Clinical Course of Atrioventricular Septal Defect(AVSD) in Down and non Down syndrome

ABSTRACT

PURPOSE: It is well known that 1/3 - 1/2 of Atrioventricular septal defect(AVSD) patients also have Down syndrome which may influence the clinical course of AVSD. To know the anatomic type of AVSD and the effect of Down syndrome on the clinical course of AVSD, we studied 37 cases( Down group 14 cases, non Down group 23 cases) who were diagnosed and followed at Department of Pediatrics, Kyungpook National University Hospital between Jan. 1987 and Dec. 1994. METHODS: Patients were diagnosed to have AVSD by 2D-echocardiography and Down syndrome by chromosome study. In 17 cases we catheterizes, Qp/Qs, Rp/Rs and Pp/Ps were compared according to the group. In 14 cases we operated on, the durations of ventilator therapy, ICU stay, and hospital admission were also compared. RESULTS: Of the 36 cases with AVSD, Down syndrome were 14 cases(38%). In Down group(14 cases), complete type of AVSD were 6 cases(43%, Rastelli A 4 cases, Rastelli B 1, Rastelli C 1) and incomplete type of AVSD were 8 cases(57%, Atrial septal defect only 4 cases, Ventricular septal defect only 4). In non Down group(23 cases), complete type of AVSD were 10 cases(42%, Rastelli A 7 cases, Rastelli B 2, Rastelli C 1) and incomplete type of AVSD were 13 cases(58%, Atrial septal defect only 10 cases, Ventricular septal defect only 3). Of the 17 cases we catheterized(Down group 9 cases, mean age 2.1+/-2.0 years, non Down group 8 cases, mean age 2.7+/-3.9 years), Qp/Qs was significantly high in non Down group(1.42+/-0.60 vs 2.61+/-1.31, p<0.05) but Rp/Rs and Pp/Ps were significantly high in Down group(Rp/Rs 0.34+/-0.31 vs 0.10+/-0.06, Pp/Ps 0.68+/-0.28 vs 0.31+/-0.09, P<0.05 respectively). Of the 14 cases we operated on(Down group 6 cases, non Down group 8 cases), the duration of ventilator therapy, ICU stay and hospital admission were not significantly different between both groups but tended to be long in Down syndrome(ventilator therapy 5.3+/-2.5 days vs 4.3+/-6.7, ICU stay 9.0+/-6.1 days vs 3.0+/-2.3, hospital admission 29.4+/-25.9 days vs 21.9+/-27.4). CONCLUSIONS: Down syndrome may adversely influencd the course of atrioventricular septal defect hemodynamically and clinically.