Congenital heart disease
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Congenital heart disease · Jul 2011
Impact of pharmacotherapy on interstage outcomes in single ventricle infants.
To characterize the pharmacotherapeutic regimens used in infants with single ventricle heart disease and determine the influence of outpatient medications on interstage weight gain. ⋯ Infants with single ventricle heart disease have a high-medication burden during the interstage period. Despite the focused and intensified use of medications to improve feeding tolerance and somatic growth, current pharmacotherapeutic regimens appear to have little effect on interstage weight gain.
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The Fontan procedure has undergone many modifications since first being performed on a patient with tricuspid valve atresia in 1968. It is now the procedure of choice for individuals born with single-ventricle physiology or for those in whom a biventricular repair is not feasible. Forty years of experience with the Fontan procedure have gradually revealed the shortfalls of such a circulatory arrangement. ⋯ The clinical significance of these findings, however, has remained poorly understood. As Fontan survivors have increased in age and number, we have begun to better recognize subclinical hepatic dysfunction and the contribution of liver disease to adverse outcomes in this population. The purpose of this review is to summarize the existing data pertaining to liver disease in the Fontan population and to identify some questions that have yet to be answered.
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Congenital heart disease · May 2011
Interhospital transport of children requiring extracorporeal membrane oxygenation support for cardiac dysfunction.
Many centers are able to emergently deploy extracorporeal membrane oxygenation (ECMO) as support in children with refractory hemodynamic instability, but may be limited in their ability to provide prolonged circulatory support or cardiac transplantation. Such patients may require interhospital transport while on ECMO (cardiac mobile [CM]-ECMO) for additional hemodynamic support or therapy. There are only three centers in the United States that routinely perform CM-ECMO. Our center has a 20-year experience in carrying out such transports. The purpose of this study was twofold: (1) to review our experience with pediatric cardiac patients undergoing CM-ECMO and (2) identify risk factors for a composite outcome (defined as either cardiac transplantation or death) among children undergoing CM-ECMO. ⋯ Air and ground CM-ECMO transport of pediatric patients with refractory myocardial dysfunction is safe and effective. In our study cohort, the need for post-CM-ECMO renal support was associated with the composite outcome of death/need for cardiac transplant.
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Congenital heart disease · Mar 2011
Multicenter StudyVariation in postoperative care following stage I palliation for single-ventricle patients: a report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative.
The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry. ⋯ Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.