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.
Objective To investigate the value of systemic-normothermic/cardiac-hypothermic cardiopulmonary bypass（CPB）on operation of congenital heart disease. Methods Thirty patients of congenital heart disease were randomly divided into two groups, the normothermia group（n=15）and hypothermia group（n=15）. The changes of CPB time, aortic cross-clamp time,operation time and postoperative drainage and the value of blood cell were observed. Results The duration of CPB （37. 5 ±11. 6rain vs. 51. 6± 12. 0 min, P〈0. 05） and operation time （2.2± 0.6h vs. 2. 7±0. 5h, P〈0. 01） in normothermia group were shorter than those of hypothermia group statistically, the differences of postoperative drainage and the value of blood cells between two groups were not statistically significant. Conclusion The use of systemic-normothermic/cardiac-hypothermic CPB on operation of congenital heart disease shows that the time of operation is shorter remarkly , and it could be clinically used safely.
Abstract： Objective To evaluate clinical outcomes of painless flexible fiberoptic bronchoscopy in the treatment for pulmonary complications in postoperative pediatric patients with congenital heart diseases. Methods We retrospectively analyzed clinical data of 58 patients who received fiberoptic bronchoscopy for pulmonary complications after surgical repair for atrial septal defect， ventricular septal defect， tetralogy of Fallot， double outlet right ventricle or transposition of the great arteries in First Affiliated Hospital of Harbin Medical University From August 2009 to February 2012. There were 26 male patients and 32 female patients with their age ranging from 20 days to 2 years. Olympus xp-60 fiberoptic bronchoscopy was used for removal of airway secretions and bronchial alveolar lavage under anesthesia with propofol， fentanyl and lidocaine. Pulse oximetry， respiratory sound and chest X-ray changes were observed. Results The examination time of painless fiberoptic bronchoscopy was 10-25 minutes in the 58 patients. After removal of airway secretions by fiberoptic bronchoscopy， their respiratory status improved significantly， pulse oxygen saturation increased by 5%-12%， and their pulmonary crackles were significantly reduced. In 29 patients with atelectasis， their pulmonary lobes demonstrated significant reexpansion in chest X-ray reviews on the next day after fiberoptic bronchoscopy， and 8 patients received another fiberoptic bronchoscopy treatment on the next day to achieve complete pulmonary reexpansion. One patient with severe pulmonary hypertension （PH） stayed in intensive care unit （ICU） for 6 days， 3 patients with transposition of the great arteries stayed in ICU for 5 days， 3 patients with double outlet of right ventricle and moderately high PH stayed in ICU for 4 days， and all the other patients were discharged from ICU within 48 hours after admission. There was no severe complication related to fiberoptic bronchoscopy， except 2 patients with minor nasal mucosa bleeding who were cured with hemostatic drugs and local compression. Conclusion Painless flexible fiberoptic bronchoscopy can significantly enhance clinical outcomes and shorten ICU stay for postoperative pediatric patients with congenital heart diseases.
ObjectiveTo explore the natural changes of procalcitonin (PCT) in the early period after pediatric cardiac surgery with cardiopulmonary bypass (CPB).MethodsA prospective and observational study was done on patients below 3 years of age, who underwent cardiac surgery involving CPB, with the risk adjustment of congenital heart surgery (RACHS) score of 2 to 5 and free from active preoperative infection or inflammatory disease. Blood samples for measurement of PCT, C-reactive protein (CRP) and white blood cell (WBC) were taken before surgery and daily for 7 days in postoperative period. Infections and complications within 7 days after operation were investigated. According to the presence or absence of infection and complications within 7 days after operation, the enrolled children were divided into an infection+complications group, a simple infection group, a simple complication group, and a normal group.Results Finally, 429 children with PICU stay≥ 4 days were enrolled, including 268 males and 161 females, with a median age of 8.0 (0.7, 26.0) months. There were 145 children in the simple infection group, 38 children in the simple complication group, 230 children in the normal group and 16 children in the infection+complications group. The levels of PCT, CRP and WBC were significantly higher after CPB. CRP and WBC peaked on the second postoperative day (POD) and remained higher than normal until POD7. PCT peaked on POD1 and would generally decrease to normal on POD5 if without infection and complications. Age, body weight, RACHS scores, the duration of CPB and aortic cross-clamping time were correlated with PCT level. There was a statistical difference in PCT concentration between the simple infection group and the normal group on POD 3-7 (P<0.01) and a statistical difference between the simple complication group and the normal group on POD 1-7 (P<0.01). A statistical difference was found between the simple infection group and the simple complication group in PCT on POD 1-5 (P<0.05).ConclusionWBC, CRP and PCT significantly increase after CPB in pediatric cardiac surgery patients. The factors influencing PCT concentration include age, weight, RACHS scores, CPB and aortic cross-clamping time, infection and complications.
Abstract: Objective To study the pathophysiological mechanism of the morphological change of immature pulmonary vessels in the piglet model of congenital heart defect with decreased pulmonary blood flow established with balloon atrial septostomy and pulmonary artery banding. Methods Twenty piglets at an age of one to two months were divided into three groups with random number table. For the control group (group C,n=6), small incisions were carried out on the right chest to produce a transient reduction in the pulmonary blood; for the lowmedium pulmonary artery stenosis group (group T1, n=7), the balloon dilator was delivered through the surface of the right atrium and septostomy and pulmonary artery banding were performed, and the systolic transpulmonary artery banding pressure (Trans-PABP) was controlled to be 20.30 mm Hg; For the severe pulmonary artery stenosis group (group T2, n=7), the same surgical procedures with group T1 were performed while TransPABP was controlled to be more [CM(159mm]than 3050 mm Hg.At 2 months after surgery respectively,a lung tissue of 1.0 cm×0.8 cm×0.8 cm from the lateral segment of the right middle lobe was taken out to be observed under optic microscope. The morphological change of the distal arterioles was detected. Furthermore, the content of vascular endothelial growth factor (VEGF) and matrix metalloproteinase2( MMP2) were also examined by the method of enzymelinked immunosorbent assay (ELISA). Results The model was successfully established in all the survival piglets of the group T1 and group T2. Two months after operation, the inner diameter of the pulmonary arterioles in group T1 was significantly higher than that in group C (82.89±10.72 μm vs.74.12±9.28 μm;t=－5.892, Plt;0.05), so as group T2 (85.47±5.25 μm vs.74.12±9.28 μm;t=-6.325, Plt;0.05); the number of arterioles per square centimeter (NAPSC) of group T1 was significantly lower than that of the group C (229.70±88.00 entries/cm 2 vs. 431.50±40.60 entries/cm2; t=39.526, Plt;0.05), so as group T2 (210.00±40.30 entries/cm2 vs. 431.50±40.60 entries/cm2; t=67.858, Plt;0.05). Two months after operation, the lung expression of MMP -2 and VEGF in group T1 was significantly lower than that in group C (58.30±19.60 ng/ml vs. 81.20±16.70 ng/ml, t=14.261, Plt;0.05; 17.80±3.00 pg/ml vs. 21.40±3.80 pg/ml, t=8.482, P＜0.05), so does group T2 (42.10±15.20 ng/ml vs. 81.20±16.70 ng/ml, t=27.318, P＜0.05; 12.30±3.20 pg/ml vs. 21.40±3.80 pg/ml, t=15.139, P＜0.05). Conclusion Structural remodeling of pulmonary extracellular matrix is an important feature of the piglet model of congenital heart defect with decreased pulmonary blood flow. The arterioles show significant hypoplasia or degradation. Change in the structural proteins and cytokines during the reduction of blood in the lung is the key to structural remodeling.
Congenital coronary artery anomalies include various congenital anatomic anomalies of coronary artery. Severe congenital coronary artery anomalies usually result in ventricular dysfunction, myocardium ischemia, and even sudden death. Hence, understanding the coronary artery anomalies is critically important for the related surgeons to make reasonable strategies. This consensus is based on current literature and opinions of Chinese experts. And we mainly discuss the anatomy, clinical manifestation, diagnosis and treatment of important congenital coronary artery anomalies in clinics including anomalous aortic origin of a coronary artery, anomalous origin of the coronary artery from the pulmonary artery and coronary fistula.
ObjectiveTo summarize surgical treatment experience on unruptured aneurysm of the sinus of Valsalva (SVA). MethodsClinical data of 33 patients with unruptured SVA underwent surgical repair at Fu wai Hospital between February 2007 and January 2012 were retrospectively analyzed. There were 27 males and 6 females with their mean age of 28.5±13.5 years (ranged from 4.5 to 58.0 years). The unruptured SVA originated in the right coronary sinus in 29 cases (87.8%), in non-coronary in 2 cases (6.1%) and in left coronary sinus in 2 cases (6.1%). There were 29 cases with ventricular septal defect and 20 cases with aortic valve insufficiency (AI). ResultsOnly 52.8% of unruptured SVA were correctly diagnosed by echocardiography preoperatively. All unruptured SVA received active surgical management, with no early death after operation. Thirty patients (90.9%) were followed up for 22 to 81 months (mean 42.9±18.8 months) and in NYHA classⅠorⅡ. None SVA recurrence occurred. Postoperative AI could be improved better by using the repair route only through the chamber of right ventricle for patients with unruptured SVA originated in right coronary sinus. ConclusionFor unruptured SVA patients who associated with other kinds of cardiovascular lesions, active surgical repair for unruptured SVA can achieve satisfactory results.
Congenital heart disease (CHD) is a birth defect with the highest incidence in China. In September 2020, Annual Report on Cardiovascular Health and Diseases in China (2019) was published by National Center for Cardiovascular Diseases. The present situation of prevention and treatment of CHD was briefly summarized in this report. In this paper, we presented the main opinions of Annual Report on Cardiovascular Health and Diseases in China (2019), supplied with relevant data, such as epidemiology, surgical treatment and interventional treatment of CHD, to further expand and explain this report.
The consensus was authored by National Society of Congenital Heart Diseases. After employing the Delphi process and incorporating literature reviews and expert discussions, seven recommendations were ultimately formulated. The consensus provides a detailed elaboration on the pathoanatomy, pathophysiology, clinical manifestations, diagnostic methods, and surgical treatment approaches for aortic valve diseases in children. It emphasizes that the treatment of aortic valve diseases in children should take into account the needs of growth and development, and recommends surgical strategies for different age groups and types of lesions, including valve plasty, Ross procedure, valve replacement, and balloon dilation. Specifically, aortic valve plasty is recommended for neonates and infants, while surgical options for older children are more diversified. The consensus only discusses isolated aortic valve disease and does not cover cases complicated with other heart malformations.
ObjectiveTo compare the anti-apoptotic potency of human mesenchymal stem cells (hMSCs) derived from patients with cyanotic congenital heart diseases (C-CHD) or acyanotic congenital heart diseases (A-CHD) in vitro and explore the possible mechanism. MethodshMSCs were isolated from patients with cyanotic (Group C) or acyanotic (Group A) congenital heart diseases and cultured in a hypoxic incubator (1% O2, 5% CO2, 94% N2) in vitro. The anti-apoptotic potency of the hMSCs was assayed by the Annexin V-FITC/PI double labeled flow cytometry. The content of B-cell lymphoma-2 (Bcl-2), Bax and caspase-3 in both groups was determined by Western blot. ResultsFlow cytometry results revealed that hMSCs from C-CHD patients presented higher level of resistance to ischemia-and anoxia-induced apoptosis with lower overall (P<0.05) and early apoptosis ratio (P<0.01). Further Western blot examination identified that C-CHD-derived hMSCs produced more Bcl-2 (P<0.05) but less Bax (P<0.05) and caspase-3 (P<0.05) in comparison to their A-CHD-derived ones. ConclusionC-CHD-derived hMSCs presented the superiority for the anti-apoptotic potential, and the possible mechanism is the favorable change of Bcl-2, Bax and caspase-3 induced by the natural hypoxic and anoxic precondition.