SEQUELAE AFTER MODIFIED FONTAN OPERATION - POSTOPERATIVE HEMODYNAMIC DATA AND ORGAN FUNCTION

Objective-To investigate the specific sequelae of the Fontan operation , and particularly the potential sequelae of chronically elevated syst emic venous pressure. Design-A retrospective analysis of clinical and haemodynamic data and evaluation of organ function in 80 surviving pat ients undergoing modified Fontan operation for various forms of underl ying functionally univentricular hearts. Patients-65 patients (81%) wh o had undergone a total cavopulmonary anastomosis and 15 an atriopulmo nary anastomosis. Follow up ranged from 12 to 106 months (mean 54 (SD 23) months). Results-62 patients underwent postoperative cardiac cathe terisation (mean systemic venous pressure 10.5 (2.5) mm Hg and cardiac index 3.1 (0.7) 1/min/m(2)). Older age at operation was significantly correlated with both higher systemic venous pressure and lower cardia c index. Atrial arrhythmia was documented on Holter electrocardiogram in 17%. Protein losing enteropathy (with abnormal alpha(1)-antitrypsin clearance) was found in 2/80 patients (2.5%). Ten patients had hypopr oteinaemia, with a significantly higher incidence in patients after to tal cavopulmonary anastomosis and young age at operation. Liver functi on tests reflecting liver synthesis and metabolism were normal in all, whereas mild cholestasis was found in nearly 30%-predominantly in pat ients with a cardiac index of < 3 1/min/m(2) (P = 0.045). Five patient s (6.2%) developed atrial thrombosis. Coagulation factor analysis in 4 4 patients showed protein C deficiency in 11 (25%); laboratory signs o f activation of the coagulation system were found in four of these (9% ). None of the abnormal laboratory indices was significantly related t o underlying cardiac malformation, postoperative systemic venous press ure, or follow up interval. Conclusions-A high proportion of clinicall y asymptomatic patients had abnormal laboratory findings on mid-term f ollow up. Detailed evaluation of organ function is necessary to detect the need for further diagnostic procedures before clinical symptoms d evelop.