Welton M. Gersony

Effects of indomethacin in premature infants with patent ductus arteriosus: results of a national collaborative study.

Among 3559 newborn infants with birth weight less than 1750 gm, 421 developing a hemodynamically significant patent ductus arteriosus were entered into a randomized trial to evaluate the role of indomethacin in the management of PDA. Indomethacin given concurrently with usual medical therapy at the time of diagnosis resulted in ductal closure in 79%, versus 35% with placebo (P less than 0.001). Indomethacin as backup to usual medical treatment resulted in similar closure rates. To assess overall effects through hospital discharge, three management strategies were compared. Although mortality did not differ significantly, infants given indomethacin only if usual therapy failed (strategy 2) had a lower incidence of bleeding than those to whom indomethacin was given with initial medical therapy (strategy 1) and lower rates of pneumothorax and retrolental fibroplasia than those to whom no indomethacin was administered, with surgery the only backup to medical therapy (strategy 3). Thus the administration of indomethacin only when medical treatment fails appears to be the preferable approach for the management of symptomatic PDA in premature infants.

Contemporary Outcomes After the Fontan Procedure: A Pediatric Heart Network Multicenter Study

OBJECTIVES We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP). BACKGROUND The characteristics of contemporary Fontan survivors are not well described. METHODS We enrolled 546 children (age 6 to 18 years, mean 11.9 years) and compared them within pre-specified anatomic and procedure subgroups. History and outcome measures were obtained within a 3-month period. RESULTS Predominant ventricular morphology was 49% left ventricular (LV), 34% right ventricular (RV), and 19% mixed. Ejection fraction (EF) was normal for 73% of subjects; diastolic function grade was normal for 28%. Child Health Questionnaire mean summary scores were lower than for control subjects; however, over 80% of subjects were in the normal range. Brain natriuretic peptide concentration ranged from <4 to 652 pg/ml (median 13 pg/ml). Mean percent predicted peak O2 consumption was 65% and decreased with age. Ejection fraction and EF Z score were lowest, and semilunar and atrioventricular (AV) valve regurgitation were more prevalent in the RV subgroup. Older age at Fontan was associated with more severe AV valve regurgitation. Most outcomes were not associated with a superior cavopulmonary connection before Fontan. CONCLUSIONS Measures of ventricular systolic function and functional health status, although lower on average in the cohort compared with control subjects, were in the majority of subjects within 2 standard deviations of the mean for control subjects. Right ventricular morphology was associated with poorer ventricular and valvular function. Effective strategies to preserve ventricular and valvular function, particularly for patients with RV morphology, are needed.

Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension.

BACKGROUND Patients with severe primary pulmonary hypertension have a poor prognosis, but those with a patent foramen ovale may survive longer. A few reports of clinical improvement after blade balloon atrial septostomy in patients with severe pulmonary vascular disease have appeared. The purpose of this study was to systematically evaluate the effects of blade balloon atrial septostomy on clinical signs and symptoms, hemodynamics, and survival in patients with severe primary pulmonary hypertension. METHODS AND RESULTS Blade balloon atrial septostomy was performed on 15 children and young adults with severe primary pulmonary hypertension. Despite maximal medical therapy, prior to septostomy all patients had recurrent syncope and 8 had severe right heart failure. Thirteen patients survived the procedure. After blade balloon atrial septostomy, no patient experienced further syncope, and signs and symptoms of right heart failure improved in all New York Heart Association Class IV patients. Within 24 hours after the procedure and at follow-up catheterization 7 to 27 months after septostomy, there was a significant increase in cardiac index, resulting in an increase in systemic oxygen transport. There was improved long-term survival in the 13 patients who survived blade balloon atrial septostomy compared with similar groups of primary pulmonary hypertension patients who received standard therapy (P < .05). CONCLUSIONS Blade balloon atrial septostomy resulted in clinical and hemodynamic improvement and improved survival in selected patients with severe primary pulmonary hypertension.

Coronary Artery Involvement in Children With Kawasaki Disease Risk Factors From Analysis of Serial Normalized Measurements

Background— Most studies of coronary artery involvement and associated risk factors in Kawasaki disease have used the Japanese Ministry of Health dichotomous criteria. Analysis of serial normalized artery measurements may reveal a broader continuous spectrum of involvement and different risk factors. Methods and Results— Clinical, laboratory, and echocardiographic measurements obtained at baseline and 1 week and 5 weeks after presentation were examined in 190 Kawasaki disease patients as part of a clinical trial of primary therapy with pulse steroids in addition to standard intravenous immunoglobulin. Maximum coronary artery z score normalized to body surface area was significantly greater than normal at all time points, decreasing significantly over time from baseline. A maximal z score ≥2.5 at any time was noted in 26% of patients. Japanese Ministry of Health dimensional criteria were met by 23% of patients. Significant independent factors associated with greater z score at any time included younger patient age, longer interval from disease onset to treatment with intravenous immunoglobulin, lower serum IgM level at baseline, and lower minimum serum albumin level. z Scores of the proximal right coronary artery were higher than those in the left anterior descending branch. Conclusions— Analyses of serial normalized coronary artery measurements in optimally treated Kawasaki disease patients demonstrated that for most patients, measurements are greatest at baseline and subsequently diminish; baseline measurements appear to be good predictors of involvement during early follow-up. When a more precise assessment is used, risk factors for coronary artery involvement are similar to those defined with arbitrary dichotomous criteria.

Propranolol administration during pregnancy: Effects on the fetus

The case of an infant born to a mother receiving continuous propranolol therapy throughout pregnancy isreported. Perinatal problems included: a small placenta, intrauterine growth retardation, fetal depression at birth, and postnatal hypoglycemia and bradycardia. These responses appeared to be the direct result of the maternal use of propranolol. Hence, infants born to mothers on continuous therapy with this agent should be considered at risk for developing these complications.

Pediatric infective endocarditis in the modern era

Sixty-two cases of endocarditis occurring in children between January 1977 and February 1992 were reviewed and compared with series from the 1970s and early 1980s. Changes in risk factors, pathogens, diagnostic modalities, and outcome were determined. Complex congenital heart disease (22 cases) and unrepaired ventricular septal defect (9 cases) were the most common underlying lesions. A total of 19 children with normal anatomy had endocarditis; 6 had community-acquired infection and 13 had hospital-acquired endocarditis (11 of these 13 children had central venous catheters in place, including 7 premature infants). Echocardiograms revealed vegetations in 25 of 49 patients; 24 of these patients had positive echocardiographic findings on the first study. Echocardiographic findings were most often negative in children with complex cyanotic heart disease. Staphylococcus aureus (39%) was the most common pathogen isolated and was associated with a higher incidence of central nervous system complications (p < 0.0015) and a greater need for surgical intervention (p = 0.01) than were other pathogens. Methicillin-resistant S. aureus (eight cases) and coagulase-negative staphylococci (three cases) emerged as important pathogens but were not associated with increased morbidity or mortality rates. Fungal endocarditis (six cases) had a 67% mortality rate. Overall the mortality rate was 11%. Endocarditis remained undiagnosed in seven seriously ill patients until postmortem examination. This study indicates that, during the past decade, important changes in risk factors, pathogens, and the susceptible population have altered the presentation and management of endocarditis in children.

Survival and Risk Factor Analysis for the Norwood Procedure for Hypoplastic Left Heart Syndrome

The optimal approach to hypoplastic left heart syndrome (HLHS) is controversial. The palliative Norwood operation, cardiac transplantation, and no surgical intervention have all been advocated. Centers that perform the Norwood operation have met with varied results, and conflicting reports exist regarding factors predictive of stage I outcome. From January 1990 to January 1996, 67 patients with HLHS were admitted with intent to perform the staged Norwood procedure. Fourteen patients did not undergo surgery. In the 53 patients treated surgically, outcome was reviewed, and 10 potential risk factors for first stage mortality were analyzed. Forty-one infants survived the Norwood I operation to hospital discharge (77% of the surgically treated patients and 61% of the entire group, including those who did not undergo operation) with 6 additional deaths 3 to 5 months after operation. Univariate analysis showed cardiopulmonary bypass time and circulatory arrest time to be significant risk factors for hospital mortality. Multivariate analysis revealed only cardiopulmonary bypass time as significant (p <0.01). Of the 15 prenatally diagnosed newborns who underwent surgery, 11 survived (p = 0.72). Ten of 11 patients with preoperative organ damage survived (p = 0.42). Among the 35 bidirectional Glenn (Norwood II) and Fontan (Norwood III) procedures performed, there were 2 deaths. The 5-year actuarial survival for patients who underwent operations was 61%. The Norwood procedure is a favorable option for the infant with HLHS. Surgical survival may be affected by a prolonged cardiopulmonary bypass time, but is not affected by other factors analyzed, including prenatal diagnosis and preoperative organ damage.

Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome.

Objective. To assess intellect and adaptive behavior in children with hypoplastic left heart syndrome (HLHS) who had undergone at least two surgical stages of the Norwood procedure. Methods. Fourteen children with HLHS >3 years of age participated in the study. The patients underwent intelligence quotient (IQ) testing, and their parents were interviewed regarding their childrens adaptive behavior. Results were compared with those of 10 family controls. Outcomes were studied for possible correlation with perioperative variables. Results. Among the HLHS patients, the median scores for full scale IQ and adaptive behavior were 88 and 91, respectively (normal = 100 ± 15). One child met criteria for mental retardation. Family controls scored generally higher than did HLHS patients, but only differences in adaptive behavior were statistically significant. A negative correlation was found between stage I circulatory arrest time and full scale IQ. Conclusions. Children with HLHS most often function in the low-normal range of intelligence and adaptive behavior. A prolonged circulatory arrest time may result in decreased intellectual function.

Arrhythmias after the Mustard operation for transposition of the great arteries: a long-term study

This study presents follow-up data on 95 patients with transposition of the great arteries who underwent a Mustard intraatrial baffle operation. The patients were followed up serially by means of scalar electrocardiograms and 24 hour Holter monitoring studies. The latter study was effective in documenting abnormal rhythm patterns when the standard electrocardiogram was within normal limits. Twenty percent of the patients had atrial arrhythmias at the time of hospital discharge. new rhythm disturbances were recognized during each year of follow-up; 75% of the patients had atrial rhythm disorders by the sixth year. Slow junctional rhythm was the most common rhythm disturbance found. Complete heart block did not occur. Supraventricular tachycardia occurred within the context of the tachycardia/bradycardia syndrome in 8 of 10 patients, and in all it was documented after hospital discharge. The incidence of sudden death in this series was 3%. Six of the patients have had pacemaker insertions and there have been no deaths among these individuals. It was observed that the slow atrial or junctional rhythms that appeared after intraatrial correction of transposition of the great arteries rarely progressed to life-threatening rhythm disorders in childhood or adolescence, but the prognosis in adult life remains unknown. The three deaths in the series occurred in patients with a history of supraventricular tachyarrhythmias. Elimination of arrhythmias caused by extensive intraatrial surgery is one of the potential advantages of the arterial switch operation for correction of transposition of the great arteries.

Patent ductus arteriosus in neonates with severe respiratory disease

Daily aortic contrast echo studies were carried out in 200 neonates with severe respiratory disease to determine the natural history of the ductus arteriosus during the first days of life and the effect of patency on subsequent morbidity and mortality. Decisions related to surgical or pharmacologic closure of a PDA were not based on the results of the contrast echo studies. The risk that intervention would be required was greater in infants weighing less than 1500 gm (P less than 0.005) and in patients in whom the ductus had not closed spontaneously by day 3 (P less than 0.001) regardless of birth weight. The clinical course in infants with a PDA on the third day of life revealed a lower survival rate (P less than 0.005), a greater requirement for prolonged respiratory support (P less than 0.005), and a higher incidence of bronchopulmonary dysplasia (P less than 0.005) in all birth weight categories. Ductal patency often was not associated with early clinical manifestations. No infant who had a negative echo study on the third day of life subsequently developed signs or symptoms of patent ductus arteriosus. We conclude that patency of the ductus arteriosus is a negative prognostic factor in the severely ill neonate with respiratory distress.