Profound hypothermia with alpha-stat pH management during open-heart surgery is associated with choreoathetosis.

A retrospective chart review was conducted to examine risk factors associated with the occurrence of choreoathetosis, a rare but significant complication of open-heart surgery in children. Ten children were identified as having developed choreoathetosis after cardiac surgery. Their charts were reviewed and compared with 33 age- and diagnosis-matched controls who underwent open-heart surgery during the same time period. Children with choreoathetosis reached lower rectal and esophageal temperatures (p = 0.0018 for both) and spent a greater portion of total bypass time at lower rectal and esophageal temperatures (p < 0.001 for both). Duration of cooling below 20 degrees C esophageal temperature and PaCO2 at the end of the cooling period were significant predictors of choreoathetosis (p = 0.023 and p = 0.0497, respectively) in a logistic regression model, and a greater fraction of choreoathetosis patients had prior developmental delays (p = 0.017). No difference was found in the age at surgery, duration of bypass, aortic cross-clamp time, arterial pH, PaCO2 or mean arterial pressure. The combination of extended exposure to profound hypothermia and alpha-stat pH management strategy and preexisting developmental delay are associated with the development of choreoathetosis following open-heart surgery 61 in children.

Improved pretransplant management of infants with hypoplastic left heart syndrome enables discharge to home while waiting for transplantation.

For infants whose families select primary transplantation for hypoplastic left heart syndrome (HLHS), the waiting time averages 3 months. Given the relative shortage of organs, the morbidity and mortality of these patients have been high. Therefore, pretransplant management is critical to improve the number of patients who survive to transplantation. This series shows our evolving management for these children, with an emphasis on nonintensive care. Fifty-two infants with HLHS were listed for primary transplantation at our institution during a 6-year period. The management was aimed at manipulating the pulmonary and systemic blood flows by low-dose continuous infusion of prostaglandin E1 (PGE1), early use of inhaled nitrogen, delayed opening of the atrial septum, and discharge to home with PGE1 infusion for continuing care when the child was on room air and growing. Almost all of the children (46/ 52) required nitrogen therapy with initial FiO2 of 0.16-0.17. Patients were weaned off nitrogen by 5 to 6 weeks of age. One fourth of the children needed atrial septal opening, typically at 2 or 3 months of age. Seventeen (32.7%) of the infants were able to spend at least some of their waiting time at home. Forty-five of the 52 children (86.5%) survived to receipt of a donor heart. Newborns with HLHS whose families select primary transplantation as their surgical option can be managed with a minimally invasive approach until receipt of a donor heart with an improvement in mortality rate.

Cardiac involvement in inflammatory disease: systemic lupus erythematosus, rheumatic fever, and Kawasaki disease.

This review focuses on treatment and preventive issues relevant to cardiac problems associated with systemic lupus erythematosus, acute rheumatic fever, and Kawasaki disease in adolescence. Cardiac abnormalities occur as a result of the immune dysfunction and as side effects of therapy. With increased survival, more cardiac complications are being detected. Screening for cardiac risk factors, such as cigarette smoking, hypertension, obesity, and hypercholesterolemia, should begin in adolescence.

Respiratory syncytial virus immune globulin for prophylaxis against respiratory syncytial virus disease in infants and children with congenital heart disease. The Cardiac Study Group.

OBJECTIVE: To examine the effectiveness of respiratory syncytial virus immune globulin administered intravenously (RSV-IGIV) in reducing hospitalization for treatment of RSV in children with congenital heart disease (CHD). METHODS: Children younger than 4 years of age were randomly assigned to a treatment group receiving RSV-IGIV, 750 mg/kg, monthly or to a control group not receiving infusions. Surveillance for respiratory tract infections was carried out and management decisions were made by physicians blinded to treatment group. RESULTS: Hospitalization for treatment of an RSV infection occurred in 32 of 214 (15%) of control children and 21 of 202 (10%) of the children receiving RSV-IGIV, a 31% reduction (P = .16). However, in infants younger than 6 months of age at study entry, 20 of 82 (24%) in the control group and 10 of 96 (10%) in the RSV-IGIV group had RSV hospitalizations (58% reduction, P = .01). The incidence of hospitalization for any respiratory tract symptomatology was lower in the RSV-IGIV group (34 of 202, 17%) than in the control group (57 of 214, 27%; P = .02). There was a significantly higher frequency of unanticipated cyanotic episodes and of poor outcomes after surgery among children with cyanotic CHD in the RSV-IGIV group (22 of 78, 28%) than in the control group (4 of 47, 8.5%; P = .009). CONCLUSION: RSV-IGIV should not be used for prophylaxis of RSV disease in children with cyanotic CHD. RSV-IGIV did not reduce RSV hospitalization in all children with CHD, but it was effective in preventing RSV hospitalization in infants younger than 6 months of age. Further studies in these children are indicated.

Pulmonary vascular disorders masquerading as interstitial lung disease.

During the acquisition of a series of 92 children with interstitial lung disease (ILD) over a 14 year period, a significant minority (8/92 or 9%) were initially diagnosed as having ILD, but were subsequently found to have a variety of arterial, venous, and/or capillary disorders that explained their initial pulmonary findings. This subgroup of patients has had a very high morbidity and mortality, with only three of eight patients currently surviving. The presentation, evaluation, and natural history of these eight children were reviewed. We developed a strategy of cardiac and pulmonary evaluation for children presenting with clinical and radiographic features of ILD that helped us to identify rapidly those with pulmonary vascular disorders.

Heart transplantation in children and young adults: early and intermediate-term results.

The purpose of this article is to report our short- and intermediate-term follow-up of cardiac transplantation for congenital heart disease and cardiomyopathy in children (age greater than 6 months), adolescents, and young adults. Thirty patients (ages 8 months to 24 years) with end-stage heart failure have undergone cardiac transplantation in our program: 12 (40%) for postoperative end-stage heart failure, 9 (30%) as primary treatment for congenital heart disease, 5 (17%) for dilated cardiomyopathy, and 4 (13%) for restrictive/hypertrophic cardiomyopathy. Nineteen patients (63%) had undergone prior operations; 4 patients received transplants for failed Fontan procedures. Induction therapy with antithymocyte therapy was used routinely, and long-term immunosuppression was by cyclosporine and azathioprine alone. Rejection surveillance/diagnosis was based on echocardiographic criteria. Posttransplantation follow-up ranges from 3 to 78 months. Operative mortality was 3.3% (1/30). No patients have been diagnosed with either accelerated allograft atherosclerosis or posttransplantation lymphoproliferative disease. We conclude that cardiac transplantation may be performed with excellent early and intermediate-term results.

Guidelines for cardiac monitoring of children during and after anthracycline therapy: report of the Cardiology Committee of the Childrens Cancer Study Group.

The anthracycline antibiotics, daunorubicin, doxorubicin, and the newer derivatives, are important components of many antineoplastic chemotherapeutic regimens. Their usefulness is limited by their cardiotoxicity. Sequential monitoring of cardiac function of patients undergoing chemotherapy allows identification of subclinical cardiotoxicity. In many patients monitoring can thus guide the modification of the chemotherapy to minimize cumulative cardiotoxicity, reducing acute and long-term clinical and subclinical sequelae. Such monitoring also aids in the comparison of cardiotoxicity produced by different drugs and different methods and schedules of drug administration. The considerable variability of monitoring regimens between institutions and in the literature has detracted from its usefulness. The Cardiology Committee of the Childrens Cancer Study Group has, therefore, reviewed the field and has formulated recommendations for standardized noninvasive monitoring of children during and immediately after chemotherapy and for the modification of the chemotherapy where indicated.

Treatment of childhood angiomatous diseases with recombinant interferon alfa-2a.

A heterogeneous group of five patients with progressive, invasive angiomatous diseases including pulmonary hemangiomatosis, angiosarcoma, or massive hemangioma with associated consumptive coagulopathy were treated with interferon alfa-2a for periods of 17 to 33 months. One patient with a large thoracic hemangioma, cardiorespiratory failure, and consumptive coagulopathy died after less than 2 months of treatment. The remaining four patients have shown beneficial responses, including (1) regression of abnormal vessels on pulmonary angiogram and improved exercise tolerance in pulmonary hemangiomatosis (two patients), (2) decreased corticosteroid and/or platelet transfusion requirements in consumptive coagulopathy (two patients), and (3) decreased size and number of tumor nodules in the one patient with angiosarcoma arising in preexisting angiomatous lesions. Responses occurred during periods of 2 to 20 months of treatment. There was no measurable progression of angiomatous lesions in any patient receiving interferon at the therapeutic dose, except possibly in the one who died. Each of the four surviving patients had improved linear growth and weight gain during interferon treatment.

Congenital polyvalvular disease in trisomy 18: echocardiographic diagnosis.

Congenital heart disease is known to occur in greater than 90% of patients with trisomy 18, with ventricular septal defect and patent ductus arteriosus being the most frequently encountered lesions. The presence of congenital polyvalvular disease in trisomy 18 as assessed by pathological specimens has also been noted. Echocardiograms were obtained in 15 patients with trisomy 18 and in 12 infants with dysmorphic features, who did not have chromosomal abnormalities, in order to obtain an echocardiographic assessment of the frequency of polyvalvular disease in living patients with trisomy 18. In this series all patients with trisomy 18 had structural defects (seven ventricular septal defects, three patent ductus arteriosus, five both). All trisomy 18 patients also had congenital polyvalvular disease with six patients having four affected valves, five patients having three affected valves, and four patients with two affected valves. In patients with normal chromosomes, two had a single abnormal valve, and structural lesions included patent ductus arteriosus (3), ventricular septal defect (2), pulmonary atresia with ventricular septal defect (1), transposition of the great arteries (1), and atrioventricular canal with patent ductus arteriosus and coarctation (1). In infants with features suggestive of trisomy 18, structural cardiac lesions are a nonspecific finding. However, the presence of polyvalvular disease may be a more specific and useful adjunct to other clinical investigations pending chromosomal analysis for definitive diagnosis.

Congenital vertebral-jugular fistula in an infant.

Pediatric cardiologists are often consulted to evaluate continuous murmurs in children. This case report describes a child with a congenital vertebral-jugular fistula who presented with torticollis and a continuous murmur. The differential diagnosis of continuous murmurs in this setting is discussed.

Hereditary atrioventricular conduction defect in a child.

Hereditary atrioventricular conduction defect is an uncommon cause of acquired complete heart block in children. We report a father and son, both of whom presented with seizures as the initial manifestation of acquired complete heart block and required permanent pacemaker implantation. A review of the variations of this entity and the histopathological findings of previously reported cases is presented. Family members of patients with acquired heart block of uncertain etiology should be examined for the presence of conduction abnormalities that may progress to complete heart block.

Normal aerobic and anaerobic exercise data for North American school-age children.

The purpose of this study was to establish normative data for untrained, healthy North American children by means of the James protocol for bicycle ergometry. Data were obtained on 151 of 185 children (70 girls and 81 boys). Their ages ranged from 7 years 6 months to 12 years 9 months. All subjects were divided into groups by gender and body surface area (BSA). Maximum heart rates were greater in girls. The mean difference between maximum and recovery heart rates differed significantly by gender, girls taking longer to recover than boys. Maximum oxygen consumption (measured in cubic centimeters per minute per kilogram body weight) did not differ in boys and girls. Ventilatory anaerobic threshold (VAT) occurred when there was an isolated increase in the slope for ventilatory equivalent for oxygen consumption (VE/VO2) with no change in the slope for ventilatory equivalent for carbon dioxide production (VE/VCO2) when both were plotted against time. Absolute oxygen consumption (VO2) at VAT increased with BSA in both sexes, and, when normalized to body size and expressed as a percent of VO2 maximum, no significant difference was observed between the sexes. These data may be used in the fitness evaluations of preadolescent children from North America.

The modified Blalock-Taussig shunt: analysis of adequacy and duration of palliation.

The modified Blalock-Taussig shunt provides excellent palliation for patients with cyanotic heart disease and may be the optimal shunt for infants less than 3 months old. The duration of palliation with flow limited by a fixed conduit between the subclavian and pulmonary artery is unknown. Between October 1980 and June 1985 forty-nine patients received 55 shunts. Age at operation was 1 day to 15 years (median 2 weeks), with 29 patients less than 3 months old. Weights were 1.7 to 53 kg, with 31 patients less than 5 kg. There were two early (4%) and four late (9%) deaths, none shunt related. The 43 late survivors were followed from 2 to 60 months (mean 27 +/- 16). Late study in 32 patients revealed a mean PO2 of 52 +/- 2 mm Hg. No patient had pulmonary arterial distortion or hypertension. In 10 patients inadequate shunt function was noted from 12 to 34 months after operation (mean 28 +/- 10). There were no sudden failures and all shunts remained patent. Univariate analysis revealed that age less than 3 months (p = .0154) and weight less than 3.6 kg (p = .0110) were risk factors predictive of earlier shunt failure. The mean satisfactory shunt survival time for these two groups was 38 +/- 3.6 and 35 +/- 3.7 months, respectively. The presence of pulmonary atresia (p = .1175) and the specific diagnostic group (p = .7620) were not of themselves risk factors.(ABSTRACT TRUNCATED AT 250 WORDS)

Sinus node shift after the Senning procedure compared with the Mustard procedure for transposition of the great arteries.

To investigate the nature of the dominant intrinsic cardiac pacemaker activity after the Senning procedure, endocardial mapping of the systemic venous atrium was accomplished a mean of 13 months after operation in 10 patients, aged 22 +/- 6 months. Multiple endocardial sites were measured to find the earliest atrial electrical activity timed back from the QRS complex. These data were compared with data from endocardial mapping performed in 6 patients late after the Mustard procedure. In 8 of 10 patients who had undergone the Senning procedure, the earliest activation time, corresponding by definition to the origin of the intrinsic pacemaker, was located in the inferior medial portion of the superior limb of the systemic venous atrium. In the other 2 patients who had undergone the Senning procedure, the earliest activity was in the high superior limb of the baffle at its junction with the superior vena cava. In contrast, the earliest activity in all patients who underwent the Mustard operation was at the junction of the superior vena cava and the superior limb of the systemic venous atrium. In response to programmed extrastimulation, the electrophysiologic behavior of the intrinsic pacemaker in the Senning group was abnormal compared with known normal sinus node (SN) data in only 3 of 10 patients, whereas all patients in the Mustard group had SN dysfunction. Abnormal SN function was noted in both patients in the Senning group, in whom a shift in the position of the earliest endocardial activation point was not seen.(ABSTRACT TRUNCATED AT 250 WORDS)