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) 1lmin/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 a,-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 300/o-predominantly in patients with a cardiac index of < 3 I/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. (Heart 1997;78:154-159)

In a high proportion of hearts with complex univentricular anatomy, associated anomalies, and borderline hemodynamics, the Fontan operation can be performed as a total cavopulmonary anastomosis with acceptable intermediate postoperative morbidity and hemodynamic results.

Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3 +/- 1.3 vs 5.6 +/- 1.4 10(12)/l p less than 0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.

Microdeletions in chromosome 22q11.2 are associated with DiGeorge syndrome (DGS), velo-cardio-facial syndrome (VCFS), and several other syndromes, collectively referred to as DG/VCF. Non-dysmorphic patients with cardiac defects have also been attributed to deletions in this chromosomal region. In this study 157 consecutively catheterized patients with isolated, non-syndromic cardiac defects, and 25 patients with cardiac defects and additional stigmata (10 of whom were clinically diagnosed as DG/VCF cases prior to chromosome analysis) were analysed by fluorescence in situ hybridization with the DGS-specific probe D0832. Chromosome 22q11.2 deletions were observed only in the ten patients with the clinical diagnosis of DG/VCF. Conclusion In a large unselected cohort of patients with congenital heart disease no association between isolated or non-syndromic heart defects and the 22q11.2 microdeletion was observed. One can conclude that testing for the 22q11.2 microdeletion is clearly indicated in cases when even mild extracardiac abnormalities are present, particularly in very young infants.

Chest closure after cardiac surgery occasionally results in cardiac compression leading to circulatory failure. In shunt-dependent circulation, the arterial oxygen saturation may decrease significantly due to the increase in pulmonary vascular resistance caused by chest closure. Temporary patch implantation with delayed sternal closure facilitates circulatory and/or pulmonary stabilization (temporary chest wall patch plasty, TCWPP). Between July 1986 and June 1991, 42 patients underwent staged chest closure (TCWPP) after open heart surgery for congenital lesions (4.9% of 854 patients). TCWPP was performed when either primary hemodynamic deterioration or an increase in cyanosis (palliative procedures only) followed by hemodynamic deterioration occurred during attempted or shortly after sternal closure. Overall mortality was 40.4% (17/42). It was 32.3% (11/34) when the patch was inserted primarily at the end of the operation. If the patch was inserted emergently 4-24 h postoperatively, mortality was 75% (6/8). Definite chest closure was performed from 4 h to 6 days (mean 72 h) postoperatively. In 2 patients closure had to be performed emergently (single ventricles); 7 patients died before chest closure. One mediastinal microbiology examination was positive. Deep sternal infection necessitating operative revision occurred in one other patient. In conclusion, TCWPP may considerably lower mortality of the illest patients after surgery for complex congenital heart disease. A timely decision as to the performance of staged chest closure is mandatory. This procedure rarely causes infection. We now apply this technique liberally, by cardio-mediastinal size judgement in over 30% of our TCWPP candidates even without a prior trial of primary closure.

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 diVer 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 intraatrial 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 eVects of total cavopulmonary anastomosis on venous return might counteract its other haemodynamic advantages. (Heart 1999;82:294-299)

In order to assess the diagnostic possibilities of magnetic resonance imaging (MRI), 30 children (mean age 11.5 years) with d-transposition of the great arteries (TGA) corrected by the Mustard procedure were studied. Patient values were compared to those of 10 healthy volunteers. The most important postoperative abnormalities such as baffle leaks, systemic or pulmonary venous obstructions, left ventricular outflow tract (LVOT) stenosis and tricuspid regurgitation were assessed. Cardiac volumes and muscle mass were measured. Baffle leaks were found in 5 and obstruction of the baffle limbs in 6 patients. On gradient echo images 13 patients showed signs of LVOT stenosis and 12 tricuspid incompetence. Right ventricular end diastolic volumes in patients were significantly higher than the left ventricular volumes, but slightly lower than right ventricular volumes in the normals. In patients with TGA and ventricular septal defect (VSD) (n = 7) right ventricular (RV) volumes were found to be higher than in patients with intact ventricular septum (n = 23) and in normals. In contrast to the normal RV function of patients after Mustard surgery only patients with previous VSD showed a diminished RV ejection fraction. The muscular mass in TGA patients showed a ratio of 1.9:1 between right and left ventricles. In normals the ratio was 1:1.5. Magnetic resonance imaging allows a quantitative insight into the ventricular function and the morphology of the heart after inflow correction. Thus, it supplements the noninvasive evaluation of TGA patients.

Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptoms.