Evaluation of cardiac functions with Doppler echocardiography in children with Down syndrome and anatomically normal heart.

OBJECTIVE: To study the cardiac functions in Down syndrome children who did not have structural cardiac lesion by conventional and tissue Doppler echocardiography. MATERIALS AND METHODS: A total of 85 children with Down syndrome without anatomic heart disease and 50 normal control children were subjected to the assessment of right and left ventricular functions by both two-dimensional and tissue Doppler echocardiography. RESULTS: Children with Down syndrome had significantly higher left ventricular ejection fraction detected by two-dimensional echocardiography and left ventricular diastolic dysfunction detected by tissue Doppler than observed in the controls. In addition, children with Down syndrome also had right ventricular systolic and diastolic dysfunctions. Children with Down syndrome had significantly higher pulmonary artery systolic pressure than the control children. There was no significant difference in the cardiac functions between children with non-disjunction Down syndrome and those with the translocation type. CONCLUSION: Despite an apparently normal heart, children with Down syndrome may have silent disturbed cardiac functions, which may be detected by two-dimensional or tissue Doppler echocardiography. This may have an important clinical implication, especially before involving Down syndrome children in surgery or strenuous exercise.

Regulated upon activation, normal T-cell expressed and secreted chemokine and interleukin-6 in rheumatic pulmonary hypertension, targets for therapeutic decisions.

BACKGROUND: Recent studies have highlighted the possible influence of chemokines and cytokines on several types of pulmonary arterial hypertension (PAH). Increasing interest has also been focussed on their role as a cause of post-cardiopulmonary bypass (CPB) organ dysfunction. HYPOTHESIS: Chemokines and cytokines are involved in the pathobiology of rheumatic PAH. METHODS: Serum levels of the chemokine, regulated upon activation, normal T-cell expressed and secreted (RANTES) and the cytokine interleukin-6 (IL-6) were measured by enzyme-linked immunosorbent assay (ELISA) in 35 patients with rheumatic mitral valve disease and 10 matched healthy subjects (control group). Eleven patients (31.4%) had severe pulmonary hypertension. Subsequently, 23 patients underwent mitral valve replacement. The relation of RANTES and IL-6 circulating level with postoperative organ dysfunction was analysed through multiple organ dysfunction score (MODS). RESULTS: Patients with severe PAH have a significantly higher mean serum level of RANTES compared with other patients (6138.6+/-3543.8 pg/ml vs 1818.2+/-475.2 pg/ml, p=0.0003). The serum level of IL-6 in the patients was statistically different from that of the control (378+/-50.8 pg/ml vs 262+/-90.5 pg/ml, respectively, p=0.002). Patients who required postoperative inotropes had higher preoperative and post-CPB levels of both RANTES and IL-6. While patients with postoperative lung dysfunction had higher levels of IL-6 preoperatively and post-CPB and lower levels of RANTES post-CPB. CONCLUSIONS: RANTES and IL-6 should be investigated as potential therapeutic targets in the control of rheumatic PAH. Improved understanding of the contribution of RANTES and IL-6 to adverse postoperative complications can lead to improved patient outcome.