Long-term results of relief of subaortic stenosis in univentricular atrioventricular connection with discordant ventriculoarterial connections.
BACKGROUND
We set out to examine the long-term results of relief of subaortic stenosis by enlargement of ventricular septal defect in patients with univentricular atrioventricular connection to a dominant left ventricle and discordant ventriculoarterial connections.


METHODS
Twenty-four patients underwent enlargement of ventricular septal defect between 1985 and 1998 at a median age of 3.2 years (range, 3 weeks to 14 years). Ten patients were younger than 1 year of age. Eighteen had undergone previous banding of the pulmonary trunk, 9 of whom also required repair of coarctation of the aorta. The median subaortic gradient before enlargement was 46 mm Hg. Twenty-three patients had a patch to enlarge the rudimentary right ventricle.


RESULTS
Five patients (21%) died in the early postoperative period. The overall survival at 1 and 3 years was 73%, and at 5 and 10 years was 68% and 60%, respectively. Complete heart block requiring insertion of a pacemaker occurred in 2 patients (8%). A Fontan operation was performed in 10 patients, 5 underwent a bidirectional Glenn procedure, and 2 required cardiac transplantation. Follow-up was complete in all survivors at a median time of 6.7 years (range, 8 months to 13 years). From the earlier part of the series, 3 patients experienced aortic insufficiency and 2 had recurrent obstruction. Factors adversely affecting survival were age younger than 1 year at operation and presence of obstruction within the aortic arch.


CONCLUSIONS
Our experience shows that, in patients with univentricular atrioventricular connection to a dominant left ventricle and subaortic stenosis, enlargement of the ventricular septal defect provides satisfactory relief of obstruction except in those younger than 1 year of age, and those who have associated obstruction in the aortic arch.
Department of Cardiology and Cardiac Surgery, Royal Brompton Hospital, London, England.
M
M
Jahangiri
E A
EA
Shinebourne
D B
DB
Ross
R H
RH
Anderson
C
C
Lincoln
D000714:Anastomosis, Surgical / Q000009:adverse effects; D001024:Aortic Valve Stenosis / Q000401:mortality / Q000601:surgery*; D006325:Heart Atria / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006352:Heart Ventricles / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011183:Postoperative Complications / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate; D013997:Time Factors