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For more than 30 years Fontan-type procedures have been performed for surgical treatment in patients with functionally univentricular hearts. Advances in proper patient selection, staging of the Fontan palliation, and modifications of the surgical technique resulted in, continuous improvement of the surgical results during the past two decades. However, increasing experience with medium term and long term follow-up of the Fontan circulation has drawn attention to a number of sequelae and chronic complications affecting a significant number of patients. [ABSTRACT FROM AUTHOR]

Objective: To assess the pressure and flow velocity relations and respiratory variability of the systemic venous and hepatic venous return in patients with univentricular circulation.Patients: 15 selected patients who had undergone cavopulmonary anastomosis (10) or atriopulmonary anastomosis (5). Mean age at operation was 55.1 months (range 9 to 145). Studies were done at 75.5 (32.6) months (mean (SD)) after the operation.Setting: Tertiary referral centre.Methods: Patients were studied using simultaneous recordings of ECG, pressure trace, respirometer trace, and pulsed Doppler echocardiography. Mean systemic venous pressure and pulmonary vascular resistance did not differ significantly between the two patient groups.Results: After total cavopulmonary anastomosis, systemic venous pressure tracings showed a flattened pressure curve without any dependence on cardiac or respiratory cycle. After atriopulmonary anastomosis, right atrial pressure tracings showed a significantly higher "a" wave corresponding to atrial contraction, without any respiratory variability. Pulsed Doppler examination of the superior and inferior caval vein and hepatic vein after total cavopulmonary anastomosis did not show a reverse flow after atrial contraction. The inspiratory to expiratory velocity ratio of antegrade flow revealed a significant dependence of flow on changes in intrathoracic pressure in the intra-atrial tunnel, caval veins, and hepatic vein. During expiration, decrease or cessation of antegrade hepatic venous flow was documented. After an atriopulmonary anastomosis, there was a biphasic antegrade venous flow pattern without significant respiratory variation.Conclusions: After total cavopulmonary anastomosis, there was marked respiratory dependence of systemic and hepatic venous return, whereas after an atriopulmonary anastomosis venous flow pattern varied according to cardiac cycle and pressure trace. The effects of total cavopulmonary anastomosis on venous return might counteract its other haemodynamic advantages. [ABSTRACT FROM AUTHOR]

Objective: To report initial findings from a selected group of patients with morphological variations of the atrial septal defect who underwent transcatheter closure with a second generation redesigned double umbrella device. Patients: Two patients with abnormal location of the oval fossa and partial deficiency of the septal rim, three patients with multiple defects, and two patients with a multiperforated aneurysm of the interatrial septum (age range, 3.6-25.5 years). Methods: Defects were closed with the double umbrella device (CardioSEAL) consisting of two sets of flexible arms (with central and two mid-arm hinges) covered with sewn Dacron patches. The implantation procedure was monitored by transoesophageal echocardiography. Results: The diameter of the defect measured during transoesophageal echocardiography ranged from 7-18 mm and the balloon stretched diameter ranged from 13-21 mm. The size of the devices varied from 28-33 mm and the ratio of device size to defect size varied from 1.6-2.1. Two devices (23 and 28 mm) were chosen in a patient with two separated defects. No complications or serious arrhythmias were observed during implantation or follow up (median, 1.8 months). Residual shunting was trivial in three patients and mild in one patient (inferiorly located additional defect). Conclusions: To extend the selection critera of an isolated central interatrial defect for transcatheter closure, some modifications of the implantation technique are needed. Using the redesigned double umbrella device, effective closure in patients with multiple or irregularly shaped atrial septal defects was achieved, indicating a broadening of the spectrum of transcatheter closure. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To investigate the specific sequelae of the Fontan operation, and particularly the potential sequelae of chronically elevated systemic venous pressure. DESIGN: A retrospective analysis of clinical and haemodynamic data and evaluation of organ function in 80 surviving patients undergoing modified Fontan operation for various forms of underlying functionally univentricular hearts. PATIENTS: 65 patients (81%) who had undergone a total cavopulmonary anastomosis and 15 an atriopulmonary anastomosis. Follow up ranged from 12 to 106 months (mean 54 (SD 23) months). RESULTS: 62 patients underwent postoperative cardiac catheterisation (mean systemic venous pressure 10.5 (2.5) mm Hg and cardiac index 3.1 (0.7) l/min/m2). Older age at operation was significantly correlated with both higher systemic venous pressure and lower cardiac 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 hypoproteinaemia, with a significantly higher incidence in patients after total cavopulmonary anastomosis and young age at operation. Liver function tests reflecting liver synthesis and metabolism were normal in all, whereas mild cholestasis was found in nearly 30%-predominantly in patients with a cardiac index of < 3 l/min/m2 (P = 0.045). Five patients (6.2%) developed atrial thrombosis. Coagulation factor analysis in 44 patients showed protein C deficiency in 11 (25%); laboratory signs of activation of the coagulation system were found in four of these (9%). None of the abnormal laboratory indices was significantly related to underlying cardiac malformation, postoperative systemic venous pressure, or follow up interval. CONCLUSIONS: A high proportion of clinically asymptomatic patients had abnormal laboratory findings on mid-term follow up. Detailed evaluation of organ function is necessary to detect the need for further diagnostic procedures before clinical symptoms develop. [ABSTRACT FROM PUBLISHER]