Specific sequelae after Fontan operation at mid- and long-term follow-up. Arrhythmia, liver dysfunction, and coagulation disorders.

Patients who have undergone a Fontan-type operation usually have an elevated systemic venous pressure. To determine the sequelae of this nonphysiologic condition, we evaluated 66 patients 1 to 14 years after a Fontan-type operation. Fifty-one patients were apparently in good clinical condition, and 15 patients had symptoms and were restricted in their daily life. Bicycle exercise capacity, tested in 41 patients, ranged from 50% to 110% (mean 85%) of the predicted value for length. In 16 patients, a decreased capacity (< 85%) was, among others, related to arrhythmias and the presence of protein-losing enteropathy. A 24-hour ambulatory electrocardiogram was available in 56 patients and found to be normal in 32 (57%) patients. Arrhythmias were present in 21 patients, six of whom had symptoms. Three patients had previous pacemaker implantation. One or more abnormalities in liver enzyme and function tests were present in 40 patients (61%) and in coagulation factors in 46 patients (69%). The most pronounced was a protein C deficiency, a known thrombotic risk factor, present in 41 patients. The occurrence of arrhythmias increased with time of follow-up (p < 0.004), the occurrence of protein C deficiency decreased with time (p < 0.0001), and the occurrence of abnormal liver enzyme and function tests was not related to time of follow-up. With regard to age at operation, arrhythmias did not occur in patients who underwent operation at a mean age of 4 +/- 1.9 years (standard deviation), in contrast to patients who underwent operation at a mean age of 7.6 +/- 4 years (standard deviation) (p < 0.001). The occurrence of the two other types of sequelae was not related to the age at operation. With regard to the type of operation, only patients with a valved right atrium-to-pulmonary artery connection had a higher prevalence of arrhythmias than patients with a nonvalved or direct right atrium-to-pulmonary artery connection (p < or = 0.001). The latter patients also had a higher prevalence of protein C deficiency (p < or = 0.001). No relationship was found among the other types of operation, the underlying structure, or the hemodynamic condition measured at rest and the presence of arrhythmias, abnormal liver enzyme and function tests, or protein C deficiency. This point survey shows that even patients with an apparently good clinical condition are at risk for arrhythmias, abnormal liver enzyme and function tests, and coagulation factor abnormalities. Serial statement of affairs is recommended to ensure that adequate preventive measures can be taken.

The asymptomatic child a long time after the Mustard operation for transposition of the great arteries.

We studied 36 asymptomatic children 7.7 +/- 2.5 years after a Mustard operation. Fifteen children had sinus rhythm on all electrocardiograms made during follow-up. Only 2 had normal 24-hour Holter recordings throughout follow-up, 6 had periods of supraventricular tachycardia, and 3 had periods of atrial flutter. The electrophysiological evaluation of sinus node function was normal in 5 of the 31 children who were studied. The behavior of the atrial myocardium was electrophysiologically abnormal in most of the children. Atrioventricular node function, on the contrary, was normal in nearly all of the children. Eleven children had normal hemodynamics. Four had severe or complete obstruction of the superior vena cava, 1 had a severe pulmonary venous obstruction, 3 had a severe left ventricular outflow tract obstruction, and 2 had a large left-to-right shunt. Only 3 children had normal hemodynamic and electrophysiological studies. We conclude that the absence of symptoms and a normal routine examination of children a long time after a Mustard operation does not exclude hemodynamic and electrophysiological abnormalities, which can sometimes be severe. In view of these disappointing results, we decided to replace the Mustard operation with the arterial switch operation in children with transposition of the great arteries.

Arrhythmias after repair of secundum atrial septal defect: the influence of surgical modification.

A retrospective study performed in our institution showed a significant correlation between venous cannulation and the incidence of arrhythmias after atrial septal defect (ASD) repair. We now report the results of a prospective study in 50 children operated on for ASD with selective cannulation of the superior vena cava. ECGs and Holter recordings were made before and after surgery, with a mean follow-up of 2.6 years. We found a significant decrease in postoperative arrhythmias (p less than 0.05) after changing the cannulation technique. Severe arrhythmias, present in 10% of the children in the retrospective group, were not found in the prospective study. Six children had asymptomatic arrhythmias on the Holter recording 1 year after surgery. Three of them, however, already had arrhythmias before the operation. We conclude that the incidence and severity of arrhythmias after ASD repair can be reduced significantly by surgical modifications, at least for the follow-up period of this study. The long-term significance has to be awaited.

Protein-losing enteropathy after Fontan operation.

Generalized edema resulting from severe protein-losing enteropathy occurred in three patients 12, 15, and 17 months after the Fontan operation. One patient originally had tricuspid atresia and the other two, univentricular heart disease. At operation a conduit had been inserted between the right atrium and pulmonary artery. Apart from the protein loss, the patients were in good health. The cardiac catheterization data obtained 0.8 to 2.4 years (median 1.3 years) after operation in the three patients with protein-losing enteropathy were compared with those of 18 patients in whom Fontan's operation had been performed because of tricuspid atresia (eight patients) or univentricular heart disease (10 patients). All had atriopulmonary connections. The mean right and left atrial pressures and systemic blood flows measured by dye dilution in the patients with and without protein-losing enteropathy did not differ. However, the patients with protein-losing enteropathy had a higher diastolic right atrial pressure. Since maximal antegrade flow in the superior vena cava after Fontan's operation occurs during atrial diastole, these observations suggest that an increase in diastolic right atrial pressure may result in protein-losing enteropathy because of impairment of blood flow and therefore congestion in the superior vena cava, subclavian vein, and thoracic duct.

Massive mediastinal hemorrhage following treatment of hyperthyroidism with radioactive iodine.

A case of superior mediastinal hemorrhage is described which was caused by necrosis of retrosternal thyroid tissue, following treatment with radioactive iodine.

Dysrhythmias after atrial surgery in children.

A retrospective study was done in 50 patients after Mustard's operation (group A) and in 204 patients after closure of a secundum atrial septal defect (group B) to analyze the postoperative dysrhythmias and to relate them to surgical factors. Forty-two percent of the patients in group A had dysrhythmias at the end of the follow-up, compared to 23% of group B patients. There was a high late mortality in group A (16%) significantly related to AF and AVJ. In group A a significant correlation was found between dysrhythmias and age at operation, use of cardioplegia, perfusion time, and the type of cannulation. In group B there was a significant relation between the location of the defect and the presence of abnormal pulmonary venous drainage. After ASD closure using hypothermia instead of cardiopulmonary bypass, the incidence of dysrhythmias was significant lower. Damage to the sinus node by cannulation and by suturing in the sinus node area is the main cause of the high incidence of dysrhythmias after atrial surgery. This high incidence should be a factor in the consideration of new types of operations.

Successful management of cor triatriatum associated with anomalous pulmonary/systemic venous connection in an infant.

Diagnosis and successful surgical repair of cor triatriatum, associated with anomalous pulmonary/systemic venous connection in a 6-month-old infant, is described. Follow-up at 2 years revealed no abnormalities. The clinical findings, hemodynamics, and surgical treatment are discussed, together with a review of the literature. This is a rare, life-threatening situation presenting significant diagnostic difficulties in infancy. It is, however, amenable to surgery provided that an early diagnosis is achieved.

Een primaire vagina-steen / A primary vaginal concretion

A primary vaginal concretion was found and removed in a 2-year-old child with severe hydrocephalus. The causative factors were probably the child's neurogenic incontinence and paresis of the legs......(AU)