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.
Objective To introduce a modified REV procedure of complicated transposition of the great arteries （TGA） or double outlet right ventricle （DORV） which was combined with ventrieular septal defect （VSD） and pulmonary valve stenosis（PS）. Methods From Sep. 2005 to Feb. 2006, 3 children with complicated transposition of the great arteries underwent a modified REV operation. This modified REV operation was designed on the basis of classical REV procedure to preserve the native pulmonary artery valve and its function. Results Two patients recovered uneventfully but one died after extraeorporeal membrane oxygenator （ECMO） treatment. After 4 and 1 months follow-up respectively, the discharged 2 patients were asymptomatie and the eehoeardiography revealed that the pressure gradient between left ventrieular-main pulmonary were estimated to be 15 and 5mmHg. Conclusion This modified REV operation for preservation of pulmonary artery valve is an ideal procedure to complicated transposition of the great arteries. Advantages and disadvantages of this modified REV procedure were discussed.
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.
ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.
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.