD and the ACHD with heart failure groups increased significantly (p<0.01). In the ACHD group, serum LEP level was positively correlated with BMI (p<0.01). In the CCHD group, there were positive correlations between serum LEP level and serum VEGF level (p<0.01) and between hemoglobin concentration and serum VEGF level (p<0.01). Arterial oxygen saturation was negatively correlated with serum VEGF (p<0.01) and LEP levels (p<0.01) in the CCHD group.

CONCLUSIONSBoth VEGF and LEP play roles in the pathophisiological process of CHD. VEGF and LEP are associated with the development of heart failure in children with ACHD.

Body Mass Index ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; blood ; Hemoglobins ; analysis ; Humans ; Infant ; Leptin ; blood ; Male ; Oxygen ; blood ; Vascular Endothelial Growth Factor A ; blood