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Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot.

Although formation of an aortic root abscess is a frequent complication of aortic valve endocarditis in adults, this complication has been rarely observed in children. In the majority of cases it has been described in children without underlying congenital heart disease. Due to the rarity of this complication, diagnosis and treatment is frequently delayed in childhood. We report a 2 1/2 year old girl who developed pericardial effusion in the course of pneumonia. Echocardiographic examinations, which were performed because of the pericardial effusion, revealed after 6 days the development of a cystic structure posterior to the aortic root. There was a perforation of this aortic root abscess to the left ventricular outflow tract; the aortic and mitral valves however were normal without endocarditic vegetations. Surgery was performed on the 10th day following a rapid increase in the size of the abscess. During surgery the abscess was drained and the perforation to the left ventricle was closed with direct sutures. Intraoperative transesophageal echocardiography confirmed a good surgical result. Blood cultures remained negative; in the material from the abscess however we found staphylococcus aureus. The postoperative course was uneventful. Our case demonstrates the necessity of detailed and repeated echocardiographic examinations in children with possible symptoms of bacterial endocarditis (in our case pericardial effusion) as well as the requirement of cultures of the abscess for identification of the infective organism. Intraoperative transesophageal echocardiography allows exact description of an aortic root abscess, its relation to other cardiac structures and immediate evaluation of the surgical result.

This report describes our results with transcatheter closure of patent ductus arteriosus between March 1993 and May 2000 including our early experience with the Amplatzer duct occluder. One hundred and sixty-six consecutive procedures were performed in 160 patients. The mean age was 6.8 years (range 0.8 to 26.2), mean weight 24.3 kg (range 7.7 to 84.0). Single or multiple coils were implanted successfully in 114 cases, a Rashind double umbrella in 35 patients, and an Amplatzer duct occluder in 16 patients. After failure to implant coils in one patient, a second attempt with a Rashkind double umbrella was successful. Complete closure of the patent ductus arteriosus was achieved in a total of 148 patients (92.5%), mean fluoroscopy time was 13.7 min (range 3.1 to 126 min). In 144 patients (90.0%), occlusion without residual shunting was achieved by the first interventional approach. Angiography showed immediate closure in 44%, echocardiography within 48 h revealed complete closure in additional 28%. In a further 18%, a residual shunt disappeared spontaneously in the following months. In five patients a second procedure was performed to terminate residual shunting after placement of a Rashkind occluder system by coils. In three patients, the residual shunt resolved. In 12 patients (7.5%) echocardiography showed a residual shunt 2-1259 days after interventional approach. Occlusion rates of the different devices were 83% for the Rashkind occluder, 92% for detachable coils, and 100% for the Amplatzer duct occluder. Coil embolisation into the pulmonary artery occurred in two patients, interventional removal was successful in one of them. There were no further complications. According to our experience interventional occlusion of patent ductus arteriosus is highly effective and associated with a low complication rate. Transcatheter closure using the Amplatzer duct occluder seems to be safe and effective also in small children with a body weight of 8 kg and a large ductus diameter. Compared to the total group of patients, the occlusion rate of the Amplatzer duct occluder was significantly higher (p = 0.005), and of the Rashkind occluder system significantly lower (p = 0.026). Therefore, we recommend the use of detachable coils in patients with small ductus (diameter < or = 2 mm) and the Amplatzer duct occluder in those with a larger ductus. Transcatheter closure of the patent ductus arteriosus according to this regimen should achieve occlusion rates above 95%.

Late postoperative arrhythmias are a common problem associated with significant morbidity following Fontan operations. We analyzed in our patients whether age at the time of a modified Fontan procedure (total cavopulmonary connection) has an influence on the frequency of late postoperative arrhythmias. We examined all 66 long-term surviving patients who underwent a total cavopulmonary connection (TCPC) in our center during a nine year period. At the time of TCPC, 31 children were younger (group I) and 35 children were older than 4 years (group II). The follow-up period was almost identical in both groups (group I: 4.29 years, group II: 4.52 years). We evaluated all ecgs performed at maximum intervals of 6 months and all Holter ecgs performed at intervals of 12 months. Atrial tachyarrhythmias (supraventricular tachycardias, atrial flutter) were registered in six patients (9%). All of these patients belonged to group II (17%). Ventricular arrhythmias (couplets, ventricular tachycardia) were found in 3% of the younger and 17% of the older patients. Abnormalities of sinus node function were registered in equal frequency in younger and older children (group I: 35%, group II: 31%), while pauses with a duration of > 2 s where present only in older children (group II: 14%). Pacemaker implantation or antiarrhythmic therapy became necessary in 10/66 patients (15%) affecting none of the children who underwent the TCPC < 4 years (group I) but 10/35 children (group II) who underwent the TCPC at an older age (29%). According to our findings in the medium-term follow-up after TCPC, atrial tachyarrhythmias and complex ventricular arrhythmias are registered less frequently in children who underwent the modified Fontan procedure at an age of less than 4 years. Although further studies will have to show whether these differences persist in the long-term follow-up, these findings represent an additional argument for the elective performance of modified Fontan operations at an age of less than 4 years.