Long-term noninvasive arrhythmia assessment after total anomalous pulmonary venous connection repair.

Background: Pediatric patients with a history of atrial surgery are at risk for the development of sinus node dysfunction and atrial arrhythmias. However, there has been no comprehensive, long-term, electrophysiologic study of patients who have undergone repair of total anomalous pulmonary venous connection.

Methods: We evaluated school-aged and adolescent survivors of isolated total anomalous pulmonary venous connection repair from January 1983 to December 1996 to assess for sinus node dysfunction, atrioventricular block, and atrial and ventricular arrhythmias.

Negative impact of new-onset diabetes mellitus on patient and graft survival after liver transplantation: Long-term follow up.

Background: Little is known about the long-term consequences of new-onset diabetes mellitus (NODM) after liver transplantation (LTX).

Methods: In a chart review between 1996 and 2004, we evaluated its incidence and possible effect on patient and graft survival. Inclusion criteria were: adult primary LTX; deceased donor LTX without combined organs; and dual immunosuppression with tacrolimus and corticosteroid.

Illustration of the additional value of real-time 3-dimensional echocardiography to conventional transthoracic and transesophageal 2-dimensional echocardiography in imaging muscular ventricular septal defects: does this have any impact on individual patient treatment?

Objective: We sought to answer the question of whether the additional morphologic details obtained by real-time 3-dimensional (3D) echocardiographic (RT3DE) imaging of muscular ventricular septal defect (VSD) has any significant impact on treatment options of individual patient.

Background: Muscular VSD can be safely and effectively closed by interventional catheterization procedure using VSD devices under transesophageal echocardiographic (TEE) guidance. Recent application of RT3DE has shown great promise for imaging VSD with better display of the exact geometry, size, and location of the defect.

Heterotaxy syndrome with functional single ventricle: does prenatal diagnosis improve survival?

Background: Despite improved outcome for many single ventricle lesions, staged reconstruction for heterotaxy syndrome with a functional single ventricle continues to have a high mortality. Prenatal identification of heterotaxy syndrome may improve long-term survival.

Methods: Our database was reviewed from January 1996 to December 2004 for patients with heterotaxy syndrome.

Outcomes of the Fontan procedure using cardiopulmonary bypass with aortic cross-clamping.

Background: Avoidance of cardiopulmonary bypass (CPB) and aortic cross-clamping during the Fontan procedure has been advocated to improve outcomes. We continue to use CPB with aortic cross-clamping for the Fontan procedure.

Methods: We performed a review of patients undergoing the Fontan procedure between January 1, 2000 and December 31, 2004.

Effect of short-term sequential administration of nonsteroidal anti-inflammatory drugs on the stomach and proximal portion of the duodenum in healthy dogs.

Objective: To evaluate effects of injection with a nonsteroidal anti-inflammatory drug (NSAID) followed by oral administration of an NSAID on the gastrointestinal tract (GIT) of healthy dogs.

Animals: 6 healthy Walker Hounds.

Procedures: In a randomized, crossover design, dogs were administered 4 treatments consisting of an SC injection of an NSAID or control solution (day 0), followed by oral administration of an NSAID or inert substance for 4 days (days 1 through 4).

Neo-aortic valvar function after the arterial switch.

Objectives: The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation.

Methods: We included all patients who had undergone an arterial switch operation at The Children's Hospital of Philadelphia, and had been followed for a minimum of 4 years, and had at least 2 postoperative echocardiograms. Neo-aortic valvar regurgitation was quantitatively assessed, and measurements were made of the neo-aortic root at the level of the basal attachment of the leaflets, mid-sinusal level, and the sinutubular junction.

Effect of positive end-expiratory pressure on oxygen delivery during 1-lung ventilation for thoracoscopy in normal dogs.

Objective: To evaluate the effect of positive end-expiratory pressure (PEEP) on oxygen delivery (DO(2)) with 1-lung ventilation during thoracoscopy in normal anesthetized dogs.

Study Design: Prospective, controlled experimental study.

Animals: Eight, adult, intact Walker Hound dogs weighing 25.

Temporary elevation of serum transaminases after pediatric intestinal transplantation: incidence and clinical correlation in multivisceral transplant vs isolated intestinal transplant.

Data were gathered from the records of 51 children of median age 1.5 years who survived more than 6 months after intestinal transplantation. Abnormal liver function tests (LFTs) were defined as serum aspartate aminotransferase (AST) greater than 100 IU/L or total bilirubin greater than 2.

Renal dysfunction following adult intestinal transplant under tacrolimus-based immunosuppression.

We analyzed data from the records of 24 adult patients who survived more than 2 years after intestinal transplantation performed between 1995 and 2002 under tacrolimus-based immunosuppression. Ages ranged from 19.3 to 59.

Renal function after pediatric intestinal transplant.

Data were analyzed from 44 patients who survived more than 2 years after intestinal transplantation performed between 1994 and 2002. Median age was 1.7 years.

Analysis of risk factors for the development of posttransplant lymphoprolipherative disorder among 119 children who received primary intestinal transplants at a single center.

Unlabelled: Posttransplant lymphoproliferative disorder (PTLD) is a well-known complication after pediatric transplantation. We analyzed all potential risk factors to assess patient and graft outcomes of 119 children who received intestinal transplantations.

Materials And Methods: Between August 1994 and March 2005, 119 patients underwent cadaveric intestinal transplantation.

Intestinal transplantation with alemtuzumab (Campath-1H) induction for adult patients.

Background: Alemtuzumab (Campath-1H [C1H]) is a humanized monoclonal antibody directed against the CD 52 antigen that is present on the surface of T cells, B cells, natural killer cells and monocytes. We studied its application in intestinal transplantation.

Methods: This is a retrospective review of adult patients who underwent intestinal transplantation between December 1994 and May 2005.

An association of lower serum citrulline levels within 30 days of acute rejection in patients following small intestine transplantation.

Introduction: In a prospective protocol we studied whether serum citrulline level within 30 days of an acute rejection was predictive of the episode.

Methods: An acute rejection episode was defined as the date of occurrence of any biopsy-proven rejection in which treatment was initiated until two successive biopsies showed no further rejection. We compared the mean citrulline level based on values determined within 30 days of the start of an acute rejection episode with the mean citrulline level measured on the same patient during a rejection-free period.

Analysis of rejection episodes in over 100 pediatric intestinal transplant recipients.

Rejection after intestinal transplant is a significant source of morbidity and mortality. We analyzed number of rejections, severity, and duration of episodes in pediatric recipients of intestinal transplants. One hundred eighteen intestinal transplants were performed: intestine (n = 27), liver-intestine (n = 27), modified multivisceral (n = 7), and multivisceral (n = 57).

Expanded use of multivisceral transplantation for small children with concurrent liver and intestinal failure.

Fifty-five children with liver and intestinal failure have been transplanted at our center under daclizumab induction therapy since 1998. Of those, 19 received five multiviceral transplantation (MVT), 12 liver-intestine-pancreas transplants, and 2 noncomposite liver and intestine transplants (NCLIT) before 2001 (group 1). During this period, MVT was only used in children with gastric dysmotility.

Growth after intestinal transplant in children.

Intestinal transplantation has been more frequent in children with intestinal failure. However, the growth after intestinal transplantation has not been well documented. The demographics, transplant information, postoperative complications, heights, and weights were obtained retrospectively from medical records on 23 children who underwent intestinal transplantation.

The Miami experience with almost 100 multivisceral transplants.

We report our experience with 98 patients who received primary multivisceral transplantations. Three eras can be distinguished based on the evolution of technique, immunosuppression, and monitoring: August 1994 to December 1997 (first era); January 1998 to December 2000 (second era); and January 2001 to present (third era). Sixteen patients were transplanted during the first era, 18 during the second era, and 64 during the third era.

Early results of single-stage biventricular repair of severe aortic hypoplasia or atresia with ventricular septal defect and normal left ventricle.

Objective: Biventricular repair of aortic atresia (or severe aortic hypoplasia) is possible in the presence of a ventricular septal defect and normal left ventricle. We considered whether primary biventricular repair was a safe alternative in all cases, even in the presence of interrupted aortic arch.

Methods: This was a retrospective analysis of patients who underwent primary biventricular repair consisting of a combination Norwood-type reconstruction of the aortic arch, baffle of the left ventricle to both semilunar roots, and conduit placement from the right ventricle to pulmonary arteries.

The nomenclature, definition and classification of hypoplastic left heart syndrome.

The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. At the severe end of the spectrum is found the combination of aortic and mitral atresia, when the left ventricle can be close to non-existent. At the mild end are the patients with hypoplasia of the aortic and mitral valves, but without intrinsic valvar stenosis or atresia, and milder degrees of left ventricular hypoplasia.

Intestinal and multivisceral transplantation in children.

Objective: To describe a single-center experience of pediatric intestinal transplantation (Itx) and to provide an overview of the children who underwent this procedure along with their outcomes.

Summary Background Data: Pediatric Itx presents multiple challenges because of the very young ages at which patients require transplantation and their higher susceptibility to infectious complications.

Methods: We have performed 141 Itx in 123 children with a median age of 1.

Heart rate variability following neonatal heart surgery for complex congenital heart disease.

Background: Altered cardiac autonomic control may play a role in the morbidity and mortality suffered by neonates who undergo surgery for complex congenital heart disease (CHD). The purpose of this study was to evaluate cardiac autonomic activity, as measured by spectral indices of heart rate variability (HRV), prior to and early after infant surgery for CHD and attempt to correlate HRV indices with clinical outcome. In addition, we assessed the hypothesis that single-ventricle physiology and surgical interruption of the great arteries negatively affects HRV.

The importance of analyzing graft and patient survival by cause of failure: an example using pediatric small intestine transplant data.

Background: Although graft and patient survival are vital in reporting overall results of clinical transplant studies, these outcomes do not account for distinct types of graft failure and death, which clearly exist in pediatric small intestine transplantation (Itx). The use of a cause-specific hazard (CSH) approach may provide more precise identification and thus greater insight as to why certain factors are prognostically important.

Methods: Among 119 pediatric patients who received primary Itx at our center since 1994, Cox model stepwise regression analyses were performed to identify prognostic factors for the following CSH rates: intestinal graft failure (IGF)/death due to rejection, death due to infection not triggered by IGF, and intestinal graft loss/death due to other causes.

Management of sinus venosus defects.

Sinus venosus defects are not atrial septal defects, but are intra-atrial communications outside of the boundaries of the atrial septum. The superior type is located above and separate from the fossal ovalis, usually adjacent to the superior vena cava and the right upper pulmonary vein. The inferior type is located near the orifice of the inferior vena cava and the right lower pulmonary vein.