Objective To investigate the impact of three kinds of palliative operation on the body and growth of pulmonary artery in patients with congenital heart diseases of diminutive pulmonary blood. Methods Clinical data was reviewed in 28 cases of congenital heart diseases with diminutive pulmonary blood who had been performed cavopulmonary connection （n = 9）, systemic-pulmonary shunt （n = 8 ）, and palliative reconstruction of right ventricular outflow tract （n=11）. The period between re-hospitalized and the first was 5-54 months （19.07±10. 06 months ）. Hematocrit （HCT）, hemoglobin （Hb）, percutaneous oxygen saturation （SpO2）, body surface area （BSA）, and pulmonary artery index （PAI） etc. were observed both before palliation and before the second operation. Results After the second hospitalization, there were 7 cases of death from hemorrhage, failure of circulation and extracorporeal circulation accident etc. The time of respirator, intensive care unit and total amount of dopamine in patients of palliative reconstruction of right ventricular outflow tract were longer and more than those in patients of cavopulmonary connection （P〈0. 05）. HCT, Hb before the second operation were decreased than thoes before palliative operations in all patients, SpO2, BSA and PAI increased significantly （P 〈 0. 01 ）. Before the second operation, BSA of patients with cavopulmonary connection, BSA and PAI of patients with systemic-pulmonary shunt, SpO2, BSA and PAI of patients with palliative reconstruction of right ventricular outflow tract were increased than those before palliative operations（P〈0. 01）. HCT of palliative reconstruction of right ventricular outflow tract was decreased（P〈0. 05）. Conclusion This results suggests that pulmonary blood of patients with congenital heart diseases of diminutive pulmonary blood can be increased, development of pulmonary arteries can be improved efficiently by systemic-pulmonary shunt and palliative reconstruction of right ventricle outflow tract, but it can not be found in cavopulmonary connection patients.
ObjectiveTo observe the mutation and expression of Nkx2.5 in congenital heart disease patients with diminutive pulmonary blood. We preliminarily explored the association between Nkx2.5 gene and pathogenesis of congenital heart disease patients with diminutive pulmonary blood. MethodsFifty six patients of congenital heart disease with diminutive pulmonary blood in the first affiliated hospital of Bengbu medical college and Anhui province children, s hospital between May 2012 and May 2014 were as an experimental group. Sixty three patients of ventricular septal defect were as a control group. In the trial group, there were 30 males and 26 females averagely aged 5.82± 4.23 years ranking from 6 months to 14 years. In the control group, there were 36 males and 27 females averagely aged 6.93± 4.56 years ranking from 6 months to 14 years. Before operation, peripheral venous blood of all the patients were collected. We used polymerase chain reaction combined with DNA sequencing technology to detect Nkx2.5 gene exon sequence and to analyze the association between Nkx2.5 gene mutation and congenital heart disease with diminutive pulmonary blood. And we got some hypertrophic myocardial tissue from right ventricular outflow tract in the operation, whose size was 0.5× 0.5× 0.5 cubic centimeter. And we extracted myocardial tissue RNA. The expression changes of Nkx2.5 gene mRNA were detected by real-time fluorescence quantitative polymerase chain reaction technique. ResultsThere was no mutations tested out in the peripheral venous blood in both two groups. The expression of mRNA in Nkx2.5 gene of the trial group was lower than that in the control group with a statistical difference. ConclusionNkx2.5 gene mutation may be associated with multiple factors. The occurrence of congenital heart disease with diminutive pulmonary blood may be related with a decline of Nkx2.5 gene expression in the myocardial tissue.