World journal for pediatric & congenital heart surgery
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World J Pediatr Congenit Heart Surg · Jan 2014
Case ReportsResuscitation of a neonate with medium chain acyl-coenzyme a dehydrogenase deficiency using extracorporeal life support.
We report a neonate with medium chain acyl-coenzyme A dehydrogenase deficiency (MCAD) who had a cardiac arrest due to ventricular tachycardia and fibrillation. Extracorporeal life support (ECLS) was deployed, from which the baby was subsequently separated and discharged from hospital. This case was a rare neonatal presentation of MCAD and an uncommon indication for ECLS. We discuss the presentations of patients with MCAD and the use of ECLS for patients with possible inborn errors of metabolism and other unknown primary diagnoses.
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World J Pediatr Congenit Heart Surg · Jan 2014
Case ReportsVideo fluoroscopy swallow study and nutritional support during ambulatory venovenous extracorporeal membrane oxygenation as a bridge to lung transplantation.
We present the successful completion of a video fluoroscopy swallow study and subsequent nutritional plan of a child bridged to lung transplantation with ambulatory venovenous (VV) extracorporeal membrane oxygenation (ECMO). With a limited number of programs bridging pediatric patients to lung transplantation with VV ECMO, a better understanding of nutritional support is needed to provide optimal care to this patient population awaiting organ donation.
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World J Pediatr Congenit Heart Surg · Jan 2014
Observational StudyDoes single ventricle physiology affect survival of children requiring extracorporeal membrane oxygenation support following cardiac surgery?
Improved survival with postoperative extracorporeal membrane oxygenation (ECMO) has expanded its application to children with single ventricle (SV) anomalies. We examine current-era outcomes of postoperative ECMO with special focus on patients with SV. ⋯ The ECMO is valuable in patients with SV however results depend on anatomy, procedure, and support indication. Persistent markers of poor perfusion, end-organ injury, and prolonged ECMO duration are associated with mortality. Those factors could be modified by early ECMO application before organ damage, meticulous homeostasis to ensure adequate perfusion, early diagnosis, and reoperation on residual lesions to expedite weaning.
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World J Pediatr Congenit Heart Surg · Oct 2013
Case ReportsSecondary repair of Ebstein's anomaly with atrioventricular septal defect using the cone technique.
A nine-year-old boy with incomplete atrioventricular septal defect (AVSD) and Ebstein's anomaly had undergone a closure of primum atrial septal defect and repair of right and left AV valves at four years of age. He presented with severe regurgitation of the right AV valve. ⋯ The association of Ebstein's anomaly with AVSD has been reported only rarely. We present a case of this association treated with the previously unreported application of the cone reconstruction (da Silva repair) as a secondary strategy.
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World J Pediatr Congenit Heart Surg · Oct 2013
Multicenter StudyFunctionally univentricular heart and the fontan operation: lessons learned about patterns of practice and outcomes from the congenital heart surgery databases of the European association for cardio-thoracic surgery and the society of thoracic surgeons.
"The term "functionally univentricular heart" describes a spectrum of congenital cardiovascular malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation and another to the pulmonary circulation." The purpose of this article is to review patterns of practice and outcomes in the Congenital Heart Surgery Databases (CHSDBs) of the European Association for Cardio-Thoracic Surgery (EACTS) and the Society of Thoracic Surgeons (STS) in patients with functionally univentricular hearts undergoing the Fontan operation. ⋯ The STS database is largest CHSDB in North America. The EACTS database is largest CHSDB in Europe. This review of data from EACTS and STS allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that patients with functionally univentricular hearts present a challenging problem; however, exclusive of Fontan revision or conversion (Re-do Fontan), the Fontan operation has a discharge mortality of 2.3%.