Surgical outcome of double-outlet right ventricle with subpulmonary VSD.
BACKGROUND
Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration.


METHODS
Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow-up was 23 +/- 18 months.


RESULTS
An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn/Fontan group. Four patients in the ASO group died within 33 days postoperatively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular septal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO group, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%).


CONCLUSIONS
The ASO remains our preferred treatment for infants with double-outlet right ventricle and subpulmonary ventricular septal defect. However, associated anatomic defects are important risk factors.
Takeuchi
K
K
Department of Cardiac Surgery, The Children's Hospital, Boston, Massachusetts 02115, USA.
McGowan
F X
FX
Moran
A M
AM
Zurakowski
D
D
Mayer
J E
JE
Jonas
R A
RA
del Nido
P J
PJ
D002675:Child, Preschool; D004310:Double Outlet Right Ventricle / Q000150:complications / Q000401:mortality / Q000601:surgery*; D018729:Fontan Procedure / Q000379:methods; D018610:Heart Bypass, Right / Q000401:mortality; D006345:Heart Septal Defects, Ventricular / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis