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Notes: Zusammenfassung Wir berichten über ein nunmehr 6 Monate altes Kind, welches im Alter von 2 Monaten durch eine Rechtsherzinsuffizienz unklarer Ursache auffiel. In der transthorakalen Echokardiographie und in der sich unmittelbar anschließenden Herzkatheteruntersuchung wurde eine fehlentspringende rechte Pulmonalarterie (RPA) aus der Aorta ascendens diagnostiziert. Die operative Korrektur erfolgte am Folgetag durch Re-implantation der RPA in den Pulmonalarterienstamm unter Erweiterung der Neo-Bifurkation mit Perikardflicken. Der postoperative Verlauf war komplikationslos.    Schlussfolgerung: Eine unklare RV-Dekompensation geht in einigen seltenen Fällen auf eine abnormal entspringende RPA zurück, die initialen Symptome hängen dabei entscheidend vom Ausmaß der sekundären pulmonalen Hypertension ab.

Notes: A bronchial P cell carcinoid, which was negative for all hormones immunocytochemically tested, showed a globular intracytoplasmic inclusion in almost every cell. The inclusions were not clearly distinguishable using the haematoxylin-eosinsafran procedure; they were best demonstrated with the Masson trichrome stain and the Grimelius technique and were easily detected in 1 μm thick Epon sections as target-like structures. On electron microscopy, they were found to be composed of filamentous aggregates entrapping a few endosecretory granules, which showed degenerative changes. The filaments, 8–10 nm in diameter, lacked any periodicity; they were randomly dispersed in the central area and arranged in broken concentric swirls at the periphery of the inclusions. The globules lacked the tinctorial properties of amyloid, but showed a strong immunostaining for keratin-like proteins. A systematic investigation of 12 APUDomas of bronchial or duodenopancreatic origin, using both light and electron microscopy, identified a few filamentous bodies in one case, a somatostatin cell tumour of ampulla of Vater. In both cases, the structures appeared similar to those previously reported in growth hormone cell pituitary adenomas as well as in a few bronchial or gut carcinoids. Whatever their nature, morphological data suggest that they are related to abnormalities in the secretory function, involving the Golgi apparatus, the endosecretory granules and the microtubular microfilamentous system.

Notes: Summary Over the last few years, various devices for the interventional closure of atrial septal defects (ASD) up to a diameter of 20mm have been developed. We report our clinical experience in closing ASD with a diameter larger than 20mm diameter with the Amplatzer Septal Occluder (ASO).¶   Method: The stretched diameter of the ASD was measured by inflating a sizing balloon within the defect until an indentation in the circumference in the balloon could be observed. An ASO with a stent diameter 2–4mm larger than the indentation in the circumference of the balloon was chosen and implanted via 9–12 French sheaths. In contrast to the closure of smaller defects, pullback of the device onto the atrial septum was only performed when the connecting stent of the ASO was completely deployed in order to achieve maximal centering characteristics and optimal support of the retention skirt of the left atrial disc on the edges of the defect. Only then was the right atrial disc deployed and actively configured by advancing the sheath and the delivery cable against the atrial septum. Implantation was only attempted if the atrial septal rims (except the anterior rim around the aorta) measured more than 7mm by echocardiography to avoid injury or disturbance of sensitive intracardiac structures. After placement, the fixation of the device and the mechanical stability was proven by an extensive “Minnesota wiggle”. The ASO was released only when TEE showed no or a trivial residual color flow through the connecting stent; otherwise repositioning was performed.¶   Results: Out of 352 patients (P) with successful closure of interatrial defects, 70 P (age: 1.1–77.3 years) had stretched defects larger than 20mm diameter (median 22mm diameter (20–36), 25/75% quartiles=20/26mm). Mean shunt size was Qp:Qs 2.1:1 (0.7–3.9:1), mean fluoroscopy time 10.9min (0–63). Complete closure could be achieved in 85.7/93.1/100% after 3 months, ¶1 and 2 years, respectively. Besides 3P with persistent atrial fibrillation, only 5 P showed transient atrial tachyarrhythmias, 2 only periprocedural and 3 within the first 3months after implantation were treated with β-blocker. In one patient, an acute embolization of the device occurred because a diminished posterior rim was not visualized by a monoplane TEE probe necessitating surgical explantation and defect occlusion. Despite oversizing the device, no “mushrooming” misconfiguration were observed.¶   Conclusion: Transcatheter closure of large atrial septal defects with the Amplatzer Septal Occluder is feasible, safe and effective. Risk of complications do not seem to occur more frequently than after closure of smaller defects if one adheres to certain sizing and implantation measures. The incidence of transient atrial tachyarrhythmias seems to be low.

Notes: Summary Background: Pediatricians and neonatologists are still reluctant to consider invasive cardiological or cardiosurgical treatment in low body weight infants because it is believed to considerably increase the risk. The aim of this study was to assess the results and complications of percutaneous transcatheter interventions in infants with a weight below 2.5 kilograms. ¶   Methods: Retrospective analysis was undertaken for all patients with a weight below 2.5 kilograms who underwent cardiac catheterization from 01/1994 to 04/1999. During this time 42 diagnostic catheterizations in 29 patients and 27 transcatheter interventions in 24 patients were performed. ¶   Results: Surgery was replaced or effectively postponed in 9 (33%) out of 27 transcatheter interventions. This was possible for pulmonary stenosis, valvular aortic stenosis and aortic coarctation. A stabilization of the hemodynamic situation was possible in 14 patients. An antegrade pulmonary flow was established in 5, an effective interatrial shunt created in 5 and the arterial duct stented in 3 patients. Only 3 patients had no benefit from the intervention; however, there were no deaths nor hemodynamic complications. Arrhythmias occurred in 9% of all catheterizations and interventions but were transient in all cases. Femoral arterial complications were observed in 30% of all arterial catheterizations. ¶   Conclusion: Diagnostic cardiac catheterization and percutaneous transcatheter interventions can be performed with low mortality and acceptable morbidity in low weight infants. Transcatheter interventions can replace surgery, postpone the necessity for surgery or stabilize the hemodynamic situation prior to surgery.

Notes: Summary In children with dilated cardiomyopathy the disease may progress so rapidly that they die during the waiting period before a suitable donor organ is found. Fifteen children of 4 months to 15 years of age had been in congestive heart failure with multiorgan failure due to dilated cardiomyopathy, where intensive medical treatment had failed. After resuscitation, a miniaturized pulsatile ventricular assist device for the mechanical replacement of heart function was implanted. The biventricular assist device (“Berlin Heart”) consists of two extracorporeal pneumatically driven polyurethane blood pumps, with a multi-layer flexible membrane that separates a blood and an air chamber. Four silicon cannulae connect the blood pumps to the patient. A three-leaflet valve prevents blood reflux. The pumps are driven by a pulsatile electropneumatic system. In 12 of the 15 children the bridging to transplantation was successful with a support time of 1 to 98days (mean 24 days). Nine of them were extubated and mobilized while assisted. Three children died during the support time due to hemorrhage, sepsis, and pulmonary failure. In addition, there was one infarction of the arteria cerebri media. To date seven of the transplanted children are in good condition on follow-up. The beneficial effects of ventricular assist device use are well known in adult patients and with special devices it can be transfered to infants and children in whom longer need for support is anticipated. Even in small infants it is an effective method for bridging to cardiac transplantation.

Notes: Summary Follow-up data for 81 consecutive patients with primary pulmonary hypertension (PPH) and pulmonary hypertension related to connective tissue diseases or liver cirrhosis, entered into a multicenter registry in Berlin between 1/96 and 11/99, are described. At entry into the registry the diagnosis of PPH was known for 22±32 months. Hemodynamically, these patients were characterized by a right atrial pressure of 8.6±5 mmHg, a mean pulmonary arterial pressure of 58.9±17 mmHg, a cardiac index of 1.8±0.6 l/min/m2 and a pulmonary vascular resistance of 1574±787dyn×s×cm–5. In about one third of the patients, a restrictive and/or obstructive pulmonary physiology was found while the majority showed signs compatible with small airway disease. Furthermore, diffusion abnormalities were found in about 65% of the patients. When added to conventional medical therapy the treatment with inhaled or continuously infused prostanoids represents a major improvement in the treatment of patients with PPH. Aerosolized iloprost therapy was started in 51 patients and was continued for 12 and 24 months in 20 and 6 patients, respectively. This therapy was well tolerated without any significant changes in pulmonary function or signs of toxicity. About 25% of these patients had to be switched to continuous intravenous therapy due to progressive clinical and hemodynamic deterioration. About two thirds of these “rescue patients” could be stabilized on intravenous therapy and discharged from the hospital. Based on the currently available evidence, the continuous infusion of prostanoids represents an important part of the standard therapy in the treatment of patients with PPH. These first long-term data on inhaled iloprost therapy in this patient group illustrate the potential value of this well-tolerated and effective treatment within the concept of a differentiated treatment plan for patients with PPH. However, the true importance of prostanoid inhalation in comparison to continuous intravenous therapy in PPH remains to be determined in randomized controlled trials.

Notes: Abstract Intracranial pseudolymphoma is a rare tumor of the central nervous system. A 35-year-old woman presented with a frontal subcutaneous tumor. Magnetic resonance imaging revealed a left frontal meningeal tumor involving subcutaneous tissue without bone involvement. The mass was completely removed and the histological aspect of all tumor sections was that of a lymphoid hyperplasia with polyclonal proliferation. These findings were characteristic of pseudolymphoma defined as a hyperplasia of follicular and diffuse lymphoid type with assessment of its polyclonality by immunophenotyping on frozen sections, completed by molecular biology techniques.