BackgroundThe prevalence of cardiovascular anomalies in Down's syndrome is well described, but there are few data on spectrum, management and outcome. The authors aimed to provide this information for infants with Down's syndrome in a defi ned population over a 22-year period. Methods The regional paediatric cardiology database in Newcastle upon Tyne provided information on all cardiovascular anomalies, surgical treatment and outcome. Data was subdivided into two eras,
While operative mortality following cardiac transplantation for adult congenital heart disease is higher than for other diagnostic groups, long-term survival is good and comparable to patients without CHD. Disappointing early results are improved with increasing experience.
A total of 10.4% of infants who died had a cardiovascular malformation and two-thirds of deaths were due to the malformation or its treatment. Mortality declined due to increasing termination of pregnancy and improved survival after operation.
Favourable cardiac transplantation outcomes can be achieved in adults with failing Fontan circulation. Patients with PVF may represent a distinct subset with more perturbed failing Fontan physiology and higher cardiac transplant mortality. We continue, however, to evolve and refine our strategies and are optimistic concerning future improvement in outcomes even in those with PVF.
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