Embolization of major aortopulmonary collateral arteries by coils introduced via catheterization is a well-established procedure for the control of excessively high pulmonary blood flow in patients with tetralogy of Fallot and pulmonary stenosis or atresia. Under special circumstances, this procedure can be life-saving. Between October 1987 and February 1992, four patients with tetralogy of Fallot and pulmonary atresia and one patient with pulmonary stenosis rather than atresia underwent transcatheter embolization of major aortopulmonary collateral arteries as an emergency. Their ages ranged from 41 days to 13 years, with a mean of 45 months. Two patients had recently undergone surgical repair of tetralogy of Fallot while one had been palliated by enlarging the right ventricular outflow tract without closing the ventricular septal defect. All three had been dependent on mechanical ventilation in the postoperative period with signs of pulmonary congestion or intraparenchymal hemorrhage. Eight embolizations were attempted with 0.025 or 0.038 inch coils; seven collateral arteries were completely occluded and one was partially occluded. Thirteen coils were used in total. The systemic saturation rose in one postsurgical patient, with radiological evidence of decreased pulmonary congestion and regression of hemorrhage. In the other cases, the systemic saturation was unchanged. Peak pulmonary arterial pressures dropped in two patients but remained unchanged in three. Our experience suggests that embolization of major aortopulmonary collateral arteries can be beneficial in critical patients, thus avoiding the risks of surgical reexploration.

Over a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.

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.

Percutaneous transcatheter embolization of the arterial duct, using new detachable coils, was performed in 19 patients aged from two to 16 years. The minimal ductal diameter ranged from 0.5 to 2.7 mm. We used a 5 mm diameter coil, 8 cm in length and/or an 8 mm diameter coil, which was 10 cm in length. The coil was connected to a delivery wire with a screw system, and could be detached by rotation of the delivery wire. The coil was straightened by inserting a mandril, with loops then being formed by withdrawal of the mandril. In all patients, the coil was advanced retrogradely and either one or two coils were successfully implanted. Implantation was performed in four patients without the catheter entering the pulmonary trunk across the arterial duct. The ductal ampulla was either absent or very small in five patients, so we implanted a coil which had been cut short. Angiography revealed no residual shunting in 14 patients (74%) and trace residual shunting in five patients. Migration of the coils to the pulmonary arteries, or to a systemic artery, did not occur in any patient. Follow-up using Doppler color flow imaging showed complete closure of the arterial duct in all patients (100%), and no turbulence either in the left pulmonary artery or in the descending aorta in any patient. The use of the new detachable coil may improve the accuracy of placement of the device and reduce the incidence of its migration.

Objectives: To determine the anatomical characteristics of systemic venous collaterals formed after the Fontan operation, and the efficacy of a transcatheter strategy for management. Methods: We reviewed retrospectively the data from cardiac catherization of 50 persistently cyanotic patients after the Fontan operation. Results: A total of 54 transcatheter interventions were performed, at a mean age of 6.3 ± 3.5 years, a mean interval of 2.7 ± 2.9 years from completion of the Fontan circulation. Of 38 patients who had fenestration of the baffle at the time of surgery, 25 had patency of the fenestration, and 24 had the fenestration occluded with a device at the time of interventional treatment for associated venous collaterals. We identified a total of 68 systemic venous collateral channels, of which 36 (53%) were supracardiac, 12 (18%) cardiac, and 20 (29%) infracardiac in origin. The most common site of origin was the brachiocephalic vein (44%), followed by the left phrenic vein (25%). A longer time from surgery, at 3.3 ± 3.4 years, was associated with the identification of collaterals having a diameter larger than 4 mm (p < 0.01). The mean pulmonary arterial pressure was higher in those with larger compared to those with smaller collaterals (13.3 ± 2.8 versus 11.1 ± 2.0 mmHg, p < 0.01). Coils were used for occlusion of 61 vessels, and a Rashkind™ occluder for the remaining 7. After exclusion of the patients undergoing simultaneous closure of their fenestration, systemic saturation of oxygen increased from 89 ± 6% to 95 ± 3% (p < 0.01). Conclusion: Venous collateral channels are common in patients suffering progressive cyanosis in the setting of the Fontan circulation. The collaterals increase in size with time, and are associated with higher pulmonary arterial pressures. Transcatheter treatment is feasible, and results in resolution of cyanosis. Only continuing follow-up will show whether further collateralization occurs in time.