Naturalistic study of course, effectiveness, and predictors of outcome among female adolescents in residential treatment for eating disorders.

Few empirical data address naturalistic outcomes of residential eating disorder (ED) treatment. Study aims were to evaluate course, effectiveness, and predictors of outcome in a residential treatment program. We evaluated 80 consecutively admitted female adolescents with the SCID-IV. Primary outcomes were treatment completion, subsequent readmission, clinical global impressions, and changes in body weight. Mean length of stay was 51 days, and 80% of patients were discharged according to treatment plans. Mean expected body weight (EBW) for AN patients increased from 80% to 91%. Patients reported significant improvements in ED symptoms, depression, and quality of life. Low admission %EBW and previous psychiatric hospitalizations were associated with premature termination. Overall, findings support that residential treatment is largely acceptable to patients, and that residential care may provide an opportunity for substantive therapeutic gains.

Influence of ventricular morphology on outcome after the Fontan procedure.

The modified Fontan procedure has gained wide acceptance in the treatment of various congenital heart defects. Determination of risk factors for mortality remains an important issue for optimizing patient selection for the Fontan procedure. Conflicting results have been reported about whether ventricular morphology is a risk factor in these patients. Survival free of Fontan takedown or cardiac transplantation was assessed in the first 500 patients undergoing the Fontan procedure at our institution. This survival was correlated with ventricular morphology as evaluated by angiography. Both multivariate and univariate analyses indicated ventricular morphology was predictive of early survival free of Fontan takedown or cardiac transplantation following the procedure. However, there was no statistical evidence for ventricular morphology being a risk factor for mortality in patients alive 6 months after the procedure. Ventricular morphology is a risk factor for early survival in patients undergoing a Fontan procedure, with left ventricular morphology associated with a better early survival than right ventricular morphology.

Repair of coarctation with resection and extended end-to-end anastomosis.

BACKGROUND: Our surgical strategy for infant coarctation changed from subclavian flap aortoplasty to resection with extended end-to-end anastomosis in 1991. The purpose of this review was to evaluate the results of that strategy. METHODS: From 1991 through 1997, 55 infants underwent repair of coarctation of the aorta using resection with extended end-to-end anastomosis. Isolated coarctation of the aorta was present in 26 patients, 20 patients had a ventricular septal defect, and 9 patients had other associated intracardiac lesions. Mean age at surgery was 0.20+/-0.24 years (median, 21 days). In 34 patients (62%), arch reconstruction was performed through a left thoracotomy. Twenty patients (36%) had median sternotomy with simultaneous repair of coarctation of the aorta and intracardiac repair of associated lesions. One patient had recoarctation repair through a median sternotomy. All coarctation and ductal tissue was resected and the anastomosis was constructed starting opposite the left carotid artery with running polypropylene suture. RESULTS: There was one early death 26 days after coarctation of the aorta and ventricular septal defect repair in a child on extracorporeal membrane oxygenation for meconium aspiration and 2 late deaths owing to pneumonia and pulmonary hypertension (1) and interventricular hemorrhage (1). There were no instances of paraplegia. Follow-up in survivors ranges from 10 to 76 months (mean, 39.8+/-17.2 months). Recoarctation has developed in 2 patients, who have had successful balloon dilation 6 and 14 months after the operation. This yields a low recoarctation rate of 3.6%. CONCLUSIONS: Resection with extended end-to-end anastomosis yields a low mortality and particularly a low recoarctation rate and is our procedure of choice for infants with coarctation of the aorta.

Angiographic determination of ventricular morphology: correlation with pathology in 36 hearts with single functional ventricles.

Among 36 patients with univentricular atrioventricular connections, 27 had left ventricular and 9 right ventricular morphology. None had a common or indeterminate ventricle on autopsy review. Angiographic analysis correctly determined ventricular morphology in 34 (94%) of 36 patients. All 9 patients with right ventricular morphology of the dominant ventricular chamber were correctly identified angiographically. Altogether 25 of 27 patients (93%) with left ventricular morphology of the dominate ventricular chamber were correctly identified angiographically. Two patients with left ventricular morphology and severely dilated ventricles were incorrectly classified as having right ventricular morphology by angiographic assessment. For patients with a single functional ventricle, we conclude that angiographic assessment is an excellent method for determining ventricular morphology.

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. METHODS AND RESULTS: Between 1979 and 1993, 125 infants and children underwent PTFE patch aortoplasty for CoAo; 111 of the procedures were primary repairs, and 14 were reoperations. Diagnoses were isolated CoAo (96 patients), CoAo and ventricular septal defect (15 patients), and CoAo with complex intracardiac anomaly (14 patients). Patient age at the time of repair ranged from 4 days to 17 years (mean age, 5.1 +/- 4.5 years). There were no instances of intraoperative mortality or paraplegia. There were 4 deaths from 10 to 40 days postoperatively, all in neonates (mean age, 33 days) who received additional intracardiac procedures for complex associated anomalies. Follow-up has ranged from 6 months to 12.5 years (mean, 4.5 +/- 3.2 years). All children had postoperative chest roentgenograms, 80 (66%) patients have had a postoperative echocardiogram and 16 (13%) a cardiac catheterization. One patient had successful repair of a false aneurysm 4 months postoperatively. No patient has developed a late true aneurysm. Of the patients < 1 month of age at the time of CoAo repair (12 patients), 6 patients had recurrent CoAo (gradient > 20 mm Hg) compared with only 4 recurrences in 97 patients > 1 month of age at the time of repair (P < .001). CONCLUSIONS: For children > 1 year of age, PTFE patch aortoplasty remains our procedure of choice for CoAo repair because of the low mortality rate, low recoarctation rate, and absence of late true aneurysms. We have stopped using this technique for infants < 1 month of age because of the high recurrence rate.

Anomalous origin of the left coronary artery. A twenty-year review of surgical management.

Children with anomalous origin of the left coronary artery from the pulmonary artery are at risk for myocardial infarction and death. Surgical management of this condition in children has evolved significantly during the past 20 years. Between 1970 and 1990, a total of 20 of these patients underwent surgical intervention at two institutions. Age at operation ranged from 3 weeks to 11 years (mean, 26 months). Twelve patients had congestive heart failure, three were in cardiogenic shock, and two had cardiac murmurs. Operative techniques included ligation (n = 9), subclavian artery anastomosis (n = 5), aortic implantation (n = 3), internal mammary artery anastomosis (n = 1), intrapulmonary tunnel from aortopulmonary window to coronary artery (n = 1), and cardiac transplantation (n = 1). The three deaths in the series occurred at 3 weeks, at 2 months, and at 9 years after ligation. There have been no deaths after establishment of a two coronary artery system or after transplantation. Two of the five patients who had subclavian artery anastomosis to the anomalous coronary artery have severe anastomotic stenosis and collateralization. For patients with anomalous origin of the left coronary artery from the pulmonary artery, we recommend direct aortic implantation of the anomalous coronary artery at the time of diagnosis. Intrapulmonary tunnel from aortopulmonary window to coronary artery, or aorta-coronary bypass with internal mammary artery are recommended for children in whom aortic implantation is not anatomically feasible. Left coronary artery ligation is not indicated for these patients; those who have survived ligation should be considered for elective establishment of a two coronary artery system because of the risk of late death.

Detecting arrhythmia by exercise electrocardiography in pediatric patients: assessment of sensitivity and influence on clinical management.

We studied 49 patients with suspected arrhythmias (group 1) and 92 patients with previously documented arrhythmias (group 2) who underwent exercise electrocardiography (EECG) at our institution between 1979 and 1987. Among group 1 patients, all of whom had sinus rhythm before exercise, 10 (20%) had abnormal findings on EECG, and treatment was modified in 4 of these 10 (8% of group 1). Further testing (24-hour ambulatory or transtelephonic electrocardiographic monitoring or electrophysiologic study) of the 39 patients with normal EECG findings revealed 8 additional patients with arrhythmias. In group 1, the sensitivity of EECG was 56%, and its negative predictive value was 79%. Group 2 consisted of 38 patients with atrial arrhythmias, 31 with ventricular arrhythmias, and 23 with atrioventricular conduction abnormalities before EECG. Of these 92 patients, 68 (74%) had abnormal EECG findings. All but 1 of the 24 patients with normal findings underwent further testing, and rhythm abnormalities were induced in 16. Patients with atrial arrhythmias were more likely to have normal EECG results (42%) than were those with ventricular arrhythmias (23%) or an atrioventricular conduction abnormality (4%). Of the 35 patients who had been referred for suppression of an arrhythmia, 25 (71%) had abnormal rhythm suppressed during exercise. Clinical management was modified in 27% of group 2 patients on the basis of EECG findings.

National survey of diagnosis and management of persistent pulmonary hypertension of the newborn.

The diagnosis and management of persistent pulmonary hypertension of the newborn remains controversial. A national survey was performed to analyze recent trends in the incidence, diagnosis, management, and survival of patients with persistent pulmonary hypertension of the neonate. Sixty-six institutions from all geographical regions responded. The overall admission incidence was 3.9% +/- 2.6%. Secondary persistent pulmonary hypertension of the neonate was more common than primary. Unexplained hypoxemia, ductal level right-to-left shunting, echocardiography, and a positive response to hyperventilation were all used frequently (in at least 79% of institutions) to diagnose persistent pulmonary hypertension of the neonate. The majority of institutions considered a positive response to hyperventilation to be determined by an increase of PaO2 by 30 mm Hg with a concomitant decrease in PaCO2 to 25 mm Hg. Approximately 70% of institutions use varying ventilator techniques (ie, with or without hyperventilation), but the majority use hyperventilation predominantly. Almost all (greater than 90%) institutions used muscle paralytic agents and pulmonary vasodilators. Tolazoline was the first choice of pulmonary vasodilator therapy. The overall survival rate of persistent pulmonary hypertension of the newborn was 77.4% +/- 13.4%. Survival rate did not differ between different geographic areas of the country. There was a trend noted for improved survival with less use of muscle paralyzing agents. Yet despite varying treatment protocols, survival rates are improving.

Occurrence of congenital heart defects in siblings of patients with univentricular heart and tricuspid atresia.

Because of the rarity of univentricular heart and tricuspid atresia, no study of a large number of patients has been made to determine the empiric occurrence risks of a congenital heart defect in their siblings. A retrospective chart review was performed of 223 patients with univentricular heart and 113 with classic tricuspid atresia evaluated from 1982 to 1987. Thirty-four patients with univentricular heart and 17 with tricuspid atresia had no siblings and were excluded. Eleven of the total 388 siblings (2.8%) of the 189 patients with univentricular heart had a congenital heart defect. Of the siblings born after the index case of univentricular heart, 7 of 169 (4.1%) had a congenital heart defect. Two of the 210 siblings (1.0%) of the 96 patients with tricuspid atresia had a congenital heart defect. The patients with univentricular heart were subdivided into 4 groups: (1) double-inlet left ventricle; (2) complex univentricular heart with single or common inlet, or with a ventricle of common or right ventricular morphology; (3) complex univentricular heart with asplenia; and (4) complex univentricular heart with polysplenia. One of the 202 siblings (0.5%) of the 102 patients with double-inlet left ventricle had a congenital heart defect, compared with 7 of the 140 siblings (5.0%) of the 69 patients with complex univentricular heart, 1 of the 29 siblings (3.4%) of the 14 patients with complex univentricular heart and asplenia, and 2 of the 7 siblings (28.6%) of the 4 patients with complex univentricular heart and polysplenia.(ABSTRACT TRUNCATED AT 250 WORDS)