As far as we are aware, ours is the first epidemiologic study concerning the frequency and type of congenital cardiac disease found in Guatemalan children with Down's syndrome. The high frequency of patency of the arterial duct, and the differential distribution of the cardiac malformations associated with Down's syndrome among Guatemalan children, differ from what has been reported in the United States of America, Europe, and Asia. This difference warrants further research.

Background— In 1997, an effort was made to disseminate US pediatric cardiac surgical practices to create a new comprehensive program in Guatemala. The objective of this study was to describe the improvement of the program by exploring the reduction in risk-adjusted in-hospital mortality. Methods and Results— A retrospective cohort study of surgical procedures performed in Guatemala from February 1997 to July 2004 was conducted. Data were divided into 3 time periods (1997 to 1999, 2000 to 2002, and 2003 to 2004) and compared with a US benchmark (2000 Kids’ Inpatient Database of 27 states and 313 institutions). The risk adjustment for congenital heart surgery (RACHS-1) method was used to adjust for case mix. Mortality rates, standardized mortality ratios, and 95% confidence intervals were calculated. A total of 1215 surgical procedures were included. Median age was 3.1 years (range, 1 day to 17.9 years). The overall mortality was 10.7% (n=130). The RACHS-1 method showed better discrimination than in prior reports (area under receiver operating characteristic curve=0.854). A decreasing trend in mortality rate was observed in every RACHS-1 risk category over the 3 time periods. When compared against the US benchmark, the reduction in risk-adjusted mortality was noted by a decrease of standardized mortality ratio from 10.0 (95% confidence interval, 7.2 to 13.7) in 1997–1999, to 7.8 (95% confidence interval, 5.9 to 10.0) in 2000–2002, and to 5.7 (95% confidence interval, 3.8 to 8.3) in 2003–2004. Conclusions— In a short time period, mortality after congenital heart surgery has been reduced substantially in Guatemala. Measurement of risk-adjusted mortality is a useful method to assess pediatric cardiac program improvement in the developing world.

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A bilateral bidirectional cavopulmonary shunt was performed in a cyanotic 14-month-old girl who had tricuspid and pulmonary valve atresia, with right pulmonary artery (RPA) hypoplasia (3 mm), bilateral superior vena cavae and a ductus arteriosus-dependent pulmonary blood flow. Because of 62% postoperative arterial oxygen saturation and a right superior vena cava (RSVC) pressure of 30 mmHg, a 5 mm Gore-Tex tube was interposed to connect the two superior venae cavae. The creation of a 'new-innominate' vein allowed decompression of the right superior vena cava and an increase in arterial oxygen saturation to 86%.