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Sankt Augustin, Germany

Pieper D.,Witten/Herdecke University | Mathes T.,Witten/Herdecke University | Asfour B.,German Pediatric Heart Center
BMC Pediatrics

Background: The volume-outcome relationship is supposed to be stronger in high risk, low volume procedures. The aim of this systematic review is to examine the available literature on the effects of hospital and surgeon volume, specialization and regionalization on the outcomes of the Norwood procedure.Methods: A systematic literature search was performed in Medline, Embase, and the Cochrane Library. On the basis of titles and abstracts, articles of comparative studies were obtained in full-text in case of potential relevance and assessed for eligibility according to predefined inclusion criteria. All relevant data on study design, patient characteristics, hospital volume, surgeon volume and other institutional characteristics, as well as results were extracted in standardized tables. Study selection, data extraction and critical appraisal were carried out independently by two reviewers.Results: We included 10 studies. All but one study had an observational design. The number of analyzed patients varied from 75 to 2555. Overall, the study quality was moderate with a huge number of items with an unclear risk of bias. All studies investigating hospital volume indicated a hospital volume-outcome relationship, most of them even having significant results. The results were very heterogeneous for surgeon volume.Conclusions: The volume-outcome relationship in the Norwood procedure can be supported. However, the magnitude of the volume effect is difficult to assess. © 2014 Pieper et al.; licensee BioMed Central Ltd. Source

Nagel B.,Medical University of Graz | Janousek J.,University Hospital Motol | Koestenberger M.,Medical University of Graz | Maier R.,Medical University of Graz | And 4 more authors.
Circulation Journal

Background: Adults with transposition of the great arteries (TGA) after atrial switch repair have an increased risk for arrhythmia and sudden cardiac death. We analyzed whether a remote monitoring (RM) system as part of an implantable cardiac device contributes to timely recognition and improved treatment of critical arrhythmias in these patients. Methods and Results: All consecutive TGA patients (n=11) requiring a pacemaker or cardiac resynchronization therapy with or without implantable cardioverter defibrillator between 2008 and 2011 were included. RM-detected arrhythmia, abnormality of device integrity and reaction time from event transmission until acknowledgement via email and clinical decision making were analyzed and compared to a control group (n=21). In 10 patients (91%) 17 arrhythmias were detected, 8 patients (80%) indicated no symptoms. In the RM group time interval from transmission to acknowledgement was 2.4 days (range, 0-4.5 days). Clinical decision-making was advanced by a mean of 77.5 days (range, 10-197 days) compared with conventional follow-up and identified adaption of anti-arrhythmic medication in 8, electrical cardioversion in 2, overdrive pacing in 1 and radiofrequency ablation in 2 patients. A coronary sinus lead fracture was identified in 1 patient followed by successful replacement. Conclusions: RM enables early detection of tachyarrhythmia followed by optimization of medical treatment and potentially life-saving anti-tachycardic intervention in adults after atrial repair of TGA. Source

Hraska V.,German Pediatric Heart Center
Annals of Thoracic Surgery

In failing Fontan circulation, the elevated central venous pressure increases lymphatic production and simultaneously retards lymphatic return to the central venous system, thus unbalancing lymphatic homoeostasis. To improve lymphatic drainage, a new concept based on decompression of the thoracic duct to the lower pressure levels of the common atrium, with concomitant increase of preload, has been developed. The thoracic duct, which in the majority of patients enters the circulation at the left subclavia-jugular junction, is decompressed by diverting the innominate vein directly to the common atrium. © 2013 The Society of Thoracic Surgeons. Source

Aszyk P.,German Pediatric Heart Center | Thiel C.,University of Hamburg | Sinzobahamvya N.,German Pediatric Heart Center | Luetter S.,German Pediatric Heart Center | And 4 more authors.
European Journal of Cardio-thoracic Surgery

Objectives: The aim of study was to analyse the mid-term results of the Ross-Konno procedure in infants. Methods: Between 2000 and 2011, 16 infants, including five newborns, with complex left ventricular outflow tract (LVOT) obstruction underwent the Ross-Konno procedure. Twelve patients (75%) required multiple concomitant procedures such as: mitral valve (MV) surgery (four patients), resection of endocardial fibroelastosis (EFE) and myectomy (six patients), closure of ventricular septal defect (four patients) and aortic arch reconstruction (three patients). The median age at operation was 4.2 months (from 6 to 333 days). Results: There was one late death with a median follow-up of 6.2 years. Actuarial survival is 93.3% at 5 years follow-up (95% confidence interval: 61.2-99.0). Postoperatively, two patients required extracorporeal membrane oxygenation support and one MV replacement. The median length of stay in hospital was 30 days (from 11 to 77 days). At 5 years of follow-up, seven patients had no aortic regurgitation (AR) and nine patients (56%) had trivial AR with no gradient in LVOT. Freedom from mitral regurgitation (MR) ≥ moderate or MV replacement was 70%. MR was associated with either structural abnormalities of MV or with development of EFE. Freedom from redo was 81 and 53% at 1 and 5 years of follow-up. Sixty percent of patients are without medication. All patients are in sinus rhythm. Conclusions: With the technical aspects of this procedure well accomplished, the risk of surgery is minimal and functional outcome is encouraging. However, early postoperative morbidity is significant. At the mid-term follow-up, there was no residual or recurrent outflow tract obstruction and excellent function of the neoaortic valve. A high incidence of MR associated with the development of EFE and structural abnormalities of the MV is worrisome; however, concomitant MV surgery is not associated with increased mortality. In the case of the development of EFE, an early indication for operation might protect MV function. The reoperation rate is high due to early conduit failure. © The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. Source

Yamamoto T.,German Pediatric Heart Center | Schmidt-Niemann M.,German Pediatric Heart Center | Schindler E.,German Pediatric Heart Center
Annals of Emergency Medicine

We report a rare case of acute upper airway obstruction caused by spontaneous retropharyngeal hemorrhage as a result of hemophilia A in a 16-year-old pediatric patient who routinely received factor VIII replacement. Initial diagnosis was delayed because the patient presented with symptoms, such as throat pain and odynophagia, similar to those of common benign upper airway infections. Within 2 days of the initial presentation of symptoms, the patient went into respiratory failure as a result of retropharyngeal hemorrhage. The possibility of spontaneous retropharyngeal or epiglottic hemorrhage or hematoma should be considered as a cause of rapidly progressing odynophagia and dyspnea by hemophilia patients. © 2015 American College of Emergency Physicians. Source

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