Saddle arterial embolus in a patient with Down syndrome.

We report acute thromboembolic events in a 14-year-old boy with Down syndrome and repaired atrioventricular septal defect. He presented with sudden onset of bilateral lower limb ischemia. Transesophageal echocardiography detected a thrombus in the right atrium. An arterial saddle embolus was removed following bilateral iliac embolectomy. Despite anticoagulation, he presented again with sudden bilateral lower limb ischemia and respiratory distress. Multiple pulmonary emboli and a thrombus in the right atrium were noted on imaging studies. An arterial embolus was removed from the abdominal aorta at the bifurcation. To our knowledge, this is the first report of a child or adolescent with a repaired congenital heart lesion and arterial embolism requiring embolectomy. This association and possible etiological factors are discussed.

Cardiovascular changes in children with mucopolysaccharide disorders.

UNLABELLED: The aims of this study were to evaluate cardiac involvement, assess risk factors and mortality, and define the outcome of cardiac abnormalities with age in the different types of mucopolysaccharidoses (MPS). The echocardiograms of 99 patients with MPS, aged 1-24 y (median age 10.3 y) were reviewed between 1978 and 2000. Mitral regurgitation (MR) was detected in 29 patients (29%). MR was more frequent in types IH [n = 11 (38%)], II [n = 10 (24%)] and III [n = 4 (20%)]. Sixteen patients (16%) developed aortic regurgitation (AR), seen mostly in types II [n = 9 (56%)] and IV [n = 4 (24%)]. AR and/or MR was detected in 37 patients and 8 had both abnormalities of borderline significance (odds ratio 2.95, 95% confidence interval 1.0-8.85, p = 0.05). Of 99 patients, 47 had a normal study on their first echocardiogram, whereas only 7 had a normal study on subsequent echocardiograms. Fifty-four (54%) had a single echocardiogram. Of these, 27 (50%) were abnormal and 27 normal. Forty-five patients had more than one echocardiogram, of which 25 (56%) were abnormal and 20 normal. In 13/20 (65%) a cardiac abnormality developed on a subsequent echocardiogram which was statistically significant (p = 0.002). Overall mitral and aortic valve abnormalities showed a positive association with age. Univariate analysis of risk factors showed that increasing age, MPS I and ejection fraction were significant risk factors for death. However, left ventricular hypertrophy, mitral valve abnormalities and type II MPS were not significant risk factors for death, with borderline significance for aortic valve abnormalities. CONCLUSION: This study demonstrates the evaluation of ventricular function, which is a significant risk factor for death, along with increasing age and MPS I, and outlines the borderline significance of aortic valve abnormalities, which has not been mentioned in previous studies. It also shows that mitral valve lesions, commonly seen in MPS, were not a significant risk factor for death. The results emphasize the importance of performing serial echocardiograms in patients with MPS to assess ventricular function and the progression of cardiac abnormalities with age.

Noninvasive measurement of cardiac output in critically ill children.

This study was performed to evaluate the hemodynamic status of children admitted to the intensive care unit, using suprasternal and transesophageal Doppler ultrasound, and to establish a suitable noninvasive technique to monitor trends in cardiac output in critically ill children. Twenty children were studied over a period of 6 months. The median age was 32.5 months and weight 14.5 kg. Minute distance (MD), which is a linear cardiac output parameter, was assessed. Seven simultaneous pairs of measurements of MD were made using transesophageal Doppler (TED) and suprasternal Doppler (SSD) by the same operator. Following a fluid challenge, seven repeat pairs of measurements were made. The mean percentage changes for MD by TED and SSD were 21.84 (SD 9.97) and 5.75 (SD 7.32). The average coefficients of variation for measurements of MD by TED and SSD were 2.34% and 15.98%, respectively. The mean difference in percentage change between MD, measured by TED and SSD, was 27.59 with a 95% confidence interval and wide limits of agreement. The repeatability of TED measurements was good, but the measurements by SSD were wide and erratic with poor reproducibility. Our study shows that TED is easy to use, reliable, and very useful for monitoring hemodynamic changes in critically ill children.