Transvenous closure of patent ductus arteriosus with Ivalon plugs. Multicenter experience with a new technique.

RATIONALE AND OBJECTIVE: The authors assess the clinical efficacy of transvenous closure of patent ductus arteriosus (PDA) with a new plug consisting of Ivalon foam and a platinum/iridium frame with four aortic and two pulmonary nitinol struts. The most widely used devices for transcatheter treatment of PDA (Porstmann plug, Rashkind umbrella, Botallo occluder, coils) have specific limitations inherent to their design (e.g., transarterial approach, residual shunts, limited retrieval). METHODS: Between 1994 and 1997, PDA closure was attempted in 33 children and 67 adolescents and adults in 7 clinical centers; PDA diameter was 2 to 11 mm. Plug diameter was 6 to 20 mm, and 8 to 16 F venous sheaths were used for insertion. RESULTS: Placement was successful in 98% (with a single plug in 88%, and a second or third plug in 10%) and unsuccessful in 2%. Plugs were retrieved after malpositioning in 12 of 12 patients and after pulmonary embolization in 2 of 3 patients. One patient underwent surgery for removal of an embolized plug. Complete PDA closure was proved by aortography and color Doppler echocardiography in 85% (40 of 43 patients with a PDA 2-3.9 mm, 30 of 36 patients with a PDA 4-5.9 mm, and 15 of 21 patients with a PDA 6-11 mm). During a median follow-up interval of 16 months, there were no complications (infection, hemolysis, fracture, embolization). CONCLUSION: The new plug device can be used successfully in patients with a PDA diameter up to 11 mm. Further investigations are underway to determine the definite clinical value of this technique.

Percutaneous closure of secundum atrial septal defect with a new self centering device ("angel wings").

OBJECTIVE: To investigate the safety, efficacy, and clinical application of a new self centering device ("angel wings") for closure of secundum atrial septal defects (ASD II) and persistent foramen ovale in all age groups. DESIGN: Multicentre, prospective, nonrandomised study. INCLUSION CRITERIA: defects with an occlusive diameter of < or = 20 mm and a surrounding rim of > 4 mm; body weight > 10 kg; and an indication for surgical closure of secundum atrial septal defect. Additionally, there were compassionate indications for closure in patients with persistent foramen ovale. INTERVENTIONS: Defects were closed by a transcatheter device consisting of two square frames made of superelastic nitinol wire. The frames are covered by elastic polyester fabric, which is sewn together at a central circle. All procedures, except for three interventions that were carried out under sedation, were performed under general anaesthesia using transoesophageal echocardiography and fluoroscopy to monitor intervention. RESULTS: Closure was attempted in 75 (71%) of 105 patients. An ASD II was present in 35 children and 15 adults. A persistent foramen ovale was present in 25 adults with suspected paradoxical embolism. Transcatheter closure was unsuccessful in three children and crossover to surgery was required. Residual shunts were found in 20 patients (27%) immediately after the procedure. A transient atrioventricular third degree block occurred in three patients (4%) and the right atrial disk was not fully deployed in three. A minor shunt (< 3 mm) was present in only three (4%) of 72 patients during follow up of 1-17 months. Blood clots on the right atrial disks in two patients (one required lysis) were seen during follow up transoesophageal echocardiography. Serious complications demanding surgical removal of the device occurred in three patients. One patient had haemopericardial tamponade because of an aortic lesion. Left atrial thrombus formation due to an unfolded right atrial disk was found in a second patient and dislodgement of the left atrial disk resulted in a large residual shunt in a third. CONCLUSIONS: Percutaneous closure of a central ASD with a diameter < or = 20 mm in paediatric and adult patients is feasible and effective with this new device. It is a promising alternative to surgical closure. Modifications of the design, however, seem to be mandatory as 4% of patients developed serious complications.

[Anatomic and functional hypoplastic left heart syndrome and its surgical Norwood and Fontan treatment]. / Das anatomische und funktionelle hypoplastische Linksherz-Syndrom und seine chirurgische Behandlung nach Norwood und Fontan.

UNLABELLED: The surgical therapy of newborns with hypoplastic left heart syndrome (HLHS) is still regarded with some distrust. The complete heart conserving palliation includes not only the Norwood operation during the newborn period but also the complete separation of both circuits by the Fontan operation some time later. Our experiences with each surgical step are presented. From 1989 to 1996, 43 infants with anatomical (n = 33) or functional (n = 10) HLHS underwent the Norwood operation. Functional HLHS were: Mitralatresia with double outlet right ventricle and subaortic stenosis (n = 2), atrioventricular septal defect with hypoplastic left ventricle, subaortic stenosis, and aortic coarctation (n = 1), hypoplastic, subaortic right ventricle with restrictive ventricular septal defect and aortic hypoplasia (n = 7). The median age at operation was 15 days (5 to 182 days), mean weight was 3.3 kg (3.0 to 4.9 kg). Total operative mortality was 32% (n = 14) with 16% since 1994 (3/19 patients). Five infants (12%) died 2 weeks to 6 months later, and 2 patients underwent cardiac transplantation. Up to now, 19 out of the 22 long term survivors underwent the bidirectional cavopulmonary anastomosis (Hemi-Fontan) at a median age of 7 months (2 to 14 months). Two infants died (10%). Up to now, 12 out of the remaining 17 survivors received the total cavopulmonary anastomosis after a mean period of 12 months. All children survived, and they are now completely palliated. The longest follow up after the complete Fontan operation is 6 years. CONCLUSION: With increasing experience the results of the Norwood operation improved. The following two-stage Fontan procedure bears only a low risk and leads to good quality of life.

Stentless xenograft valve in a truncus arteriosus communis.

We report the use of a recently developed stentless porcine valve in a 2-year-old child with truncus arteriosus communis (type I). The child had received no previous surgical palliation. Despite a markedly increased pulmonary arteriolar resistance the right ventricle was connected to the pulmonary artery with a homograft. The postoperative course was complicated at first by right ventricular failure due to pulmonary hypertension and secondarily by left ventricular failure caused by rapidly increasing truncal valve incompetence. Because we did not have a suitable homograft at hand we replaced the truncal valve with a stentless porcine xenograft valve.

Early rhythm disorders after arterial switch and intraatrial repair in infants with simple transposition of the great arteries.

Between 1986 and 1990 68 infants with transposition of the great arteries (TGA) have been repaired within their first six months of life. From the 51 simple TGA, forming the study group, 20 underwent intraatrial repair according to Mustard and Brom (group I) and 31 received the arterial switch procedure (group II). We compared the observed rhythm disorders after both surgical methods. There were no deaths in group I and 3 deaths in group II. Group I: 7 cases (35%) had early postoperative arrhythmias, 6 patients received transient therapy. There were 3 cases of supraventricular tachycardia (SVT), 2 cases of frequent supraventricular premature beats (SVPB), 1 infant with temporary AV junctional rhythm and 1 with temporary total AV block. Both needed external pacemaker support for maximal two days. Group II: 14 cases (50%) presented rhythm disorders, 13 patients received transient therapy. There were 4 SVT and 3 SVPB. The time of occurrence was between the first and the sixth postoperative day. 2 cases of temporary AV junctional rhythm and 2 infants with transient total AV block needed external pacemaker support for maximal two days. 3 cases with relative sinus bradycardia (less than 130 beats/min) improved clinically with temporary external pacemaker support for several hours. Finally all patients of both groups were in sinus rhythm and after two months all antiarrhythmic drugs could be stopped. There have not been any ventricular arrhythmias in the early postoperative period. During the whole follow up (mean 28 months, range 4-60 m) no arrhythmias reappeared with two (4%) exceptions.

Primary repair of complete atrioventricular septal defect in infancy.

Forty infants with complete atrioventricular septal defect have undergone primary repair within their 1st year of life. The mean age at time of surgery was 4 months (range 1-12 months) and the mean weight 4.2 kg (range 2.9-7.0 kg). Either the one- or the two-patch method was used. Four patients died (10%). There were no late deaths. Three patients needed reoperation due to mitral valve insufficiency. Postoperative complications were: 1 total atrioventricular block in an infant with an absent ventricular septum 3 weeks after surgery, 9 complete right bundle branch blocks, 3 small residual ventricular septal defects. Mean follow-up of the 36 survivors is 22 months (3-46 months). Thirty (83%) are in NYHA functional class I, 4 (11%) in NYHA class II and 2 (6%) in NYHA class III. No atrioventricular valve regurgitation is detectable by the color Doppler technique in 19 (53%) patients. Mitral incompetence is mild in 13 cases (36%) and moderate in 4 (11%). In ten recatheterized patients the average systolic pulmonary to systemic artery pressure ratio dropped from 1.0 to 0.42 and the average pulmonary arteriolar resistance was reduced from 5.5 to 3.9 U/m2. All but one patient gained weight and underwent adequate statomotoric development. Primary repair should be performed at the age of 5-6 months or even earlier, if pulmonary arteriolar resistance exceeds 5 U/m2.

[Significance of laundry detergent residues from a dermato-toxicological viewpoint]. / Bedeutung von Rückständen von Textilwaschmitteln aus dermatotoxikologischer Sicht.

Detergent residues are often accused to cause efflorescences of the skin. In the present studies different textiles were washed under standardized conditions and the residues analyzed. Textile probes were patch tested for 48 h on seborrheic and sebostatic volunteers. A second series of textiles was given to babies and small children. In all cases no reactions were observed. According to these results and further literature the contribution of detergent residues as a releaser of skin reactions is rather insignificant.

Surgical repair and postoperative course of an infant with infracardiac total anomalous pulmonary venous connection, cor triatriatum sinistrum and transposition of the great arteries.

The combination of a d-transposition of the great arteries, cor triatriatum sinistrum and a total anomalous pulmonary venous connection of the infracardiac type is a very rare condition. Up to now, one surgical repair in an adolescent with transposed great arteries and total anomalous pulmonary venous drainage of the supracardiac type has been reported. In this paper, an infant with the above mentioned cardiovascular malformation is presented. The common pulmonary vein drained into the inferior vena cava and was obstructed. There were arborisation abnormalities in both lungs with mild pulmonary hypertension. The infant has been successfully operated upon at the age of 6 months and a weight of 4.5 kg. The membrane within the left atrium was resected, the common pulmonary vein was anastomosed to the left atrium and a Mustard procedure was performed. During the first 6 postoperative weeks, the infant had problems with adaptation. There was both a transient ballooning of the Mustard patch with significant obstruction of the pulmonary venous drainage and a delayed pulmonary recovery. Two months later, the patch was straightened and the child could be discharged from hospital. After 12 months, the child died from an infection of the airways.

Comparison of two in vitro and two in vivo methods for the measurement of irritancy.

A number of in vitro assays have recently been developed that need further validation in order to judge their value for local tolerance testing. For this purpose, results from the following experiments were compared: (A) cytotoxicity testing in cell culture (neutral red assay); (B) tests with the chorioallantoic membrane of fertilized chicken eggs; (C) rabbit eye mucous membrane tests; and (D) occluded epicutaneous testing in human volunteers. The data presented indicate that in vitro testing with the first two methods yields reliable results with respect to the eye and human skin irritation data within homologous substance classes. A test procedure is proposed that involves comparative testing of the chemicals with unknown irritant properties together with known weak and strong irritants from the same class of chemicals as standards in the test series. This procedure seems to be suitable as a preliminary screen for identifying severe irritants prior to the performance of any in vivo studies. It would reduce the number of animals to be used in vivo and lead to the avoidance of exposure of animals to harmful substances.

[Changes in fatty acid metabolism in dilated cardiomyopathy and hypertrophic cardiomyopathy in childhood]. / Veränderungen im Fettsäuremetabolismus bei dilatativer und hypertropher Kardiomyopathie im Kindesalter.

The aetiology and the pathomechanisms in both types of cardiomyopathy (CM) are still unknown. In vivo measurements of myocardial metabolism in CM may be useful, but there is hardly any information on this subject. Free fatty acids (FFA) are the main source of energy for the normal myocardium. A method for external measurement of the FFA extraction rate (FFA-ER) in different myocardial regions by the simultaneous use of two isotopes has been developed. 201 TI indicates the myocardial perfusion and 15-(p-123 I-phenyl)-pentadecanoid acid (IPPA) represents the FFA uptake. The relation of IPPA/TI reflects the FFA-ER. 8 patients with hypertrophic CM (HCM), age 0.2-20 years, and 8 patients with dilated CM (DCM), age 0.2-18 years were investigated. 12 healthy adults and 4 infants after an arterial switch operation were used as a control group. All patients with HCM showed normal myocardial perfusion but the FFA uptake was strongly diminished, resulting in a reduction in FFA-ER to 42 +/- 12% of the normal value. The maximal influx rate (IR) of FFA was diminished too. In patients with DCM both the myocardial perfusion and the FFA uptake were globally reduced, resulting in a virtually normal FFA-ER. The IR was slightly increased. In HCM and DCM FFA utilisation was disturbed. The alterations were significantly different in both types of CM and different pathomechanisms can be assumed.

Ewing's sarcoma metastazing from the right pelvis via the inferior vena cava into the heart: diagnosis and successful surgical treatment.

A case of Ewing's sarcoma metastazing from the pelvis into the heart is presented. Fainting as a result of circulatory obstruction within the right heart was the leading symptom. Open-heart surgery was performed to excise the tumor from the right atrium and the inferior vena cava. Postoperative angiography showed the tumor coming up out of the right iliac vein and growing along the wall of the inferior vena cava.

Left aortic arch, retro-esophageal aortic segment, right descending aorta and right patent ductus arteriosus--a very rare "vascular ring" malformation.

A vascular ring formed by a left aortic arch, a retro-esophageal segment, a right descending aorta and right-sided ductus or ligamentum arteriosum is a very rare anomaly up to now; only 16 cases have been reported in the literature. This report presents the case of an infant suffering from the symptoms of airway and esophageal compression, who was successfully operated on for this anomaly at the age of 5 months. The diagnosis was established by means of esophagogram and angiography. Diagnostic criteria and guidelines for therapy are discussed.

[The clinical significance of the oxytocin challenge test (author's transl)]. / Klinische Bedeutung des Oxytocinbelastungstests.

In order to test the function of fetoplacental unit 398 oxytocin challenge tests were performed on 280 patients who had exceeded the calculated date of delivery or where factors of risk were involved. 8% of the tests performed showed a positive result, 1% was suspect and 91% were negative. The figure of 20% of false-positive test results was relatively high compared to only 1.6% in the case of false-negative test results. In cases where the calculated date of delivery has been exceeded, pregnant women who showed a negative test result are kept under observation by us as out-patients, whilst patients whose oxytocin challenge test was positive are kept under close observation in hospital.