Deletion of chromosome 22 and DiGeorge syndrome are associated with inappropriate development of the neural crest and abnormalities of the ventricular outflow tracts. Bis-diamine induces comparable cardiac anomalies by inhibiting the normal development of the neural crest. In order to clarify the effect of damage on the development of the neural crest and its relation to the cardiac anomalies, the effects of giving a small dose of bis-diamine were compared with the results of a large dose of bis-diamine. A small dose of bis-diamine (20 mg), therefore, was administered to 40 pregnant rats on the 10th day of pregnancy, and 330 fetal hearts were studied on the 21st day with the rapid whole-body freezing method. Intracardiac anomalies were rare, being found in only 55 fetuses. The anomalies discovered were: aberrant origin of the subclavian artery in 88 fetuses, right aortic arch in 26 fetuses, interruption of the aortic arch in 23 fetuses, and high location of the aortic arch in 8 fetuses. Anomalies of the arterial duct were also common; with a vascular ring formed by the aortic arch and contra-lateral duct being seen in 21 fetuses, isolation of the right subclavian artery associated with either bilateral ducts (9 fetuses) or a right duct and absent left duct (9 fetuses), absent duct with tetralogy of Fallot in 7 fetuses, and with common arterial trunk in one fetus. In conclusion, a small dose of bis-diamine induced extra cardiac anomalies of the aortic arch and arterial duct, indicating a greater susceptibility of the aortic arch and the duct to inappropriate formation of the neural crest cells in the early developing embryo. Clinically, these anomalies of the aortic arch and the duct should be searched for in all patients suspected of having deletion of their 22nd chromosome.

We evaluated the physical characteristics of the pulmonary trunk in 32 patients with complete transposition after the arterial switch operation, calculating the pulmonary input impedance. In 6 of the patients, the characteristic impedance was equal to, or less than, the value in patients with ventricular septal defect. In those patients with an intact ventricular septum, or those with a ventricular septal defect who had undergone the arterial switch operation or banding of the pulmonary trunk before 3 months age, the frequency of the first modulus minimum also agreed with the values obtained in patients with ventricular septal defect. The value was high, however, in patients with complete transposition with ventricular septal defect who had undergone an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Our data suggest decreased distensibility of the proximal pulmonary trunk after the arterial switch operation, and mild changes in the pulmonary vasculature in patients with complete transposition with ventricular septal defect who had undergone either an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Long-term follow-up is needed clarify whether or not these features affect right ventricular function by means of an increase in afterload.

Balloon dilation was performed in four patients with postoperative pulmonary stenosis who had undergone surgical creation of a coronary arterial tunnel in the pulmonary trunk. Two patients had complete transposition in whom the arterial switch operation had been performed using the modified Aubert method. The other two patients had anomalous origin of the left coronary artery from the pulmonary trunk treated with the Takeuchi procedure. Balloon dilation was performed at 11 locations. The pressure gradient decreased from 48 ± 22 to 24 ± 14mmHg (p<0.01), and the diameter of the narrowest segment increased from 5.3 ± 2.5 to 7.5 ± 2.8mm (p<0.01), respectively. Of the 11 procedures, 8 (73%) were judged successful with use of the criterion of success as a greater than 50% decrease in pressure gradient, and/or a greater than 50% increase in diameter. The inflated balloon must have compressed the coronary arterial tunnel in the pulmonary trunk, but there was no apparent myocardial damage in any patient, although transient and mild ST-T changes appeared on electrocardiographic monitoring during the procedure in 2 patients. Rupture of the wall of the pulmonary trunk occurred in two patients, one of whom required elective surgical intervention. These data suggest that balloon dilation should be performed with caution for management of postoperative pulmonary arterial stenosis in patients with a surgically created intrapulmonary coronary arterial tunnel, since tearing the wall of the pulmonary trunk may occur.

We studied factors that predict in children catecholamine induced vasoconstriction of the persistent arterial duct. Epinephrine at 2.0–10.0 (4.9 ± 1.3, mean ± standard deviation) μg/kg was injected intravenously into 30 patients with persistent arterial duct whose age ranged from 1 to 91 (27 ± 26) months. In 11 patients aged 10–66 (34 ± 23) months (responders), a continuous murmur had almost completely disappeared on auscultation, and both the diameter and the shunt flow area of the arterial duct had become smaller as shown by Doppler echocardiography. On the other hand, there was no such change in 19 patients aged 1–91 (23 ± 27) months (non-responders). Although there was no significant difference in the age, the body weight, the minimal diameter of the arterial duct, or the doses of epinephrine between responders and non-responders, the gestational age at birth was slightly less (p=0.09) and the birth weight was significantly smaller in responders than in non-responders (p<0.05). The persistent arterial duct of those who had a history of low birth weight always responded to epinephrine. In infants younger than 6 months, the persistent arterial duct was never constricted by epinephrine regardless of the birth weight and gestational age. In low birth weight infants, the vascular smooth muscle of the persistent arterial duct can usually constrict beyond infancy. There may be some age dependent difference in responsiveness to epinephrine until 6 months of age.

We reviewed retrospectively the clinical and surgical data from 9 children with postoperative chylothorax secondary to cardiac surgery for congenital heart disease seen over a 3 year period. Mean age was 26 months and 6 patients were female. The procedures performed were 2 Blalock-Taussig shunts, 6 Bidirectional Glenn anastomoses and 1 modified Fontan procedure. Diagnosis of chylothorax was made by classical means. The overall incidence of chylothorax was 0.8%, with the relative incidences being 2.5% for Blalock-Taussig, 2.7% for Fontan, and 11.7% for the Glenn procedures. All patients were initially treated conservatively with chest tube drainage and hypercaloric diet suplemented with medium chain triglycerides. Parenteral nutrition was needed in 2 patients, one because of sepsis and the other because of progressive malnourishment. High output fistulas and long periods of drainage were noted after cavo-pulmonary procedures. Up to 12% weight loss was observed in 6 patients, one patient was successfully treated of pneumonia and another died of Pseudomonas aeruginosa sepsis. Two patients required chemical pleurodesis because of unabated drainage, with the others being successfully treated conservatively. Chylothorax is a common complication after bidirectional Glenn procedures, and is associated with considerable morbidity and mortality. Earlier operative intervention may be required in these patients. Pleurodesis is a simple, safe, and effective procedure to stop chylous flow.

To determine whether a newly synthesized oral analog of prostacyclin, called beraprost sodium, could cause pulmonary vasodilation, we studied its hemodynamic effect on pulmonary hypertension of children, comparing it to other vasodilatory agents, such as nitric oxide and tolazoline. We studied 20 children (mean age 24 months) having pulmonary hypertension secondary to congenital heart disease. A single oral dose of beraprost sodium resulted in an appreciable reduction of pulmonary vascular resistance (mean 34%), which was comparable to that induced by inhalation of nitric oxide and intravenous delivery of tolazoline (mean 41% and 31%, respectively). The results suggest that beraprost sodium may serve as a novel and safe vasodilator for the screening of pulmonary vasoreactivity, as well as the treatment of pulmonary hypertension in children.

Although patients undergoing surgery with the Fontan procedure or its modifications are increasingly recognised to be at risk for thromboembolism, further knowledge is needed to minimise this complication and its sequels. To address this issue, we reviewed 100 patients operated with the Fontan procedure, the bidirectional Glenn anastomosis and/or the total cavopulmonary connection to describe our incidence and clinical characteristics of postoperative formation of thrombus. Symptomatic thrombosis or cerebrovascular accidents were found in 5 patients. Asymptomatic thrombus were found in another 5 patients. Three patients had venous thrombi. In 6 patients the thrombosis was found on the arterial side. In all these cases, the thrombus was located in the stump of the divided pulmonary trunk. In 3 of these patients, sudden onset of hemiparesis preceded the diagnosis of the thrombus while the remaining 3 patients were asymptomatic. In 3 cases, the formation of thrombus in the stump of the divided pulmonary trunk occurred after a bidirectional Glenn anastomosis. One patient developed severe neurological symptoms 2 months after a total cavopulmonary connection. No thrombus was found, but the patient had a small right-to-left shunt and embolization could not be excluded. The incidence of thrombosis after Fontan-type surgery in this study is 10%. Although not all episodes of thrombosis are symptomatic, there is a significant risk of severe sequels due to embolization to the pulmonary or cerebral circulations. The stump of the divided pulmonary trunk is one of the main sites for intracardiac formation of thrombus, even after a bidirectional Glenn anastomosis.

Severe protein losing enteropathy occurred in 3 of our 128 patients undergoing the Fontan procedure in the period of 1982 to 1994. The complication occurred 4, 4.5 and 5 years after the procedure, respectively. One patient had double inlet left ventricle while the other two had tricuspid atresia. All had initially undergone an atrio-pulmonary connection, and the duration of the enteropathy was 12, 13,5 and 15 months. All patients were in functional class IV of the New York Heart Association. The presence of protein losing enteropathy was confirmed by determining levels of fecal alfa-1 antitrypsin, enteric clearance of 51 Cromo labeled albumin, and endoscopy with histopathological study of the small bowel.

They underwent further surgery to convert the atrio-pulmonary to a cavo-pulmonary anastomosis which excluded all the hepatic veins. A bovine pericardial baffle was sutured in the inferior caval vein so as to leave the hepatic venous flow draining to the right atrium and thence through an atrial septal defect to the left atrium and systemic circulation. The inferior caval venous flow was directed through an intra-atrial tunnel to the right pulmonary artery, and the superior caval vein was anastomosed to the superior aspect of the right pulmonary artery, thus completing the total cavo-pulmonary anastomosis with exclusion of the portal venous flow.

Postoperatively, there was total regression of the clinical and laboratory findings. All patients are now in functional class I or II of the New York Heart Association. They have mild to moderate cyanosis up to three and a half years from the reoperation. We recommend the technique as an alternative for patients with severe protein losing enteropathy occurring after the Fontan procedure.

To demonstrate the use of transthoracic contrast echocardiography in the detection of pulmonary arteriovenous fistulas in patients with a previously constructed anastomosis between the superior caval vein and the right pulmonary artery (Glenn shunt), and to examine their prevalence in this special population, we evaluated prospectively all patients followed up in the Grown-Up Congenital Heart Unit subsequent to construction of a classical or bi-directional Glenn shunt. We studied 12 patients, aged from 21 to 38 (mean 28 ± 4.8) years who had had a previous cavopulmonary shunt in place for a period of 4 to 33 years (mean 24±9 years). All were examined with cross-sectional contrast echocardiography, 11 patients had cardiac catheterisation and angiography, and 6 patients had magnetic resonance imaging. Systemic arterial oxygen saturations at rest, and during exercise using the modified Bruce protocol, were measured in all patients. Contrast echocardiography showed evidence of pulmonary arteriovenous fistulas in 7 of the 12 patients, with appearance of echo contrast in the left atrium 1–8 seconds after peripheral venous injection in the arm. Simultaneous appearance of microbubbles in the right atrium revealed a residual communication between the superior caval vein and the right atrium in 2 patients, and presence of collaterals between the superior and inferior caval veins in one. Cardiac catheterisation and angiography showed obvious fistulas in 4 patients, and revealed suggestive findings in 2. In patients deemed to have pulmonary arteriovenous fistulas on contrast echocardiography, arterial oxygen saturations at rest (51–94%, mean 75±15.3%) and on exercise (23–91%, mean 53±24.2%) were significantly lower compared to patients judged to be without fistulas (p<0.005). Pulmonary hypertension in the contralateral lung was more common in patients with fistulas (mean left pulmonary arterial pressure 22–110 mm Hg, p=0.014). In patients with cavopulmonary anastomoses, pulmonary arteriovenous fistulas occur frequently in the long term (10–33, mean 25.7±8 years), and are associated with worsening systemic arterial desaturation. Contrast echocardiography should be included in the regular evaluation of these patients as a simple and sensitive technique for the detection of pulmonary arteriovenous fistulas, particularly with the devel opment of increasing cyanosis.

We present an unusual case of criss-cross heart with supero-inferior ventricles, double outlet of the right ventricle, restrictive ventricular septal defect, severe infundibular stenosis and “absent” pulmonary valve who underwent successful surgical repair. Complete preoperative diagnosis was established with cross-sectional echocardiography combined with colour Doppler and angiocardiography, which facilitated successful surgical correction.

We report three cases of pulmonary venous stenosis developing after repair of totally anomalous pulmonary venous connection. In each case, we used steroids as a therapeutic measure following re-operation. In two cases, we were successful in preventing significant re-stenosis. The third patient rapidly developed stenosis (mean pulmonary wedge pressure approximately 20 mmHg at cardiac catheterisation) after stopping steroids. This patient subsequently died after a third operation. The remaining patients continue to do well.

We report two cases with persistence of the hepatic venous complex, underdevelopment of the inferior caval vein and azygos continuation to the superior caval vein. We suggest that the hepatic venous complex is a true congenital malformation rather than the result of passive enlargement of venous channels subsequent to high venous pressures. Preoperative diagnosis is important in patients with complex congenital heart disease undergoing palliative venous redirection procedures if a postoperative right-to-left shunt and cyanosis are to be avoided.

Either arrangement of the abdominal vessels or the morphology of the bronchial tree are usually used to predict atrial arrangement. We report a patient in whom, in the setting of normal physiology as seen with mirror-imaged venous return, and due to disparity between the arrangement of the abdominal vessels (suggestive of right isomerism) and thoracic arrangement (indicative for left isomerism), atrial arrangement could not be inferred. Direct echocardiographic imaging of both appendages revealed left isomerism.

In three children with coronary arterial fistulas, two extending from the right coronary artery to the right atrium and one from the left circumflex coronary artery to the right ventricle, multiplanar cine magnetic resonance imaging was able accurately to demonstrate dilation of the involved coronary artery, the tortuous nature of the dilated fistulas, blood flow within the fistulas, and luminal narrowing. The technique provides a new adjunct for management of patients, especially young children, with coronary arterial fistulas.