Objectives: Tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCA) is a well-known but always severe congenital heart disease. This study was designed to explore proper management after radical correction of TOF with MAPCA based on a hierarchical approach.
Methods: The following data were collected from 39 patients planned to undergo radical correction of TOF: age, weight, number of aortopulmonary collaterals, total lumen diameter and collateral diameter-to-body weight ratio, transcatheter occlusion and cardiac catheterization findings, mechanical ventilation time, and ICU monitoring time. The patients were divided into 4 groups by collateral diameter-to-body weight ratio as follows: <0.200 mm/kg (group 1), 0.200-0.500 mm/kg (group 2), >0.500 mm/kg (group 3), and no MAPCA (group 4). Data analysis was performed using IBM SPSS Statistics software for Mac version 22.0 (SPSS Inc., Chicago, Ill., USA) with logistic regression and Fisher's exact test.
Results: Most of the patients recovered well after radical correction; postoperative complications occurred in 12 patients and included bloody sputum, low cardiac output syndrome, and severe pulmonary infection that led to tracheotomy. By prolonging the mechanical ventilation time of the patients with postoperative complications, the conditions in 3 patients were improved. However, in the remaining patients, the condition worsened until transcatheter occlusions were performed. Transcatheter occlusion was performed in all 7 patients in group 3 (100%). Only 2 of the 8 patients in group 2 required transcatheter occlusion (25%), and none of the 9 patients in group 1 required transcatheter occlusion (0%). Only 1 patient (group 3) died after radical correction. The transcatheter occlusion results showed a strong association with the total lumen diameter and the collateral diameter-to-body weight ratio (p < 0.05) but no obvious association with age, weight, or the number of aortopulmonary collaterals (p > 0.05).
Conclusions: Postoperative management of patients with TOF and MAPCA has great significance. To reduce the morbidity and mortality, transcatheter coil embolization or surgical ligation should be performed in patients with a collateral diameter-to-body weight ratio of at least 0.500 mm/kg. In patients with values between approximately 0.200 and 0.500 mm/kg, prolongation of mechanical ventilation should have priority over transcatheter occlusion, and for patients with values below 0.200 mm/kg no additional treatment is needed.
© 2016 S. Karger AG, Basel.