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Background Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. Methods Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3). Results All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years). Conclusions Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.

Genetic syndromes, especially 22q11 deletion (del22q11) syndrome, are common in patients with pulmonary atresia and ventricular septal defect (PA-VSD), but their association with long-term outcomes varies. The purpose of this study was to evaluate the long-term outcome after complete repair of PA-VSD and to determine the impact of genetic syndromes.We reviewed our experience of 125 patients with PA-VSD who received primary or staged repair between 1978 and 2010. Evaluations for genetic syndromes included clinical features, cytogenetic analysis, and fluorescence in situ hybridization or multiplex ligation-dependent probe amplification.Genetic syndromes were documented in 26 patients (20.8%), including del22q11 in 16 patients, trisomy 21 in 2 patients, and other syndromes in 8 patients. The prevalence of hypoplastic pulmonary arteries was not significantly different between the syndromic and nonsyndromic groups. After 1,069 patient-years of follow-up, 20-year survival was 90% ± 6% in patients without syndromes and 14% ± 23% in patients with syndromes (p0.01). Multivariate analysis identified the presence of a genetic syndrome as an important risk factor for hospital and late mortality. Subgroup analysis showed that genetic syndromes other than del22q11 were associated with worse outcome. The rate of 10-year freedom from cardiac reintervention after repair was 53% ± 11%, with hypoplastic pulmonary arteries before repair as a major risk factor (p = 0.02).Genetic syndromes significantly affect survival after repair of PA-VSD, whereas genetic syndromes do not represent additional risk for reintervention. Repair is feasible in patients with syndromes, but suboptimal long-term outcome should be addressed when counseling parents.

Pulmonary regurgitation after repair of tetralogy of Fallot has always been an important issue for pediatric cardiac surgeons. Some of the patients may need pulmonary valve replacement in the future. Conventional prosthesis valves were seldom custom made for the pulmonary valve and were often inadequate to use because of variable age and size of these patients. Through evolution of techniques, we have developed a simple, efficient, and theoretically anatomic way of constructing trileaflet polytetrafluoroethylene valved conduit used for right ventricle outflow tract reconstruction. It was surprisingly straightforward, time efficient, and capable of being produced in various sizes.

Background We retrospectively reviewed cases of left pulmonary artery (LPA) sling complex with computed tomography to explore its possible meaning in embryogenesis. Methods Eighteen children (6 girls and 12 boys aged 5 days to 17.5 years; mean, 1.5 years) were identified as having LPA sling during the period of October 1996 to March 2006. Another 2,364 cardiac computed tomography scans that had no LPA sling were included as control. The associated bronchopulmonary and vascular anomalies were identified. Chi-square tests were used to compare the probabilities of coexistence of these anomalies, with or without LPA sling. Results All patients with LPA sling presented with tracheal stenosis (100%), and there was a high incidence of combined right tracheal bronchus (22%), underdeveloped right lung (22%), persistent left superior vena cava (22%), and left patent ductus arteriosus (39%). These associated anomalies were more highly associated with subjects who had LPA sling than with those who did not (p Conclusions Owing to the spatial coexistence of LPA sling with the associated anomalies in the embryonic foregut mesoderm, we propose that a "space available" hypothesis in the local environment appears to be important in the embryogenesis of an LPA sling complex.

A 30-year-old man who is a heroin addict was diagnosed with uncontrolled tricuspid valve endocarditis and repeated lung abscesses. He underwent tricuspid valvectomy for the endocarditis. After surgery the patient had severe tricuspid regurgitation and hypoxemia develop. Due to severe tricuspid regurgitation-induced ventricular distension and persistent low cardiac output, reimplantation of the tricuspid valve was planned for 2 weeks after the first operation. To avoid lung injury caused by the cardiopulmonary bypass and to preserve right ventricular function, a self-made superior and inferior vena cava shunt was connected to the pulmonary artery. The tricuspid valve was implanted without cardiopulmonary bypass.

Background B-type natriuretic peptide (BNP) is a marker of heart failure. In adult patients with heart failure, decreased BNP levels after implantation of ventricular assist devices may be indicative of recovery. However, BNP levels among pediatric patients receiving mechanical support are largely unknown. Methods Fifteen pediatric patients with cardiogenic shock who were supported by extracorporeal membrane oxygenation (ECMO) were evaluated. The BNP levels were determined before ECMO initiation, during ECMO support, and after ECMO removal. Results All patients had elevated BNP levels before initiation of ECMO (median, 1,430 pg/mL; range, 361 to 5,000 pg/mL). Among the 15 patients, 1 received heart transplantation. Extracorporeal membrane oxygenation was withdrawn in 2 patients, and the other 12 patients were weaned from ECMO. Four patients died after initially successful weaning from ECMO. The BNP levels of the nonsurvivors (median, 3,685 pg/mL; range, 2,494 to 5,000 pg/mL) were higher than those of the survivors (median, 1,127pg/mL; range, 108 to 3,030 pg/mL) on the next few days after ECMO removal ( p = 0.016). The BNP levels on the fourth day after removal of ECMO among the survivors (median, 498 pg/mL; range, 108 to 890 pg/mL) were lower than those among the nonsurvivors (median, 3,900 pg/mL; range, 3,230 to 5,000 pg/mL; p = 0.017). Conclusions Among pediatric patients supported with ECMO, the survivors had lower BNP levels than those who did not survive. We suggest that serial blood BNP levels can be potential markers for monitoring pediatric patients on mechanical circulatory support, and the concept merits further study.

Background Aortic valve prolapse is frequently associated with juxta-arterial ventricular septal defect. The significance of its association with other outlet types of ventricular septal defect, however, remains unclear. Methods From 1987 to 2002, 677 patients (male:female ratio, 424:253) who received surgical repair for ventricular septal defect extending to the outlet septum were reviewed. Based on surgical findings, ventricular septal defects were classified as juxta-arterial, perimembranous outlet, or muscular outlet type. Results Aortic valve prolapse occurred in 373 of 677 patients (57.2%) with 209 juxta-arterial, 103 perimembranous outlet, and 61 muscular outlet type. Significant aortic regurgitation developed in 51 of 373 (14%). Among 252 patients with regular follow-up, the mean onset ages of aortic valve prolapse in juxta-arterial, perimembranous outlet, and muscular outlet type were 4.9, 5.0, and 5.1 years, respectively (no statistical difference). The presence of larger shunt and probably anterior malalignment predicted an earlier onset of aortic valve prolapse. Perimembranous outlet and muscular outlet type ventricular septal defect were frequently associated with infundibular hypertrophy and subaortic ridge, and perimembranous outlet type was associated with anterior septal malalignment. In juxta-arterial ventricular septal defect and ventricular septal defect with anterior malalignment, prolapsed cusp was always the right coronary cusp, but noncoronary cusp involvement was also common in perimembranous outlet type (17 of 103, 16.5%). Conclusions The association with anterior septal malalignment, infundibular stenosis and subaortic ridge is related to the location of the outlet ventricular septal defect. The age of onset of aortic valve prolapse in each type was quite similar, and a larger shunt may predict an earlier onset.

Background. Conventional and modified ultrafiltration was used in pediatric cardiac operations to reduce volume overload and total body water. The purpose of this study was to compare the efficacy of these techniques in removing inflammatory mediators during cardiopulmonary bypass. Methods. Fifty pediatric patients undergoing cardiac operations were randomized into a modified or conventional ultrafiltration group. Blood samples were obtained before and after ultrafiltration to assess the plasma concentrations of leukocyte elastase, tumor necrosis factor- α , interleukin-6, and interleukin-8. Results. Except for plasma concentrations of tumor necrosis factor- α in the modified ultrafiltration group, the plasma concentrations of all the mediators measured increased after ultrafiltration in both groups of patients. The volume of ultrafiltrate and the total amounts of tumor necrosis factor- α and interleukin-6 removed by ultrafiltration were significantly greater in the modified group. The concentrations of mediators in the ultrafiltrate and the ratio of ultrafiltrate to plasma concentrations of the mediators did not differ between the groups. Ultrafiltration was more efficient in removing tumor necrosis factor- α than the other mediators. Conclusions. The efficacy in removing the inflammatory mediators generated during cardiopulmonary bypass did not differ between modified and conventional ultrafiltration.