COMPARISON OF HEMODYNAMIC DATA BEFORE AND AFTER CORRECTIVE SURGERY FOR DOWNS-SYNDROME AND VENTRICULAR SEPTAL-DEFECT

Left ventricular function and the extent of pulmonary vascular disease were studied in 18 children with Down's syndrome and 20 children with out Down's syndrome who underwent corrective surgery for ventricular s eptal defect (VSD) and severe pulmonary hypertension. This study was c onducted between 1985 and 1993. All patients underwent routine cardiac catheterization preoperatively and postoperatively (mean, 11.4 months after surgery). Left ventricular function was estimated using cineang iographic levograms. In both groups, the pulmonary-to-systemic arteria l pressure ratio (Pp/Ps) and pulmonary vascular resistance (PVR) were significantly lower after surgery (P < 0.05). Postoperative improvemen t was more remarkable in the non-Down group (P < 0.05). Left ventricul ar end-diastolic volume (percent of normal) (LVEDV%N) was significantl y lower after surgery in both groups (P < 0.01). The left ventricular stroke work-to-end-diastolic volume ratio (LVSW/EDV) was significantly higher after surgery in the non-Down group only (P < 0.01). Postopera tive left ventricular ejection fraction (LVEF) was significantly lower in the Down group than in the non-Down group (P < 0.01). Some degree of irreversible pulmonary vascular disease was present after repair of VSD in patients with Down's syndrome. In the Down group, there were n o significant changes in left ventricular function after surgery, desp ite the relief of volume overload. These results suggest that early di agnosis and surgical repair are key elements in the management of pati ents with Down's syndrome and VSD.