OBJECTIVES: We investigated whether mortality in totally anomalous pulmonary venous connection could be predicted from preoperative individual pulmonary vein size. BACKGROUND: Some infants with this anomaly die with or without surgical repair because of stenosis of individual pulmonary veins. METHODS: Individual pulmonary vein, vertical vein and pulmonary venous confluence diameters were retrospectively measured from preoperative echocardiograms in 32 infants with totally anomalous pulmonary venous connection presenting to Children's Hospital, Boston over a 4 1/2-year period. Data on body surface area, other cardiac anomalies, presence of initial pulmonary venous obstruction and early surgery and outcome were also recorded. RESULTS: Of 32 patients, 6 (18.8%) died before hospital discharge, and 8 (25.0%) died subsequently. Six (75.0%) of the eight patients who died late had individual pulmonary vein stenosis at sites remote from the surgical anastomosis to the left atrium. 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 confluence size (p = 0.02) and presence of heterotaxy syndrome (p = 0.008) were each significant univariate predictors of survival. Multivariate analysis showed that small pulmonary vein sum was a strong predictor of survival (p = 0.008), independent of the presence of heterotaxy syndrome. An analysis stratified by the presence of heterotaxy syndrome showed that the predictive effect of small pulmonary vein sum on survival was strongest in patients without heterotaxy syndrome. CONCLUSIONS: 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 and small individual pulmonary veins, the anomaly may not be correctable by surgical creation of an anastomosis between the pulmonary venous confluence and the left atrium.
OBJECTIVES: We investigated whether mortality in totally anomalous pulmonary venous connection could be predicted from preoperative individual pulmonary vein size. BACKGROUND: Some infants with this anomaly die with or without surgical repair because of stenosis of individual pulmonary veins. METHODS: Individual pulmonary vein, vertical vein and pulmonary venous confluence diameters were retrospectively measured from preoperative echocardiograms in 32 infants with totally anomalous pulmonary venous connection presenting to Children's Hospital, Boston over a 4 1/2-year period. Data on body surface area, other cardiac anomalies, presence of initial pulmonary venous obstruction and early surgery and outcome were also recorded. RESULTS: Of 32 patients, 6 (18.8%) died before hospital discharge, and 8 (25.0%) died subsequently. Six (75.0%) of the eight patients who died late had individual pulmonary vein stenosis at sites remote from the surgical anastomosis to the left atrium. 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 confluence size (p = 0.02) and presence of heterotaxy syndrome (p = 0.008) were each significant univariate predictors of survival. Multivariate analysis showed that small pulmonary vein sum was a strong predictor of survival (p = 0.008), independent of the presence of heterotaxy syndrome. An analysis stratified by the presence of heterotaxy syndrome showed that the predictive effect of small pulmonary vein sum on survival was strongest in patients without heterotaxy syndrome. CONCLUSIONS: 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 and small individual pulmonary veins, the anomaly may not be correctable by surgical creation of an anastomosis between the pulmonary venous confluence and the left atrium.