Резултати хируршког лечења тоталног аномалног споја плућних вена

Abstract

Introduction Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease and in some variants represents the only true surgical emergency in congenital heart surgery. Basic anatomical characteristic of this anomaly is an abnormal connection of pulmonary veins with systemic venous circulation. Although the results of TAPVC repair in infancy have been markedly improved in recent years, the recurrent pulmonary venous obstruction (RPVO) remains relatively frequent complication of surgical treatment.

Objective The aim of this study was a retrospective evaluation of TAPVC repair at a single institution, identifying the risk factors associated with the increased mortality and morbidity.

Methods Between January 2001 and January 2010, 43 consecutive patients underwent repair of TAPVC at the University Children’s Hospital, with median weight of 3.8 kg (1.8–13 kg). Median age at surgery varied from 5 days to 5 years. Distribution of TAPVC types was as follows: supracardiac 19 (44%), cardiac 12 (28%), infracardiac 9 (21%), and mixed 3 (7%). Eleven patients (26%) were emergencies due to obstructed drainage.

Results Early mortality was 9.30% (4/43). An average time of followup/survival for 95% interval of confidence was 101.6±6.7 months. Kaplan–Meier cumulative survival was 83.7±5.7%. Freedom from reintervention after 10 years was 87.2±0.5%. The principal reason for reintervention was RPVO.

Conclusion Preoperative obstruction is not a risk factor of early mortality and RPVO. Low body mass (below 2.5 kg) is the only identified risk factor of early mortality. Complex morphology of the confluens, particularly in a mixed type of TAPVR, is the main risk factor of RPVO development.

Keywords: total anomalous pulmonary venous connection; congenital heart disease; results of surgical repair