Wir berichten uber einen Patienten mit Budd-Chiari Syndrom welcher unter intravenoser Antikoagulation eine Heparin induzierte Thrombozytopenie entwickelte. Die chirurgische Behandlung bestand aus einer dorsocranialen Leberresektion mit anschliessender hepato-atrialer Anastomose unter Verwendung der extrakorporellen Zirkulation. Die perioperative Antikoagulation wurde mittels Hirudin durchgefuhrt. Erstaunlicherweise wurde wahrend der Operation eine Thrombusbildung im Kardiotomie-Reservoir der Herz-Lungenmaschine beobachtet, obwohl die intraoperativ gemessene Antikoagulationsparameter (ACT und aPTT) im therapeutischen Bereich waren. Mit einem zusatzlichen Bolus Hirudin in das extrakorporelles Circuit und dank Spulung des Reservoirs konnte die Operation ohne weitere thrombotische Ereignisse zu Ende gefuhrt werden.

Background. Despite advances in surgical and interventional techniques, the optimal surgical treatment of severe aortic (re) coarctation and hypoplastic aortic arch is still controversial. Anatomic repair may require extensive dissection, cardiopulmonary bypass, and deep hypothermic circulatory arrest with their inherent risks. The aim of this study was to analyze the outcome of off-pump extraanatomic aortic bypass as a surgical alternative to local repair.Methods. From February 2000 to December 2005, ten consecutive patients (median age 20 years; range, 11 to 38 years) with severe aortic (re) coarctation (n = 4) and (or) hypoplastic aortic arch (n = 7) underwent off-pump extraanatomic aortic bypass through median sternotomy. All but three patients had undergone previous surgery for coarctation and angioplasty or stenting. Three patients underwent concomitant replacement of the ascending aorta because of an aneurysm using cardiopulmonary bypass.Results. Postoperative hospital course was uneventful in all patients. There was no perioperative mortality or significant morbidity. During a mean follow-up of 48 +/- 22 months no patient required additional procedures. All patients were free of symptoms; no patient showed signs of heart failure after follow-up. At last follow-up, no patient presented with claudication, nor any patient experienced orthostatic problems due to a steal phenomenon. During follow-up, hypertension resolved in all patients with residual mild hypertension in two patients.Conclusions. Off-pump extraanatomic aortic bypass is an attractive treatment option for complex aortic (re) coarctation and hypoplastic aortic arch. Perioperative risks are minimized, hypertension is influenced favorably, and midterm survival is event-free.

Objective: Surgical ablation therapy for atrial fibrillation (AF) is a successful and effective treatment option. Recently success rate for the novel St. Jude EPICOR device utilizing high-intensity-focused-ultrasound of >80% has been reported.

Abbildung 1Präoperative Angio-CT der langstreckigen, postduk-

Myocardial dysfunction and arrhythmias may be induced by congenital heart defects, but also be the result of heart surgery with cardiopulmonary bypass (CPB), potentially caused by differential expression of connexin40 (Cx40) and connexin43 (Cx43). In 16 pediatric patients undergoing corrective heart surgery, connexin mRNA expression was studied in volume overloaded (VO group, n = 8) and not overloaded (NO group, n = 8) right atrial myocardium, excised before and after CPB. Additionally, in eight of these patients ventricular specimens were investigated. The atrial Cx43 expression decreased during CPB, which was restricted to the VO group (p = 0.008). In contrast, atrial Cx40 mRNA did not change during CPB. In ventricular myocardium compared to atrial mRNA levels, Cx40 was lower (p = 0.006) and Cx43 higher (p = 0.017) expressed, without significant change during CPB. This study revealed a significant influence of CPB and the underlying heart defect on Cx43 expression.

Abstract: The science of blood groups has made giant steps forward during the last decade. Blood‐group typing of red blood cells (RBCs) is performed on more than 15 million samples per year in Europe, today much less often for forensic reasons than for clinical purposes such as transfusion and organ transplantation. Specific monoclonal antibodies are used with interpretation on the basis of RBC agglutination patterns, and mass genotyping may well be on its way to becoming a routine procedure. The discovery that most blood group systems, whose antigens are by definition found on RBCs, are also expressed in multiple other tissues has sparked the interest of transplantation medicine in immunohematology beyond the HLA system. The one and only “histo‐blood group” (HBG) system that is routinely considered in transplantation medicine is ABO, because ABO antigen‐incompatible donor/recipient constellations are preferably avoided. However, other HBG systems may also play a role, thus far underestimated. This paper is an up‐to‐date analysis of the importance of HBG systems in the alloimmunity of transplantation and autoimmune events, such as hemolytic anemia.

A bstract : The science of blood groups has made giant steps forward during the last decade. Blood-group typing of red blood cells (RBCs) is performed on more than 15 million samples per year in Europe, today much less often for forensic reasons than for clinical purposes such as transfusion and organ transplantation. Specific monoclonal antibodies are used with interpretation on the basis of RBC agglutination patterns, and mass genotyping may well be on its way to becoming a routine procedure. The discovery that most blood group systems, whose antigens are by definition found on RBCs, are also expressed in multiple other tissues has sparked the interest of transplantation medicine in immunohematology beyond the HLA system. The one and only “histo-blood group” (HBG) system that is routinely considered in transplantation medicine is ABO, because ABO antigen-incompatible donor/recipient constellations are preferably avoided. However, other HBG systems may also play a role, thus far underestimated. This paper is an up-to-date analysis of the importance of HBG systems in the alloimmunity of transplantation and autoimmune events, such as hemolytic anemia.

Cardiopulmonary bypass (CPB) may induce serious side effects, potentially leading to myocardial failure. The Na(+)-K(+)-ATPase is a key component for myocardial function. Due to its developmental regulation, results from adult studies cannot be adopted to the situation in childhood. Right atrial myocardium from patients with left-to-right shunts at atrial level (VO, n=8) and those without (NO, n=8) was excised during heart surgery before and after CPB. Na(+)-K(+)-ATPase isoforms ATP1A1 (p=0.008) and ATP1A3 (p=0.038) decreased during CPB, which decrease was restricted to the VO group. This study highlights the importance of the underlying heart defect for susceptibility to the effects of CPB, showing a reduced Na(+)-K(+)-ATPase mRNA expression only in patients with left-to-right shunts on the atrial level. This seemed to be an early molecular event, as apart from one, none of the patients showed heart failure before or after surgery.

A differential expression of sarcoplasmic reticulum calcium-ATPase (SERCA2a) and phospholamban (PLB) characterizes the remodeling process in heart failure and atrial arrhythmias in adult patients. Gender is known to modulate the course and prognosis of different forms of heart disease. We hypothesized that gender plays a role in molecular changes of myocardial calcium regulating components already in childhood. Moreover, we studied the influence of volume overloaded (VO) on SERCA2a and PLB in pediatric patients. Quantitative reverse transcription-polymerase chain reaction was used to measure mRNA expression of SERCA2a and PLB in atrial myocardium from 30 pediatric patients (12 girls, 18 boys). Eighteen patients had VO right atria, and 12 patients had not-overloaded atria (NO). Protein expression was studied by Western blot. In the entire population, SERCA2a and PLB expression was not different between girls and boys. If hemodynamic overload was taken into account, SERCA2a mRNA expression was significantly reduced in the VO group compared with the NO group (P = 0.021). The VO versus NO difference was restricted to boys, which corresponds to a highly significant interaction of gender versus VO status (P = 0.002). The PLB to SERCA2a protein ratio was significantly lower in girls (P = 0.028). The decrease in SERCA2a mRNA expression in VO atrial myocardium and the PLB to SERCA2a ratio of protein expression was modulated by gender in this pediatric population. To our knowledge, this study is the first to show the impact of gender on the differential expression of calcium-regulating components in pediatric cardiac patients.

Differential expression of sarcoplasmic reticulum Ca2+-ATPase (SERCA2a) and phospholamban (PLB) has been shown in heart failure and atrial arrhythmias. We investigated the influence of volume overload and age on their expression in pediatric atrial myocardium. Right atrial specimens from 18 children with volume overloaded right atrium (VO) and 12 patients without overload were studied. Each group was further divided into patients less than and older than 12 months of age. Only in the younger patients SERCA2a was significantly reduced in the VO group. In younger patients PLB mRNA level tended to be lower in VO. The PLB:SERCA protein ratio was significantly reduced in the VO group. Age itself did not influence the SERCA2a and PLB expression, if the hemodynamic overload was not taken into account. This study is the first to show a combined influence of volume overload and age on atrial SERCA2a expression.

Objectives: Anulo-aortic ectasia represents the most common cardiovascular manifestation requiring Surgery in Marfan patients. Aim of this report was to analyze the type of presentation and file incidence of cardiovascular lesions and the clinical follow-up after initial surgery. mainly aortic root repair or replacement. Methods: Between 1/1990 and 6/2003 a total of 71 patients (mean age 29 +/- 17 years, 8-65 years) received first surgical treatment at our institution. 69 received root repair or replacement. 22 patients presented with acute aortic dissection (31%). out of them. 3 pregnant females and 1 just after delivery. Composite graft replacement was performed in the majority of patients (61/7 1 85%). Aortic valve sparing, root repair was performed in 7 patients. supra-coronary graft with refixation of the aortic valve in 2 and replacement of the descending aorta in 1 patient. All patients underwent close clinical and imaging follow-up ill a specialized outpatient consultation. Results: During a mean follow-up interval of 5.2 +/- 1.8 years, 14 patients (20.5%) underwent a total of 27 aortic reoperations. Seven patients had one and 6 patients had up to 4 reoperations: 13/14 patients had chronic aortic dissection. There was no hospital mortality and no major cardiac Or neurologic morbidity following reoperation. During follow-up, 2 patients Suffered front acute type B dissection following aortic root surgery and 3 patients Surprisingly died: 2 from 1 rupture of a normal-sized descending aorta and one front intracranial hemorrhage. These 5 patients had had uncomplicated primary aortic root operation. Conclusions: The incidence of reoperations is significantly higher in patients who presented initially with acute type A aortic dissection than in it those with dilatation only. In addition, this survey demonstrates that unexpected fatal outcome may appear in the remaining native aorta following Uncomplicated elective aortic root surgery. even if the aorta is normal-sized. A close follow-up of all Marfan patients is necessary to detect asymptomatic changes requiring surgery because complex elective redo-operations can be performed with a very low perioperative risk. (C) 2004 Elsevier B.V. All rights reserved.

Background. Current techniques for repair of the right ventricular outflow tract (RVOT) may require interposition of a valved conduit between the right ventricle and the pulmonary artery bifurcation. Recently, the Contegra conduit (Medtronic, Inc.) was introduced as an alternative xenograft tissue for RVOT reconstruction. Promising early hemodynamic and clinical results have been reported so far, but still less is known about mid-term adverse outcome.Methods. A total of 38 Contegra valved conduits (12 to 22 mm) were implanted from October 1999 to June 2004, in 36 children less than 5 years old and in 2 patients 8 and 21 years old. Diagnosis included the following: tetralogy of Fallot (n = 21); pulmonary atresia (n = 4); double outlet right ventricle + pulmonary stenosis (n = 3); d-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (n = 3); truncus arteriosus (n = 3); and other complex malformations (n = 4).Results. There was no mortality following initial surgery and no valved-conduit-related early morbidity. Early postoperative echo cardiographic assessment after 3 months demonstrated favorable hemodynamics in all patients. However, during further follow-up, 5 conduits had to be replaced because of severe stenosis at the level of the distal anastomosis (2 of them had moderate to severe dilatation of the conduit proximally to the valve). Excessive intimal peel formation and severe perigraft scarring reaction were observed in all cases. One child died before surgery.Conclusions. The Contegra valved conduit is an interesting concept for reconstruction of the RVOT. However, because of unpredictable incidence of supravalvar stenosis during mid-term results, we cannot recommend routine use of this material. (C) 2005 by The Society of Thoracic Surgeons.

Using echocardiographic quantification of aortic pulsatility distal to the site of the surgical anastomosis, we evaluated whether the preoperatively impaired poststenotic aortic pulsatility returned to normal after repair of coarctation with a hemodynamically ideal result. Patients who underwent repair of aortic coarctation without residual obstruction were compared to a matched group of normal children. A standardized M-mode echocardiographic evaluation of the aorta at the diaphragmatic level was performed for all patients. Measurements consisted of maximum and minimum aortic diameters, time intervals, and a calculated pulsatility index. Compared to normal children (n = 19), 20 children with operated coarctation and with a hemodynamically ideal result showed a significantly smaller increase in aortic diameter in systole (mean of 29 ± 7% in patients versus 37 ± 7% in normals; p < 0.01). In contrast to patients with coarctation in whom the maximum aortic distension is reached much later during the cardiac cycle, hemodynamically normalized, operated patients in our study had no such delay (maximum aortic pulsation at 28% of cardiac cycle time compared to 27% in normals; p = not significant). The pulsatility index of the poststenotic aorta was clearly lower in operated children (mean, 130 ± 50%/sec) compared to a normal mean value of 202 ± 33%/sec but was still significantly higher than that in patients with unoperated coarctation, who showed a low mean value of 51 ± 24%/sec (p < 0.01). After correction of aortic coarctation with a hemodynamically ideal result, the pulsatility of the poststenotic aorta, severely impaired prior to repair, did not return to normal during the observation period in the patients studied.

An 11-year-old boy with severe mitral regurgitation due to hypereosinophilia caused by infection with a filaria (Mansonella perstans) required mitral valve replacement with a prosthetic valve. During recurrent postoperative hypereosinophilia, the patient experienced severe mitral stenosis due to thrombosis of the mitral prosthesis. Despite adequate anticoagulation, the prosthesis had to be replaced as an emergency with a second prosthetic valve. Permanent control of the eosinophil count was achieved with chronic oral steroid administration. In contrast to other microfilariae, M. perstans is non-pathogenic to humans; nevertheless, longstanding hypereosinophilia may lead to severe cardiac involvement endangering the patient's life.

BACKGROUND AND AIM OF THE STUDY:Beside aortic valve-sparing surgery, a composite graft, homograft or (more rarely) an autograft are the most common options to replace a diseased or destroyed aortic root in adults. Recently, a new stentless xenograft valved conduit (Shelhigh, No-React) was introduced in Europe. This totally biologic conduit is glutaraldehyde cross-linked, detoxified and heparin-treated with No-React; this process eliminates residual glutaraldehyde and ensures stable tissue cross-linking. The initial clinical and hemodynamic results with this porcine valved conduit in the aortic position are presented herein.METHODS:Among 308 patients who underwent thoracic aorta surgery during a 30-month period, 127 had aortic root repair or replacement. The Shelhigh stentless aortic valve conduit was implanted in 35 patients (30 males, 5 females; mean age 68 +/- 7.2 years; mean body mass index 27.5 +/- 4.1 kg/m2). Of these patients, 15 had aortic valve stenosis and ascending aortic aneurysm, 10 had a dilated aortic root with or without aortic regurgitation, four had acute aortic dissection type A, and six had a complex pathology (destructive endocarditis or re-do surgery).RESULTS:One patient with prosthetic valve endocarditis died on postoperative day 1 from uncontrolled septicemia (30-day mortality, 2.7%). There were no conduit-related adverse events in the surviving patients, but one re-exploration was required for bleeding in a re-do case. Transthoracic echocardiography was available in 30 patients after six months, and in 15 patients after 12 months. The mean gradient across the aortic valve was 8.5 +/- 5.1 mmHg (range: 6 to 14 mmHg for conduit sizes 21 to 29 mm).CONCLUSION:The Shelhigh valved conduit shows promising hemodynamic properties. As the conduit contains no fabric or mechanical components, it is ideal for treatment of the infected aortic root, and an excellent alternative to homografts. In older patients, this conduit has the additional advantage that no long-term anticoagulation is required.