PEDIARTRIC MID-TERM COMPLICATIONS OF TOTAL CAVOPULMONARY CONNECTION WITH EMPHASIS UPON THROMBOEMBOLISM

Abstract

Background :Children with congenital heart disease constitute a major proportion of children
seen in tertiary hospitals with thromboembolism (TE) . Total Cavo-pulmonary Connection
(TCPC) surgery can result in TE and other complications as protein-losing enteropathy
(PLE), arrhythmias, ventricular dysfunction & neurologic sequelae . There are few well
designed studies in the literature determining the epidemiology of thrombosis after TCPC
operarion, however, TE has been diagnosed in children, especially following the BT shunt
and the Fontan surgery (1).Aim of work. To revise King Faisal Specialist Hospital &
Research Center (KFSH&RC)-Jeddah in TCPC in an attempt to provide evidence-based
recommendations for postoperative management of theses patients. Methods. Sixty-five
pediatric patients who underwent TCPC were retrospectively reviewed. The mean age at
operation was 4.9 ± 1.9 years. The following items were considered as the potential risk
factors according to previous reports: (1)aged more than 4 years (7 cases); (2)heterotaxy (8
cases); (3) systemic ventricular ejection fraction less than 60% (6cases); (4) atrioventricular
valve regurgitation moderate or greater (4 cases); (5) mean pulmonary arterial pressure 15
mmHg or greater (3 cases); (6) pulmonary arterial resistance 4.0 wood units or greater (9
cases); (7) arrhythmias (8 cases); (8) protein-losing enteropathy (3 cases); (9) previous TCPC
procedure (2 cases); (10) systemic ventricular outflow obstruction (1 case); and (11) enddiastolic
pressure of the systemic ventricle 11 mm Hg or higher (5 cases). Results. The
median follow-up period was 43 ( 3-96 ) months . Twenty-one patients had at least 1 risk
factor (range, 1 to 4). Early postoperative complications comprised ascites 2 ( 3% ) cases;
prolonged pleural effusion 12 ( 18.4 % )cases; low cardiac output syndrome 4(6.1%) cases;
significant postoperative bleeding 3 (4.6% ) cases; acute renal failure 6 (9.2%) cases; and
oxygen desaturation 4 (6.1%) cases; late postoperative complications took the form of new
onset of PLE 6 (9.2% ) cases; hepatic failure 1 (1.5%) case; worsening heart failure 5 (7.6%)
cases; atrial tachyarrhythmias 5 (7.6%) cases; sick sinus syndrome 2 (3%) cases; and
thrombosis 4 (6.1%)cases. There was 2 early deaths and 5 late deaths. The overall mortality
was 10.8%. Comparing the late survivors and nonsurvivors, no statistical significance was
identified in the above risk factors. Conclusions. The majority of the pediatric TCPC
candidates tolerated the TCPC procedure in the early postoperative period. The threshold for
diagnostic and interventional cardiac catheterization should be lowered post-TCPC .It seems
reasonable to recommend chronic oral anticoagulation in those patients despite it did not
prevent thrombosis in our patients who developed TCPC circuit blockade