Use of extracorporeal life support in total anomalous pulmonary venous drainage

The objective of this study was to analyze the clinical course and neurodevelopmental outcome of infants with total anomalous pulmonary venous drainage (TAPVD) who were treated with venoarterial extracorporeal life support (ECLS).

The study was done by retrospective national survey of ECLS centers located in the United States and Australia. Sixty-six patients from 28 centers that reported cases from 1976 to October 1992 to the Extracorporeal Life Support Organization registry were included in the study. Data regarding type of TAPVD, whether the diagnosis was known or suspected before the initiation of ECLS, method of diagnosis, timing of repair if done, outcome, and follow-up were collected.

Fifty-six of the patients were placed on ECLS at ages < 14 days (neonatal) and 10 patients underwent ECLS at ages > or = 14 days (pediatric). TAPVD was known or suspected before the initiation of ECLS in 35 (53%) of 66 and was most commonly diagnosed by color-flow Doppler echocardiography if initially missed. Surgical repair was not attempted in four of the 66 patients, leaving a total of 62 patients for comparison. The overall operative survival for both neonatal and pediatric patients was 24 (39%) of 62. The survival rate for neonates who underwent repair before ECLS was seven (54%) of 13, for those who underwent repair after ECLS it was six (60%) of 10, and for those who underwent repair during ECLS survival was seven (24%) of 29. Neonatal survival (20/52, 38%) was statistically more likely (p = 0.05) if the repair was done before or after ECLS rather than during ECLS, with each group compared separately. Follow-up data were available on 13 of 20 neonates and three of four pediatric patients. Bayley Scales of Infant Development scores were normal in only six (54%) of 11 survivors who returned for testing.

The diagnosis of TAPVD was often known before the initiation of ECLS. Neonates were more likely to survive if the repair could be done before or after ECLS rather than during ECLS. The lower survival of infants who underwent repair during ECLS reflects the degree of illness in many of these infants who were placed on ECLS on an emergency basis because their condition was too unstable to permit detailed cardiac evaluation. The survival rate of infants with TAPVD requiring ECLS is poor, with approximately one half of the survivors having mental and motor deficiencies; however, these infants represent a subset of patients with TAPVD who probably would have died without ECLS. We recommend that infants who are not starting to wean from ECLS at 7 days undergo reevaluation with color-flow Doppler echocardiography with consideration for cardiac catheterization if the diagnosis is in doubt. We also recommend that before infants with known TAPVD are placed on ECLS parents should be informed that survival with the use of ECLS is no different from survival with operation alone and that many of the survivors are impaired. Each active ECLS center should periodically review its accuracy in making this definitive diagnosis.