Sudden unexpected death in children with congenital heart defects.

AIMS: Congenital heart defects (CHDs) are the most common birth defects and are an important cause of death in children. The fear of sudden unexpected death has led to restrictions of physical activity and competitive sports. The aim of the present study was to investigate the rate of sudden unexpected deaths unrelated to surgery in children 2-18 years old with CHDs and, secondarily, to determine whether these deaths were related to cardiac disease, comorbidity, or physical activity. METHODS AND RESULTS: To identify children with CHDs and to determine the number of deaths, data concerning all 9 43 871 live births in Norway in 1994-2009 were retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospital's Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012, and information for the deceased children was retrieved from medical records at Norwegian hospitals. Among 11 272 children with CHDs, we identified 19 (0.2%) children 2-18 years old who experienced sudden unexpected deaths unrelated to cardiac surgery. A cardiac cause of death was identified in seven of these cases. None of the children died during physical activity, whereas two children survived cardiac arrest during sports. CONCLUSION: Sudden unexpected death was infrequent among children with CHDs who survived 2 years of age. Comorbidity was common among the children who died. This study indicates that sudden unexpected death in children with CHDs rarely occurs during physical activity.

[Transcatheter closure of atrial septal defects in the oval fossa]. / Kateterlukking av atrieseptumdefekter.

BACKGROUND: We report five years' experience with transcatheter closure of defects in the oval fossa at Rikshospitalet University Hospital in Oslo, Norway. MATERIAL AND METHODS: We reviewed the results in 69 patients between 1.3 and 74 years of age (median 5.3), weighing median 20 kg (7.8-109), with pulmonary blood flow 2.40.74 (1.3-4.8) times systemic flow, and median diameter of the defect 18 mm (9-34). The patients were followed clinically for one year. RESULTS: In 69/77 (90%) the defect could be closed. None had a significant residual shunt. One patient showed a very small residual leak after one year. Two children, not yet seen after one year, had a small shunt after one month. The complication rate has been low, and we have not been able to demonstrate any sequelae. The hospital stay is two nights without any convalescence period. The results compare favourably to surgery. INTERPRETATION: The method was efficient and safe in all age and weight groups. The very limited total volume of such procedures indicates they should be centralised and only carried out by a small number of physicians.

An extensive long term follow-up of a cohort of patients with hypoplasia of the left heart.

We report the results of follow-up of the complete cohort of Norwegian children born in the period from 1987 through 1998 in whom there was the intention to treat surgically hypoplasia of the left heart using the Norwood sequence of operations. Of the 54 children, 21 are alive. Of these, 15 have been extensively studied, while the medical state of all the remaining survivors is known from reports from other hospitals. Of the survivors, the majority have reasonably acceptable cardiac and haemodynamic function, but significant neurological and neuropsychological morbidity is identified within the group as a whole, which requires special attention from qualified personnel of various kinds.

A population-based study relevant to seasonal variations in causes of death in children undergoing surgery for congenital cardiac malformations.

AIMS: Our objectives were, first, to study seasonal distribution of perioperative deaths within 30 days after surgery, and late death, in children undergoing surgery for congenitally malformed hearts, and second, to study the causes of late death. METHODS: We analysed a retrospective cohort of 1,753 children with congenital cardiac malformations born and undergoing surgery in the period from 1990 through 2002 with a special focus on the causes of late death. The data was obtained from the registry of congenital cardiac malformations at Rikshospitalet, Oslo, and the Norwegian Medical Birth Registry. The mean follow-up from birth was 8.1 years, with a range from zero to 15.2 years. RESULTS: During the period of follow-up, 204 (11.6%) of the children died having undergone previous surgery. Of these 124 (7.1%) died in the perioperative period, and 80 (4.5%) were late deaths. There were 56 late deaths during the 6 coldest months, compared with 24 during the 6 warmest months (p < 0.01). There was no significant seasonal variation in perioperative deaths. Respiratory infection was the most common cause of late death, and occurred in 25 children, of whom 24 died during the 6 coldest months. Of the 8 sudden late deaths, 7 occurred during the 6 coldest months. There was no seasonal variation for the other causes of death. CONCLUSIONS: In children undergoing surgery for congenital cardiac malformations in Norway, there is a seasonal variation in late death, with a higher proportion occurring in the coldest months. Death related to respiratory infections predominantly occurs in the winter season, and is the overall most common cause of late death.

A population-based study of extra-cardiac anomalies in children with congenital cardiac malformations.

We describe the prevalence of extra-cardiac anomalies in children with congenital cardiac malformations, and their impact on survival, compared to the outcome in children with the congenital cardiac lesions as the only recognised anomaly. Our population comprises the 3527 children born with congenital cardiac anomalies between 1990 and 1999, and registered at the largest tertiary centre for Paediatric Cardiology in Norway. Extra-cardiac anomalies were found in one-fifth of the population, with Down's syndrome accounting for nearly one-third. Survival improved for children born between 1995 and 1999 compared with those born in the period from 1990 to 1994 for all groups, except for children with additional extra-cardiac anomalies in the absence of Down's syndrome. The results were the same for children undergoing surgical treatment of their cardiac malformation. The survival in children with Down's syndrome improved in comparable fashion to those without extra-cardiac anomalies. Children with extra-cardiac anomalies in the absence of Down's syndrome represent a heterogeneous group, with varying patterns of survival. Survival did not improve in these latter patients during the period of our study.