Impaired weight gain predicts risk of late death after surgery for congenital heart defects.

OBJECTIVES: To describe long-term somatic growth in terms of weight for age in children operated on for congenital heart defects who die late (after the first 30 postoperative days) and to study the relationship between postoperative weight gain and survival after surgery for congenital heart defects. METHODS: This was a nested case-control study of 80 children born in 1990-2002 who died late after surgery for congenital heart defects at Rikshospitalet, Norway. Weight data were obtained for 74 children, of whom 31 with no extra-cardiac anomalies were defined as cases and 31 surviving children with similar surgical complexity were defined as controls. RESULTS: In the 74 children who died late, mean weight for age converted to z scores at birth, at last operation and at last recorded weight were 0.12, -1.31 and -2.09. In the 31 children defined as cases, the same weight z scores were 0.07, -1.21 and -2.01 compared with 0.05, -1.10 and -0.99 in the 31 matched controls. The odds ratio (OR) for death was 13.5 (95% CI 3.6 to 51.0) if there was a decrease in weight z score of >0.67 after the last operation. Median follow-up time after operation was 5.7 months. CONCLUSIONS: A decrease in weight for age during the first months after surgery for congenital heart defects of more than 0.67 z scores, corresponding to a downward percentile crossing through at least one of the displayed percentile lines on standard growth charts, is strongly related to late mortality in children operated on for congenital heart defects.

Psychosocial functioning in children after transplantation of the heart, and heart and lungs.

We studied 10 boys, and 15 girls, all below the age of 16, who had been referred to the National Hospital in Norway for evaluation for transplantation of either the heart, or the heart and lungs. These represent the complete cohort of patients being considered for transplantation between 1990 and 1997. Of the 25 children and their families, 24 sets underwent a comprehensive psychosocial assessment, including interviews with both parents and their children. The parents completed the Child Behavior Checklist and the General Health Questionnaire. We had accepted 15 children for transplantation and placed them on the waiting list. The others were rejected for medical reasons, and 3 died whilst waiting for an organ. One was reconsidered for conventional surgery and removed from the list. Transplantation was performed in 11 children, whilst one of the patients we had rejected underwent transplantation abroad, and was included in the study. This left 12 patients in the final sample, with a mean age of 8 years, and with a range from 11 months to 13.9 years. We reassessed their psychosocial and physical functioning two years after transplantation. Of those undergoing transplantation of the heart and lungs, two were severely affected by progressive obliterative bronchiolitis. The others were in good general physical condition. At the assessment prior to transplantation, three already fulfilled the criterions for diagnosis of an overanxious disorder. Two others had symptoms of anxiety and depression, but without fulfilling the accepted criterions. At follow- up, two patients retained this psychiatric diagnosis. Increased levels of stress were uncovered in the parents prior to surgery, but these had normalised at follow-up. The study shows that, in general, physical and psychological conditions improve in children undergoing transplantation, but they and their parents live in a stressful environment, and are in need of psychosocial support both before and after transplantation.

[Catheter closure of open ductus arteriosus--the first 100 patients]. / Kateterbasert lukking av åpen ductus arteriosus--de første 100 pasienter.

BACKGROUND: Patent ductus arteriosus is increasingly treated with catheter based techniques. We present our results as by the end of 1998 of the first 100 patients given such treatment. MATERIAL AND METHODS: The 100 patients between 0.6 and 31.4 years were initially treated with one of these devices: The Rashkind umbrella (60 patients), Cook PDA coils (31 patients) or Amplatzer ductal occluders (ten patients). Seven umbrella patients were treated twice, one with an additional umbrella, six with coils; two coil patients twice, one with another coil and one--after embolisation--with a peg. RESULTS: The overall complete closure rate for all groups was 90% after nine patients had been treated a second time. The primary complete closure rate in pegs was 100%, after reintervention 85% in umbrella and 97% in coil patients. Neither umbrellas nor pegs have embolised. One coil embolised in the course of implantation of 40 coils (2.5%). No other complication has occurred. INTERPRETATION: The closure of the arterial duct with catheter techniques compares favorably with surgery and is now established as the method of choice. Following the investigational introduction of pegs there is no longer an upper limit in the size of ducts suitable for such treatment.

BERTE--a database for children with congenital heart defects.

To secure the follow-up of children with congenital heart defects in Norway the Dept. of Pediatric Cardiology has developed a tailor-made database. This also simplifies the regular flow of information about these patients both inside the hospital and between the hospital and the others who are in charge of the patient care on various levels.

[Hypoplastic left heart syndrome. Present experiences and future possibilities]. / Hypoplastisk venstre hjerte-syndrom. Erfaringer hittil og mulige veier videre.

The authors report experiences from Norwood's staged palliation for hypoplastic left heart syndrome performed in Philadelphia on Norwegian children. Although there is no universal agreement that these children should be operated at all, the Norwegian health authorities have decided that all expenses for transport, examination and treatment shall be covered by the National Health Insurance Scheme. Since 1987 a total of 21 children have been transported to USA for operation. Eight of these children have completed all the planned stages of the operative treatment. Two are waiting for stage 3. 11 children have died. The authors discuss how to proceed with regard to treatment of this complicated patient group in future.

Pulmonary vascular resistance in complete atrioventricular septal defect. A comparison between children with and without Down's syndrome.

Invasive data obtained in children with complete atrioventricular septal defect over the last three years are presented to determine the age at which pulmonary vascular obstructive disease develops. Comparisons were made between children with (n = 21) and without Down's syndrome (n = 12). The investigation was restricted to patients less than one year of age at initial catheterization. Patients with complicated associated heart defects were excluded. Patients with Down's syndrome had a higher ratio of pulmonary to systemic vascular resistance than children without Down's syndrome in the basal situation. This difference almost disappeared after 100% oxygen had been given to patients with elevated pulmonary vascular resistance, indicating that hypoxia and/or hypoventilation is of importance, especially in children with Down's syndrome. Fixed elevated pulmonary vascular resistance was found in 11% of Down patients under one year, with the youngest patient being 5.5 months old. One child without Down's syndrome may have had fixed elevated pulmonary vascular resistance. It is concluded that all children with complete atrioventricular septal defect should be evaluated and operated on at a very young age.

Aortic wall lesion in balloon dilatation of coarctation of the aorta.

A successful balloon dilatation of a native coarctation of the aorta was done in a five-day-old child. Two days later she was operated upon because of critical aortic stenosis, but died. The post-mortem examination showed a lesion of the intima and media at the coarctation site, and it seemed that the adventitia alone was what prevented rupture of the vessel.