The influence of cardiovascular malformations on the prognosis of esophageal atresia and distal tracheoesophageal fistula

Two hundred and seventy-six cases of esophageal atresia (EA) and tracheoesophageal fistula (TEF) (173 males and 103 females) were studied. A cardiovascular malformation (CVM) was the most common associated anomaly (79 cases, 32%). The most common extracardiac malformations (ECM) included skeletal, gastrointestinal, and urinary tract anomalies. There was a significantly lower mortality in patients without associated anomalies (5%) compared to those with a major CVM (41%) (P<.01) but not compared to those with a minor CVM (10%). No significant difference in survival could be attributed to the specific types of CVM, although the overall survival in those with a non-complex CVM (64%) was higher than in those with a complex CVM (12%). It was not possible to associate a particular ECM with a specific form of CVM, but the greater the number of ECMs present, the greater the likelihood of associated CVM.