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Unlabelled: Formation of systemic to pulmonary venous or systemic venous left atrial collaterals frequently occurs in patients after Glenn or Fontan-type operations. Embolization with detachable metal coils is the therapy of choice for the closure of small vessels. These devices however are not appropriate for the occlusion of large collaterals, e. g. recanalized bilateral caval veins. We report two patients who presented late after Fontan-type operations with a gradual decrease in oxygen saturation due to recanalisation of bilateral caval veins. Interventional closure of these large veins was carried out successfully with the use of 8 mm Amplatzer muscular VSD Occluders, resulting in an increase of arterial oxygen saturations., Conclusion: The closure of recanalized bilateral superior caval veins after Fontan procedures is possible without technical problems by means of the Amplatzer muscular VSD Occluder. In order to avoid future formation of venous collaterals via the azygos or hemiazgos system, the occluder should be placed in the vena cava below the orifice of the azygos/ hemiazygos vein.

We report on an asymptomatic 2 1/4 year old girl. Echocardiography revealed cor triatriatum with a membrane separating the left atrium. Transesophageal echocardiography demonstrated 3 large perforations of the intraatrial membrane. Using Doppler sonography, no pressure gradients across these orifices were identified. Obstruction of the intraatrial membrane and pulmonary hypertension were excluded by cardiac catheterization. We therefore decided against surgical resection of the membrane and to follow the patient noninvasively by echocardiography. Follow-up over 18 months revealed no development of an obstruction across the intraatrial membrane. Our case shows that immediate surgical correction is not necessary in all patients with cor triatriatum. Conservative management of these patients requires full evaluation of the hemodynamics and careful follow-up examinations by echocardiography.

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.

The pulmonary sling or aberrant left pulmonary artery has an incidence of 3% to 6% of all anomalies of the aortic arch system. We report a boy with coexistence of a normal and an anomalous left pulmonary artery. Associated congenital anomalies were coarctation, mitral stenosis, and imperforate anus. Cardiac catheterization and angiocardiography at the age of 5 months demonstrated an inconspicuous pulmonary trunk dividing into left and right branches. There was a normal right pulmonary artery, and a main left pulmonary artery supplying the left upper lobe, lingula, and anterior segments of the left lower lobe. In addition, an anomalous left lower lobe artery originated from the proximal right pulmonary artery, passed inferior to the tracheal bifurcation to the left supplying posterior segments of the left lower lobe. Chest X-rays and bronchoscopy could not detect any malformation of the trachea and pulmonary lobulation. There are only three reports of partial anomalous origin of the left pulmonary artery, and only one had a similar course of the accessory artery. This report is the first to present selective angiography and echocardiographic findings of the partial anomalous left pulmonary artery. The development of the partial anomalous pulmonary artery can be explained by the plexiform nature of the primary pulmonary vascular bed and can help to understand the embryology of the pulmonary arteries.

Seventeen consecutive newborn and premature babies with critical pulmonary stenosis underwent a technically successful balloon valvuloplasty at our institution from March 1991 to February 1998. The only major complication was a thrombosis of the femoral vein in one patient, causing no clinical problems. Four patients (24%) showed a reactive infundibular obstruction after balloon valvuloplasty. The outflow tract obstruction became evident immediately after successful dilatation of the pulmonary valve with persistently high pressures in the right ventricle. Pathognomonic was a typical notch in the ascending part of the right ventricular pressure curve. We were unable to predict this reaction based on echocardiography or angiography. To relieve the muscular subvalvar obstruction, we treated the first two patients with i.v. Propranolol (0.05 mg/kg over 2 min). The last 2 patients received Esmolol (0.5 mg/kg over 2 min followed by a continuous infusion with 100 micrograms/kg/min), a very short acting beta-blocker. In the medium-term follow-up, all 17 patients had a very good result with only mild pulmonary valve regurgitation. All 4 patients with a reactive infundibular obstruction required no repeat intervention. In the medium-term follow-up there were no differences between these 4 patients and the whole group.

Hypocalcemia is a relatively uncommon reversible cause of congestive heart failure. There are a few reports of hypocalcemic children who developed congestive heart failure associated with hypoparathyroidism. In all these patients, however, cardiac failure did not occur before the age of nine years. In addition, other striking noncardial manifestations of hypoparathyroidism, e.g., convulsive seizures, had been present prior to cardiac symptoms. We report on a 3.7 year old girl with mitral insufficiency and severe cardiac failure due to hypocalcemia secondary to familial hypoparathyroidism. The infant's mother was suffering from idiopathic hypoparathyroidism, but her own history lacked any evidence for parathyroid hormone deficiency. On admission, she presented with fatigue, dyspnea, and pedal edema. Liver edge was palpable 4 cm below the right costal margin, and a 3/6 systolic murmur was heard. A chest x-ray showed cardiac enlargement; electrocardiogram demonstrated a prolonged QTc interval of 0.46 s. The echocardiography revealed a cleft in the mitral valve with mitral insufficiency and markedly reduced contractility of the left ventricle. Laboratory studies demonstrated a low total serum calcium level of 1.3 mmol/l; serum magnesium level was slightly decreased (0.5 mmol/l), and parathyroid hormone level was not detectable. Partial monosomy of chromosome 22 was excluded. Ophthalmological examination, audiometry, and renal ultrasonogram were normal. Oral calcium supplementation and anticongestive therapy with metildigoxin, furosemid, and captopril was initiated but no improvement of the heart failure occurred. However, normalization of serum calcium level by calcium infusions caused prompt clearing of the clinical symptoms, complete normalization of liver size, reduction of cardiac enlargement (thoracic ratio decreased from 0.68 to 0.57), and marked improvement in contractility (left ventricular shortening fraction increased from 21% to 34%). The QTc interval decreased to 0.39 s. The successful treatment following normalization of serum calcium level proved the superiority of hypocalcemia over mitral valve insufficiency in the etiology of the cardiac failure. To our knowledge, this is the first report of congestive heart failure due to hypocalcemia as the first manifestation of hypoparathyroidism in childhood. Hypocalcemia should be kept in mind in any congestive heart failure in children with or without underlying cardiac malformation.

We report on 2 infants with Ullrich-Turner syndrome in whom partial anomalous pulmonary venous drainage was diagnosed noninvasively by color-coded Doppler sonography. Several patients with the combination of anomalous drainage of one or more pulmonary veins and Ullrich-Turner syndrome have been described in the literature. However, in the majority of those previously reported cases the diagnosis of partial anomalous pulmonary venous drainage was established by angiography during cardiac catheterization performed for confirmation of other cardiovascular malformations. Our patients show that partial anomalous pulmonary venous drainage can be diagnosed easily in neonates and young infants, as long as this anomaly is taken into consideration.

Although carditis associated with Mycoplasma pneumoniae is infrequent it is an important cause of death in M. pneumoniae infections. We report on a 4-year-old boy with a M. pneumoniae infection, who developed a large hemorrhagic pericardial effusion and was successfully treated by percutaneous catheter drainage. Except in cancer patients, nontraumatic hemorrhagic pericardial effusions are rare. Our case strongly supports direct bacterial invasion into pericardial tissue as a cause of M. pneumoniae pericarditis rather than autoimmune phenomenon.

Major systemicopulmonary collateral arteries (spca) frequently contribute to collateral lung perfusion in patients with pulmonary atresia and ventricular septal defect or in children with tetralogy of Fallot. Since the surgical access to these vessels is difficult, corrective surgery may become impossible. We report our experience with interventional occlusion of spca in 10 patients. In these patients (age range 13 months-19.5 years) selective injections demonstrated a total of 27 spca. Sixteen of these were coil-occluded by interventional cardiac catheterization. Total occlusion was achieved in 15 cases, in one case we found a small residual shunt. Occlusion was performed using platinum coils (2 cases), Gianturco coils (11 cases) and detachable steel coils (4 cases, including 1 with prior incomplete occlusion by Gianturco coils). 4 patients required 2 interventional cardiac catheterizations. Complications occurred only in procedures that were performed with conventional coils (peripheral pulmonary embolizations of platinum coils in 2 patients, dislocation of the delivery catheter with a partially extruded coil to the descending aorta in 1 patient). Operative ligation had to be performed in 6 spca, since those vessels were not suitable for interventional occlusion. The remaining 5 spca were left unoccluded, since they were of minor hemodynamic relevance due to a subsequent decrease in size. At the end of the follow-up period corrective surgery had been completed in 8 of our 10 patients. In the remaining 2 patients corrective surgery is planned in the near future. According to our experience interventional occlusion of spca is a major improvement in the management of a selected cohort of patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot. The introduction of detachable steel coils facilitates the embolization of those vessels.

The intention of this study was to determine the efficacy and complication rate of the interventional duct occlusion. Furthermore, we wanted to compare the meanwhile well-known Rashkind-PDA-Occluder system (ROS) with the results of the considerably cheaper detachable duct occlude coils (PDA-S). For this purpose, we analyzed the data of 53 consecutive patients who received a total of 28 ROS and 25 PDA-S. The ROS was implanted in the slightly larger ducts (O 3.1 +/- 0.6 mm), the PDA-S in the smaller ones (O 2.2 +/- 0.6 mm). The rate of residual shunting was 20% in the ROS-group (follow-up: 104 to 1099 days, mean 580 +/- 300 days) and 7.7% in the PDA-S-group (follow-up: 90 to 384 days, mean 161 +/- 101 days). One patient after ROS showed a significant residual shunting and received 2.5 years after the first procedure a PDA-S which eliminated the shunt. In a second patient the attempt of implanting two coils in a 4 mm duct resulted in the embolization of the first coil into the right pulmonary artery. The coil could not be retrieved and was left in the pulmonary artery without sequelae. All other patients received only one ROS or one PDA-S. There were no further complications. The complication rate for all interventional duct occlusions therefore was 1.9% (0% for the ROS-group; 4.0% for the PDA-S-group). In comparison with the Gianturco coils that have been introduced since 1975, the new detachable coils can be manipulated much more easily, because they remain fixed at the delivery wire until an ideal position is achieved. Then the coil is set free by unscrewing it from the delivery wire. For small ducts with a diameter up to 3 mm the detachable coil is an effective and cheap alternative which can be handled easily. Because of the smaller delivery systems in comparison to the ROS (4-5 F versus 8 F or 11 F) the PDA-S is also suitable for small children and infants. larger ducts should be closed with the ROS.

Since 1983, when c-DNA was isolated, recombinant tissue plasminogen activator (rtPA), an endothelial-cell-produced activator of fibrinolysis is used, more increasingly often in therapy of thrombosis. Whereas some studies have been published regarding efficacy and safety rtPA in different thrombotic states of adults, only case reports exist in children. Doses vary widely (0.8-6 mg/kg/d), bleeding complications are reported in up to 50%. We report on four infants with complex cyanotic congenital heart disease who developed an early post-operative thrombosis of a modified Blalock-Taussig shunt. By local low dosage application of rtPA we could achieve a complete lysis of the thrombus in three of our four patients. In one patient we were unsuccessful due to a distal stenosis of the shunt. This infant required repeat surgery with creation of a central aortopulmonary shunt. We saw severe bleeding in one, requiring transfusion of packed cells, and formation of a perigraft reaction in another patient. In our experience local application of rtPA in low doses is a good therapeutical option in patients with thrombosis of aorto-pulmonary shunts, especially in the first postoperative days.

A balloon-valvuloplasty of the pulmonary valve (BVP) was carried out in 14 patients within the first 40 days of life. Six infants had a critical valvular pulmonary stenosis with intact ventricular septum (group S), 5 infants had a pulmonary atresia with intact ventricular septum (group A), and 3 infants had a tetralogy of Fallot (group F). In 4 of the 5 patients of group A the BVP was preceded by operative opening of the pulmonary valve. In one case radiofrequency perforation of the valve during the first diagnostic cardiac catheterization could be carried out successfully. The average value of the ratio of the balloon diameter to the diameter of the pulmonary valve was 1.31 +/- 0.20. The systolic pressure in the right ventricle dropped 51 mm Hg (from 106 +/- 32 mm Hg to 55 +/- 9 mm Hg) in group S and 33 mm Hg (from 101 +/- 27 mm Hg to 68 +/- 19 mm Hg) in group A. In group F the arterial oxygen saturation increased 15%, however the pressure of the right ventricle was, after the BVP, slightly higher than before. The average residual gradients after BVP were in group S 28 +/- 15 mm Hg, in group A 34 +/- 12 mm Hg and in group F 48 +/- 18 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

In recent years, an increasing number of modified Fontan-operations has been performed in children younger than 4 years of age. The purpose of this study was to identify preoperative risk factors in this age group. From February 1990 until February 1993, we performed in our center a modified Fontan-operation using the technique of total cavopulmonary anastomosis (TCPA) in 37 consecutive patients (17 pts. < 4 years = group I, 20 pts. > 4 years = group II). Early postoperative mortality occurred in patients of group I only (n = 3 pts.). All of these patients had additional preoperative risk factors. Pulmonary vascular resistance (PVRI) > 2 U x m2 was a significant risk factor for the younger patients while pulmonary artery size alone (expressed as the McGoon-ratio or Nakata-index) could not be identified as a separate risk factor. Using two additional indices (McGoon-ratio/PVRI and Nakata-index/PVRI), we were able to identify patients with unfavorable postoperative hemodynamics as high-risk patients. In our experience, TCPA can be performed in patients younger than 4 years of age with a low mortality, if there are no additional preoperative risk factors. For high-risk patients we recommend either a bidirectional Glenn-anastomosis as a first step procedure or a TCPA with fenestration of the intraatrial tunnel-patch.

Aortico-left-ventricular tunnel is a rare congenital cardiac lesion that often results in severe aortic insufficiency in infancy. Normally, the defect appears immediately above the right coronary sinus of Valsalva and consists of a tunnel-like connection between the aortic root and the left ventricle. The differential diagnosis to coronary artery fistulae and ruptured aneurysms of the sinus of Valsalva is often difficult. We describe a patient with an aortico-left-ventricular tunnel originating from the left aortic sinus. Similar findings have been described in only three case reports. Additionally, in our case there was a stenosis in the midportion of the tunnel which was connected to an intraseptal aneurysm communicating with the left ventricular chamber via a "septal" defect. The infant underwent successful surgical correction of this malformation at 1 year and 5 months of age.

Pulmonary hemorrhage due to rupture of pulmonary arteries causes significant morbidity and mortality in the natural history of older patients with pulmonary atresia and ventricular septal defect. The only therapeutical option for patients with persisting pulmonary hemorrhage was surgical resection of the involved pulmonary segments. In this case report we describe the successful termination of an acute pulmonary hemorrhage in a 9-year-old patient by coil-embolization of a systemicopulmonary collateral artery, which provided additional blood supply to the left upper-lobe segments. The hemorrhage ceased within 24 h and has not recurred during a follow-up period of 7 months. In our opinion, the success of the embolization can be attributed to a reduction of the perfusion pressure and a normalization of the arterial blood flow in the affected lung segments.

Between 1970 and 1981 we observed 26 children with pulmonary atresia and ventricular septal defect (PA-VSD). 24 patients had two pulmonary arteries with a bifurcation, one patient had only one central pulmonary artery and one patient had none. Patients in whom lung perfusion was carried out by the ductus arteriosus usually had well developed pulmonary arteries. Palliative surgery (aortopulmonary shunts, Brock procedure, right ventricular outflow tract patch without closure of the VSD) was performed in cases with hypoplastic pulmonary arteries. A comparison of pre- and postoperative angiocardiographies showed an improvement of pulmonary perfusion in all patients. In 3 of 8 children there was an increase in pulmonary artery diameter, which made them more suitable candidates for corrective surgery. Intrapulmonary stenoses and lung segments perfused exclusively by large systemicopulmonary collaterals made corrective surgery impossible in the other 5 children.

Rhabdomyomas are benign cardiac tumors often occurring as multiple lesions. We describe an infant with multiple cardiac rhabdomyomas causing severe right-ventricular and left-ventricular outflow tract obstructions. The intracavitary parts of the rhabdomyomas were successfully resected when the patient was 5 weeks old. Echocardiography showed regression in size of the remaining intramural tumors. Since there is only a low growth potential in cardiac rhabdomyomas, surgical management is indicated only in patients with hemodynamic obstruction or significant arrhythmias. Surgery should be primarily directed to relieve hemodynamic obstruction. If complete resection is not possible without damage to vital structures, resection should be restricted to the intracavitary parts of the tumor.

From 1970 to 1986, 43 patients with pulmonary atresia and ventricular septal defect (PA-VSD) were diagnosed in our hospital. 19 children had a collateral lung perfusion via a ductus botalli (DB), three children had a DB and systemico-pulmonary collateral arteries (SPCA), and 21 children had only SPCA. Patients with a DB alone had significantly larger pulmonary arteries. Children with SPCA always had hypoplastic pulmonary arteries and intrapulmonary arborization anomalies and in a high percentage (19 patients) they showed intrapulmonary stenoses. 26 palliative operations were performed in 24 children. After palliative operations in 15 cases (patients with DB: RVOT-Patch: one patient, aortopulmonary shunt: three patients, Brock procedure: one patient, patients with SPCA: RVOT-Patch: five patients, aortopulmonary shunt: four patients, Brock procedure: one patient) we checked the possibility of corrective surgery with the equation of Alfieri. According to this equation, corrective surgery without excessive right ventricular pressure would have been possible in all post-operatively catheterized patients with DB after primary palliative operations but, due to the pulmonary artery anomalies, only in seven out of nine patients with SPCA (one patient of the latter group was operated twice, Brock procedure and RVOT-Patch). In four cases we could prove the value of the Alfieri equation after corrective operations. There was an excellent correlation between the predicted relation of PRV/PLV and the actually measured pressures. In our opinion, the Alfieri equation forms a valuable aid in the preoperative assessment of children with PA-VSD.