INDIVIDUAL PULMONARY VEIN SIZE AND SURVIVAL IN INFANTS WITH TOTALLY ANOMALOUS PULMONARY VENOUS CONNECTION

Objectives. We investigated whether mortality in totally anomalous pul monary venous connection could be predicted from preoperative individu al pulmonary vein size. Background. Some infants with this anomaly die with or without surgical repair because of stenosis of individual pre dicted veins. Methods. Individual pulmonary vein, vertical vein and pu lmonary venous confluence diameters were retrospectively measured from preoperative echocardiograms in 32 infants with totally anomalous pul monary venous connection presenting to Children's Hospital, Boston ove r a 4 1/2-year period. Data on body surface area, other cardiac anomal ies, presence of initial pulmonary venous obstruction and early surger y and outcome were also recorded. Results. Of 32 patients, 6 (18.8%) d ied before hospital discharge, and 8 (25.0%) died subsequently. Six (7 5.0%) of the eight patients who died late had individual pulmonary vei n stenosis at sites remote from the surgical anastomosis to the left a trium. The remaining 18 patients (56.3%) are alive at a mean follow-up period of 9.7 months. A Cox proportional hazards model revealed that small sum of individual pulmonary vein diameters (p = 0.0004), small c onfluence size (p = 0.02) and presence of heterotaxy syndrome (p = 0.0 08) were each significant univariate predictors of survival. Multivari ate analysis showed that small pulmonary vein sum was a strong predict or of survival (p = 0.008), independent of the presence of heterotaxy syndrome. An analysis stratified by the presence of heterotaxy syndrom e showed that the predictive effect of small pulmonary vein sum on sur vival was strongest in patients without heterotaxy syndrome.Conclusion s. These data show that individual pulmonary vein size at diagnosis is a strong, independent predictor of survival in patients with totally anomalous pulmonary venous connection. In patients with this anomaly a nd small individual pulmonary veins, the anomaly may not be correctabl e by surgical creation of an anastomosis between the pulmonary venous confluence and the left atrium.