Objective To summarize the experience of open heart operation on neonates with critical and complex congenital heart diseases and evaluate the methods of perioperative management. Methods From May 2001 to January 2003, 12 patients of neonates with congenital heart diseases underwent emergency operation. Their operating ages ranged from 6 to 30 days, the body weights were 2.8 to 4.5 kg. Their diagnoses included D-transposition of the great arteries in 4 cases, ventricular septal defect with atrial septal defect in 5 cases, complete atrioventricular septal defect, obstructed supracardiac total anomalous pulmonary venous drainage and cardiac rhabdomyomas in 1 case respectively. 12 cases were operated under moderate or deep hypothermic cardiopulmonary bypass. Results All cases were observed in ICU for 2-11 days and discharged 7-19 days after operation. The postoperative complications included low cardiac output, mediastinal infection, respiratory distress syndrome, systemic capillary leak syndrome and acute renal failure. All cases were cured and the follow-up (from 6 months to 2 years) showed satisfactory outcome. Conclusion A particular cardiopulmonary bypass and proper perioperative management is very important to ensure the successful outcome. Peritoneal dialysis is an effective and safe method for treating acute renal failure after cardiac operation in neonates.
Abstract: Eisenmenger’s syndrome is a kind of pathophysiologic syndrome that occurs in the end stage of congenital heart disease. The diagnosis of Eisenmenger’s syndrome is clear, but its early clinical presentations are nonspecific and its prognosis is poor. Recently, the therapeutic regimen is steadily improved by the endless efforts that lots of scientists have made. Treatments including new drugs, modified surgery, extracorporeal membrane oxygenation, transplantation and gene therapy can not only relieve symptoms, correct abnormal pulmonary hemodynamics, but also can improve prognosis to some extent. We will review and introduce the advance of research on Eisenmenger’s syndrome, including its pathogenesis, diagnosis and treatment.
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.
Abstract: Objective To summarize the immediate effects and the near and midterm followup results of transthoracic balloon valvuloplasty for newborns and infants with severe and critical pulmonary valve stenosis to find out an effective plan for onestop balloon valvuloplasty. Methods From March 2006 to March 2010, 32 patients including 23 males and 9 females with severe and critical pulmonary valve stenosis were treated in Fu Wai Hospital. Their age ranged from 5 days to 11 months (4.59±3.21 months). Weight of the patients ranged from 2.3 to 10.5 kg (6.48±2.05 kg). Dilatation was performed under general anesthesia with intubation and the guidance of echocardiography. During the follow-up period, all survivors had serial echocardiographic assessment to measure the transpulmonary pressure gradient (TPG) and the degree of pulmonary regurgitation. Results All operations were successful with no severe postoperative complications. Hemodynamic indexes were stable after operation with TPG lowered from from 82±27 mm Hg preoperatively to 23±12 mm Hg postoperatively (t=15.28, Plt;0.05). Only 4 patients had a TPG of more than 40 mm Hg on echocardiography before leaving the hospital. Tricuspid regurgitation was decreased significantly with 17 cases of nonregurgitation, 13 cases of light regurgitation and 2 cases of moderate regurgitation. Saturation of peripheral oxygen in all the patients increased to higher than 95%. Followup time ranged from 1 month to 4 years (16±11 months). The results of the follow-up were satisfying for all the patients. The average TPG was 17±10 mm Hg with only one above 40 mm Hg. Pulmonary valve regurgitation was found in 24 patients including 23 with light pulmonary regurgitation and 1 with moderate regurgitation. Conclusion Transthoracic balloon valvuloplasty for newborns and infants with severe and critical pulmonary valve stenosis is safe and effective.
Objective To investigate the iron deficiency (ID) in children with congenital heart disease (CHD) and find high risk factors of ID. Methods The clinical data of 227 pediatric patients with CHD from February to June 2016 were retrospectively analyzed. The incidence of ID according to the result of iron metabolism examination (serum ferritin <12 μg/L as the diagnostic criteria) was investigated. According to their basic CHD types, patients were divided into a cyanotic group and an acyanotic group. We tried to find the high risk factors of ID in those pediatric CHD patients by comparing their age, gender, growth condition and blood routine test results. Results There were 19.8% pediatric CHD patients complicated by ID. The incidence of ID in the cyanotic patients was higher than that in the acyanotic patients (31.0% vs. 17.3%, P=0.045). In both groups, ID patients presented the characteristics of younger age, higher anemia rate, lower mean corpuscular volume (MCV), lower mean corpuscular hemoglobin (MCH), lower mean corpuscular-hemoglobin concentration (MCHC) and longer red blood cell distribution width (RDW). Conclusion Cyanosis, younger age (infant), anemia, decreased MCV, decreased MCH, decreased MCHC and increased RDW are high risk factors of ID in CHD children.
Objective To compare the long-term results between theMckay procedure and the musclestrength balancing procedure in treatment of congenital clubfoot (CCF).Methods Thirty-seven children with 54 clubfeet were treated by the muscle-strength balancing procedure (31 feet) or the Mckay procedure (23 feet).There were 27 males (38 feet) and 10 females (16 feet). The average age at the time of surgery was 1.2 years (range, 5 months to 3.5 years). The deformity occurred on the left side in 7 patients, on the right side in 13, and on both sides in 17. During the musclestrength balancing procedure, the anterior tibial tendonwas transplanted to the middle or the lateral cuneiform, and the Achilles tendon was lengthened. During the Mckay procedure, the complete releasing of the softtissues and the lengthening of the tendons were performed routinely; in addition, the abductor hallucis was also excised. The clinical outcomes were evaluated with the Diméglio classification method. According to the Diméglio scoring system, 3 clubfeet were at Grade Ⅱ (score, 6-10); 26 clubfeet at Grade Ⅲ (score, 11-15); 25 clubfeet at Grade Ⅳ (score, 16-20). Based on the Diméglio grading system, all the patients were divided into two groups before operation. Group Aconsisted of 29 feet at Grade Ⅱ or Ⅲ (score, 12.55±1.84); Group B consistedof 25 feet at Grade Ⅳ (score, 17.20±1.08). The score in the group undergoingthe musclestrength balancing procedure was 14.16±2.83, and the score in the group undergoing the Mckay procedure was 15.43±2.63. Results All the patients were followed up for an average of 8.2 years (range, 5.0-10.5 years). According to the Diméglio grading system, 32 patients were at Grade Ⅰand 22 patients at Grade Ⅱ, and none of the patients at Grade Ⅲ or Ⅳ. Two patients undergoing the Mckay procedure developed the postoperative incision infection, but the incision wound healed after the dressing changes. The Diméglio score was 4.07±1.25 in Group A and 6.52±1.74 in Group B after operation, with a significant difference when compared with before operation (Plt;0.05). In Group A the two procedureshad no significant difference in effectiveness (Pgt;0.05); however, in Group B they had a significant difference (Plt;0.05). Judging by the correction degrees for the deformity on the different planes, the two procedures had no significant difference for correcting the equinus of hind foot (Pgt;0.05); however, in the correction degrees for the cross-foot and supination or adduction of the anterior foot, the Mckay procedure was significantly finer than the muscle-strength balancing procedure. It has a good biocompatibility. The mechanical test has showed that the Mckay procedure had the best result in the correction of the forefoot adduction. Conclusion For treatment of congenital clubfoot at Grades Ⅰ-Ⅲ, the musclestrength balancing procedure can achieve an excellent correction result; for treatment of congenital clubfoot at Grade Ⅳ, the Mckay procedure should be performed. No matter whichprocedure, the abductor hallucis excision is recommended to prevent poor correction for the anterior foot adduction.
In 1984, according to the criteria of the classifieation for congenital hand deformity which wasput out by the International Hand Surgery Committee, we had made an investigation for congenitalhand deformity among 318066 newborns in Shanghai. It was found that the inctdence of a congenitalhand malformation was 0. 0808 percent among the total newborns. The congenital malformation ofthe thumb was 37. 74 percent of all deformities of the hand. According to the statistical analysis, we ...
ObjectiveTo explore the prognostic value of serum cystatin C (Cys C) in patients with congenital heart disease-associated pulmonary arterial hypertension (PAH-CHD).MethodsA retrospective cohort study was conducted on adult PAH-CHD patients who were hospitalized for the first time in the First Affiliated Hospital of Xinjiang Medical University from January 2010 to January 2020. The serum Cys C and other related data of patients were collected. The median follow-up time was 57 months. The main end event was all-cause death. According to the prognosis, the patients were divided into a survival group and a death group. Cox regression was used to analyze the risk factors for all-cause death in patients with PAH-CHD.ResultsA total of 456 patients were enrolled, including 160 males and 296 females, aged 38.99±14.72 years. The baseline data showed that there were statistical differences in resting heart rate, serum Cys C, creatinine, NT-proB-type natriuretic peptide (NT-proBNP), high-sensitivity cardiac troponin T (hs-cTnT), high-sensitivity C reactive protein (hs-CRP), New York Heart Association (NYHA) cardiac function classification and serum potassium between the survival group and the death group. Univariate Cox regression analysis showed that serum Cys C, NT-proBNP, hs-cTnT, creatinine and NYHA cardiac function classification were related risk factors for all-cause death in patients with PAH-CHD. Multivariate Cox regression analysis showed that serum Cys C (HR=3.820, 95%CI 2.053-7.108, P<0.001), NYHA grade Ⅲ (HR=2.234, 95%CI 1.316-3.521, P=0.010), NYHA grade Ⅳ (HR=4.037, 95%CI 1.899-7.810, P=0.002) and NT-proBNP (HR=1.026, 95%CI 1.013-1.039, P<0.001) were independent risk factors for all-cause death in patients with PAH-CHD and had a good predictive value.ConclusionAs a new cardiac marker, serum Cys C can predict all-cause death in patients with PAH-CHD and is an independent risk factor.