Unusual Longevity of Edwards Syndrome: A Case Report.

Background: Trisomy 18, also known as Edwards syndrome, was first described in the 1960s and is now defined as the second most common trisomy. While this genetic disease has been attributed to nondisjunction during meiosis, the exact mechanism remains unknown. Trisomy 18 is associated with a significantly increased mortality rate of about 5-10% of patients surviving until 1 year of age.

Iodixanol pharmacokinetics in children.

The objective of this report was to study the elimination pharmacokinetics of iodixanol in children. Iodixanol (Visipaque, Nycomed Inc., Wayne, PA, USA) is a new iso-osmolar iodinated radiocontrast agent.

Pulmonary arteriovenous malformations after cavopulmonary connection.

We report the case of a child with hypoplastic left heart syndrome who developed pulmonary arteriovenous (AV) malformations after superior cavopulmonary anastomoses. Resolution of the pulmonary AV malformations occurred following a completion Fontan procedure. This phenomenon has been reported previously, but only in patients with heterotaxy and polysplenia.

Surgical palliation of truncus arteriosus with mitral atresia and hypoplastic left ventricle.

A rare case of truncus arteriosus associated with mitral valve atresia, hypoplastic left ventricle, and intact ventricular septum is reported. Successful medical management and surgical palliation of this defect is described. The possible embryology, pertinent hemodynamics, and clinical concerns with this unusual case are discussed.

Outcome of listing for heart transplantation in infants younger than six months: predictors of death and interval to transplantation. The Pediatric Heart Transplantation Study Group.

Background: The major limiting factor to successful heart transplantation in infants is the limited supply of donors. To examine the impact of donor limitations on survival after listing, a multiinstitutional study was designed to identify risk factors for death while waiting and for longer interval to transplantation.

Methods: Between January 1 and December 31, 1993, 118 infants 6 months of age or younger (86 younger than 29 days) were listed for heart transplantation from 21 institutions.

Myocardial acoustics in pediatric allograft rejection.

Background: Ultrasonographic tissue characterization is the assessment of physical properties of biologic tissue on the basis of quantitative analysis of its acoustic characteristics. Abnormalities in microscopic structure that occur with cardiac allograft rejection may result in characteristic alterations in myocardial acoustics. Ultrasonographic tissue characterization may allow noninvasive detection of rejection.

Expanding indications for pediatric coronary artery bypass.

Pediatric coronary artery bypass has been done mostly for ischemic complications of Kawasaki disease. We reviewed our clinical experience between 1987 and 1994 with internal thoracic artery-coronary artery bypass in one infant and five children for varying indications. Indications for coronary bypass included Kawasaki disease (2), congenital left main coronary ostial stenosis, iatrogenic coronary cameral fistula, anomalous origin of the left coronary artery from the pulmonary artery, and single coronary artery traversing between the great arteries in a patient after cardiac transplantation.

Inhaled nitric oxide for children with congenital heart disease and pulmonary hypertension.

Background: Endothelium-derived nitric oxide (NO) is a potent vasodilator and a major mediator of pulmonary vascular tone.

Methods: Five infants underwent a trial of inhaled NO with hemodynamic monitoring in the operating room after atrioventricular canal repair. An additional 15 patients with congenital heart disease and refractory pulmonary hypertension were treated with inhaled NO for 1 day to 10 days postoperatively.

Coarctation of the aorta. Repair with polytetrafluoroethylene patch aortoplasty.

Background: The first successful surgical repair of coarctation of the aorta (CoAo) was performed in 1944, but during the years that followed a high incidence of recoarctation was seen, ranging from 20% to 86%. In response to that problem, the patch aortoplasty was introduced in 1957; however, true aneurysms were found in the aortic wall opposite the patch after Dacron patch aortoplasty, particularly when the coarctation ridge was excised. The purpose of our review was to evaluate the results of patch aortoplasty for CoAo using a relatively new material, polytetrafluoroethylene (PTFE), and an operative technique that does not involve resection of the coarctation ridge.

Repair of complete atrioventricular canal defects: results with the two-patch technique.

Background: Between 1983 and 1994, 115 infants and children underwent repair of a complete atrioventricular canal defect with the two-patch technique and routine mitral valve "cleft" closure.

Methods: A retrospective review of these 115 patients was performed. Age at the time of repair ranged from 1 month to 108 months (mean age, 14.

Influence of gender on allograft rejection in a rat heart transplant model.

Background: Clinical studies have reported increased rejection in female heart transplant recipients. Conflicting data exist as to whether rejection is increased with male donors or female donors in these female recipients.

Methods: We investigated in this study allograft rejection in four sets of gender combinations with and without immunosuppression with the use of a heterotopic rat heart transplant model: male donor heart to female recipient, female donor heart to female recipient, male donor to male recipient, and female donor to male recipient.

Reversible cardiomyopathy due to carnitine deficiency from renal tubular wasting.

The clinical course of a 4-month-old male infant with a dilated cardiomyopathy secondary to renal tubular losses of carnitine is outlined. He was admitted to the hospital with severe congestive heart failure. An echocardiogram demonstrated normal anatomy.

Tetralogy of Fallot with anomalous origin of the right coronary artery.

This report describes a child with tetralogy of Fallot and acquired pulmonary valve atresia in whom the pulmonary blood flow was provided solely by retrograde flow from an anomalous right coronary artery originating from the main pulmonary artery. Complete repair with preservation of the native pulmonary valve and implantation of the right coronary artery into the ascending aorta is described.

Normal left ventricular muscle mass and mass/volume ratio after pediatric cardiac transplantation.

Background: The adaptive growth of the transplanted heart within the growing child may contribute to long-term cardiac performance. The ability to achieve increased ventricular volume and appropriate muscle mass in the face of immunosuppression and cardiac denervation has not been studied. We previously reported normal left ventricular (LV) volume growth over a 3-year period after cardiac transplantation.

Posttransplant coronary artery disease in children. A multicenter national survey.

Background: Accelerated coronary atherosclerosis after heart transplantation (TCAD) is a major cause of late death. It is progressive and eventually fatal, and currently no therapy exists. The purpose of this study was to determine the incidence as well as the clinical and age-dependent characteristics of pediatric patients with TCAD.

Pharmacodynamics and pharmacokinetics of esmolol, a short-acting beta-blocking agent, in children.

Esmolol, a short-acting intravenous cardioselective beta-blocking agent, was evaluated for age-dependent pharmacodynamic and pharmacokinetic features in 17 young patients (6 months to 14 years). A loading dose (500 micrograms/kg/min) alternating with a maintenance dose (25-200 micrograms/kg/min, titrating by 25 micrograms/kg/min every 4 min) was infused until the heart rate or mean arterial pressure decreased 10%. Cardiac index, left ventricular shortening fraction, and systemic vascular resistance were measured at baseline, peak esmolol effect, and recovery.

Role of endomyocardial biopsy in rejection surveillance after heart transplantation in neonates and children.

Objectives: The aim of this study was to retrospectively evaluate the sensitivity of noninvasive surveillance (physical examination, echocardiography) of rejection in accurately predicting histologically documented rejection episodes. Additionally, the usefulness of routine scheduled biopsy and its safety in pediatric patients was explored.

Background: Endomyocardial biopsy has been utilized as the standard for rejection surveillance after heart transplantation in adults, but its role in documenting clinically suspected rejection and in routine surveillance of pediatric patients has not been agreed upon.

Pharmacologic reduction of pretransplantation pulmonary vascular resistance predicts outcome after pediatric heart transplantation.

Pulmonary hypertension leading to donor right ventricular dysfunction remains a major risk factor associated with poor outcome after heart transplantation. This study evaluated a pretransplantation protocol to assess pulmonary vascular resistance index and its response to pharmacologic modulation. Cardiac catheterization was performed in 25 patients (mean age, 8.

Restrictive ventricular septal defect: how small is too small to close?

Historically, indications for ventricular septal defect closure have included congestive heart failure, pulmonary hypertension, aortic insufficiency with or without aortic valve prolapse, and prior bacterial endocarditis. However, controversy exists as to how the lifetime risk of an isolated, nonoperated restrictive ventricular septal defect compares with the risk of surgical closure in an asymptomatic child. Between 1980 and 1991, cardiac catheterization and elective ventricular septal defect closure (age > 1 year, pulmonary to systemic flow ratio < 2.

Captopril reduces graft coronary artery disease in a rat heterotopic transplant model.

Background: Transplant coronary artery disease (CAD) is characterized by severe myointimal proliferation causing vascular stenosis. Nontransplant vascular injury models have shown that angiotensin converting enzyme (ACE) inhibitors reduce myointimal proliferation and preserve lumen integrity. We examined the effect of the ACE inhibitor captopril on graft CAD in a Lewis to F344 rat heterotopic cardiac transplantation model.

Neonatal and infant heart transplantation.

Since the advent of cyclosporine A, cardiac transplantation has been offered routinely for adolescents and children with cardiomyopathy refractory to medical management. Recently, cardiac transplantation has been offered to neonates with severe structural congenital heart disease. This article reviews the indications, pretransplant evaluation, and post-transplant management of neonates and infants undergoing cardiac transplantation.

Failure of the Hemashield extension in right ventricle-to-pulmonary artery conduits.

Between 1989 and 1991, 17 children underwent 18 right ventricle-to-pulmonary artery conduit placement operations using a composite of an aortic or pulmonary valved homograft and a Hemashield extension to the ventricle. Hemashield is a collagen-coated knitted Dacron graft with excellent compliance and hemostatic properties. Diagnoses included tetralogy of Fallot with pulmonary atresia (7), truncus arteriosus (6), and complex transposition of the great arteries (4).

Spectrum of humoral rejection after pediatric heart transplantation.

The deposition of immunoglobulin and complement 3 in the coronary microvasculature (humoral rejection) has been associated with poor outcome after heart transplantation. The purpose of this study is to relate the incidence of immunoglobulin and complement 3 myocardial deposition to the clinical course of pediatric heart transplant patients. One hundred thirty-one biopsy specimens from 30 patients, whose mean age at transplantation was 4.