Double-inlet ventricle presenting in infancy. II. Results of palliative operations.
The influence of palliation on survival was studied in 191 consecutive infants, presenting at under 1 year of age, with double-inlet ventricle (1973 to 1988, median follow-up 8.5 years). Palliative operations were performed on 154 occasions in 121 patients (63%). Survival after a systemic-pulmonary arterial shunt (n = 57) and banding of the pulmonary trunk (n = 35) was comparable (84% and 77% at 1 year, 62% and 45% at 5 years), but those who underwent repair of aortic arch obstruction fared worse (n = 18, 44% and 22% at 1 and 5 years, p less than 0.001). The remainder did not undergo an operation because of balanced physiology (n = 17, 9% of entire group), complex anatomy (n = 32, 15%), or irreversible low output (n = 19, 12%). Palliative surgery, overall, had a deleterious effect on immediate survival (greater than 1 month relative risk 6.6, p less than 0.001), but, in the survivors, medium-term outcome was improved (greater than 6 months, 0.68, p less than 0.05). This effect was most marked for those undergoing a systemic-pulmonary artery shunt (less than 1 month, 2.52; greater than 6 months, 0.43); by contrast, after banding of the pulmonary trunk, with or without additional repair of the aortic arch repair, medium-term risk was not altered (greater than 6 months, 1.13 and 0.91, respectively). These data will assist the clinician in making decisions concerning the management of infants with double-inlet ventricle and in the judicious use of palliative surgery.
Franklin
R C
RC
Thoracic Unit, Hospital For Sick Children, London, England.
Spiegelhalter
D J
DJ
Anderson
R H
RH
Macartney
F J
FJ
Rossi Filho
R I
RI
Rigby
M L
ML
Deanfield
J E
JE
D000714:Anastomosis, Surgical; D001017:Aortic Coarctation / Q000601:surgery; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000401:mortality / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D006801:Humans; D007223:Infant; D015999:Multivariate Analysis; D010166:Palliative Care; D011651:Pulmonary Artery / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate