10:50 AM - 11:50 AM
[III-CHSS-03] Current surgical outcomes of congenital heart surgery for patients with Down syndrome : Scientific report using JCCVSD
Background: Current surgical outcomes of congenital heart surgery for patients with Down syndrome is unclear.
Methods: Of 29087 registered operations between 2008 and 2012 in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures: ventricular septal defect repair (n= 752), atrioventricular septal defect repair (n= 452), patent ductus arteriosus closure (n= 184), atrial septal defect repair (n= 167), tetralogy of Fallot (TOF) repair (n= 108), as well as 2 major single ventricular palliations: bidirectional Glenn (n= 21) and Fontan operation (n= 25) were selected and their outcomes were compared.
Results: The 90-day and in-hospital mortalities of all 5 major biventricular repair procedures were similarly low in patients with Down syndrome compared to patients without Down syndrome. On the other hand, mortality after Fontan operation in patients with Down syndrome was significantly higher than in patients without Down syndrome (2.7% vs 12.0%, p= 0.005), nevertheless all patients with Down syndrome showed preoperative pulmonary vascular resistance (PVR) of less than 4 WU/m2.
Conclusions: Although intensive management for pulmonary hypertension was essential, the analysis of JCCVSD revealed that favorable early prognostic outcomes after 5 major biventricular procedures in patients with Down syndrome. Mortality after Fontan operation was still high, but seemed improving.
Methods: Of 29087 registered operations between 2008 and 2012 in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures: ventricular septal defect repair (n= 752), atrioventricular septal defect repair (n= 452), patent ductus arteriosus closure (n= 184), atrial septal defect repair (n= 167), tetralogy of Fallot (TOF) repair (n= 108), as well as 2 major single ventricular palliations: bidirectional Glenn (n= 21) and Fontan operation (n= 25) were selected and their outcomes were compared.
Results: The 90-day and in-hospital mortalities of all 5 major biventricular repair procedures were similarly low in patients with Down syndrome compared to patients without Down syndrome. On the other hand, mortality after Fontan operation in patients with Down syndrome was significantly higher than in patients without Down syndrome (2.7% vs 12.0%, p= 0.005), nevertheless all patients with Down syndrome showed preoperative pulmonary vascular resistance (PVR) of less than 4 WU/m2.
Conclusions: Although intensive management for pulmonary hypertension was essential, the analysis of JCCVSD revealed that favorable early prognostic outcomes after 5 major biventricular procedures in patients with Down syndrome. Mortality after Fontan operation was still high, but seemed improving.