Total anomalous pulmonary venous connection in Children.

ABSTRACT

A retrospective analysis of 36 children diagnosed as having total anomalous pulmonary venous connection (TAPVC), admitted to Siriraj Hospital from December 1979 to September 1991, was undertaken. The children, 17 males (47%) and 19 females (53%), ranged in from one day to 13 years. Most cases (63%) presented early within the first year of life. All were normal full-term infants with 86 percent having normal birth weight of large for gestational age (over 2,500 grams). The predominant clinical presentation was mild cyanosis and congestive heart failure with cardiomegaly and increased pulmonary blood flow. Echocardiography and cardiac catheterization were used as diagnostic tools to demonstrate pulmonary venous connection. Cardiac and supracardiac types were the most two common ones (47 and 41%, respectively). Surgical correction was the specific treatment in these patients and the results were good. The total mortality rate was 19 percent, the most common cause of death was complicated pulmonary infections. However, chronic heart failure due to surgical obstruction was encountered even through surgical intervention was performed.