Better Late Than Never - A Case of a Congenital Left Partial Anomalous Pulmonary Venous Drainage Diagnosed and Treated in The Sixth Decade of Life.

Partial anomalous pulmonary venous drainage (PAPVD) is a relatively uncommon cardiac anomaly. The diagnosis might be challenging as are the presenting symptoms. Its clinical course mimics more familiar diseases, e.g., pulmonary artery embolism. We present a case of PAPVD, which had been misdiagnosed for more than two decades. After establishing the correct diagnosis, the patient got his congenital anomaly surgically corrected and showed excellent cardiac recovery in the six months follow up.

[Medical care of adults with congenital heart diseases : Present and future]. / Medizinische Versorgung von Erwachsenen mit angeborenen Herzfehlern : Gegenwart und Zukunft.

Congenital heart diseases (CHD) are the most common types of congenital organ defects. Thanks to medical progress in congenital cardiology and heart surgery, most children with CHD reach adulthood. Despite primarily successful treatment residual and subsequent conditions as well as (non)cardiac comorbidities can influence the chronic course of the disease and lead to a higher morbidity and mortality. Adults with congenital heart disease (ACHD) in Germany are not tied to the healthcare structure despite the great need for aftercare. According to the results of the medical care of ACHD (MC-ACHD) study, ACHD centers and specialists in Germany are insufficiently perceived despite increased complication rates and the great need for specialist guidance. General practitioners and patients are not adequately informed about existing ACHD facilities. A better awareness of the ACHD problem should be created at the level of primary medical supply in order to optimize care and to reduce morbidity and mortality. Improved future-oriented patient care includes lifelong regular follow-up and the possibility of interdisciplinary, integrated medical care of CHD.

[Sternal osteomyelitis - Surgical treatment concepts]. / Sternumosteomyelitis - chirurgische Behandlungskonzepte.

Sternal osteomyelitis is a potentially fatal condition following cardiac surgery. Sternal osteomyelitis should be diagnosed as early as possible followed by an interdisciplinary radical debridement. Subsequently plastic reconstructive surgery is necessary for defect reconstruction. This can be achieved by a number of established pedicled and free flap plastic surgery procedures. The choice of flap procedures is based on defect geometry and the individual patient situation, including potential flap donor sites. Smaller defects can generally be reconstructed by pedicled flap transfer. For extensive sternal defects, free flap transplantation is now a well-established therapeutic option. In some patients lacking sufficient recipient vessels, the creation of an arteriovenous (AV) loop as recipient vessel is necessary. In summary, successful therapy of sternal osteomyelitis is based on early interdisciplinary treatment by the various surgical subspecialties.

Procurement regimens to reduce ischemia reperfusion injury of vascular grafts.

BACKGROUND: Ischemia reperfusion injury is an important nonimmunological factor contributing to the development of chronic rejection. The aim of this study was to compare different cell culture media in terms of vascular lesion formation after ischemia reperfusion injury. METHODS: BALB/c aortic grafts were incubated in different cell media (endothelial cell growth, ECG, RPMI-1640 and Waymouth/Ham's F12) for various time spans (5, 6.5 and 8.5 h) at 37°C and implanted into syngeneic BALB/c recipients. On day 30 after implantation, histology, immunofluorescence and morphometric measurements were performed. RESULTS: A total of 36 transplants were performed for this study with an overall survival rate of 72.2%. The most frequent complication was thrombosis of the aortic graft (n = 9) and there was one late death due to other courses. All the recipients with vascular grafts incubated in the ECG medium survived and showed no signs of intimal proliferation independent of the time of ischemia. Aortic grafts incubated in the RPMI medium resulted in a reduced recipient survival rate of 66.7% and grafts incubated in the Waymouth medium showed only a 50% survival by day 30. Analysis of the vascular morphology revealed moderate amounts of intimal proliferation within two aortic grafts in this group. CD31 staining revealed superior endothelial cell integrity after incubation with the ECG medium. CONCLUSIONS: Data from the current study suggest that under optimized conditions vascular grafts can be safely kept in tissue culture up to 8.5 h without significant ischemic damage. Differences in vascular integrity and animal survival depended mostly on the respective tissue culture medium used for the storage of the vessel.

Human cytomegalovirus infection leads to elevated levels of transplant arteriosclerosis in a humanized mouse aortic xenograft model.

Recent findings emphasized an important role of human cytomegalovirus (HCMV) infection in the development of transplant arteriosclerosis. Therefore, the aim of this study was to develop a human peripheral blood lymphocyte (hu-PBL)/Rag-2(-/-) γc(-/-) mouse-xenograft-model to investigate both immunological as well as viral effector mechanisms in the progression of transplant arteriosclerosis. For this, sidebranches from the internal mammary artery were recovered during coronary artery bypass graft surgery, tissue-typed and infected with HCMV. Then, size-matched sidebranches were implanted into the infrarenal aorta of Rag-2(-/-) γc(-/-) mice. The animals were reconstituted with human peripheral blood mononuclear cells (PBMCs) 7 days after transplantation. HCMV-infection was confirmed by Taqman-PCR and immunofluorescence analyses. Arterial grafts were analyzed by histology on day 40 after transplantation. PBMC-reconstituted Rag-2(-/-) γc(-/-) animals showed splenic chimerism levels ranging from 1-16% human cells. After reconstitution, Rag-2(-/-) γc(-/-) mice developed human leukocyte infiltrates in their grafts and vascular lesions that were significantly elevated after infection. Cellular infiltration revealed significantly increased ICAM-1 and PDGF-R-ß expression after HCMV-infection of the graft. Arterial grafts from unreconstituted Rag-2(-/-) γc(-/-) recipients showed no vascular lesions. These data demonstrate a causative relationship between HCMV-infection as an isolated risk factor and the development of transplant-arteriosclerosis in a humanized mouse arterial-transplant-model possibly by elevated ICAM-1 and PDGF-R-ß expression.

Noninvasive magnetic resonance imaging of vessels affected by transplant arteriosclerosis in an experimental mouse aortic allograft model.

BACKGROUND: Transplant arteriosclerosis is still the leading cause of late mortality after heart transplantation despite advances in immunosuppression regimes. Experimental mouse models have substantially contributed to a better understanding of the multifactorial pathogenesis, but the major limitation of these studies is the difficulty in monitoring progression of transplant arteriosclerosis over time. Therefore, the aim of this study was to investigate whether MR measurements are sensitive enough to detect characteristic vascular lesions in a small animal transplantation model. METHODS: For this purpose we investigated 22 iso- and allogeneic aortic graft transplanted mice in vivo with a 4.7 T MR scanner using a 2D-RARE technique, 3D time-of-flight angiography and 3D phase contrast angiography as well as a special snake-based reconstruction algorithm. The MR lumen values of patency from native images and from 3D vessel reconstructions of the respective methods were correlated with conventional histological analysis. RESULTS: A comparison of the different techniques showed that angiographic MR modalities correlated well with histological measurements. 2D-RARE sequences were inferior to the sequences obtained by other ones. Superior correlations and the most accurate results were found for vessel reconstruction based on 3D angiographic time-of-flight data. CONCLUSION: These data demonstrate that mouse in vivo MR imaging is sensitive enough to detect and quantify vascular changes caused by transplant arteriosclerosis.

Transient hypoxia induces ERK-dependent anti-apoptotic cell survival in endothelial cells.

Ischemia-induced apoptosis of endothelial cells may contribute to tissue injury, organ failure, and transplantation rejection. However, little is known about survival mechanisms capable to counteract endothelial apoptosis. This study investigated the potential role of an endogenous anti-apoptotic response elicited by transient hypoxia, capable to avert ongoing apoptosis in endothelial cells. Experiments were carried out in three different types of cultured endothelial cells (human umbilical vein, pig aorta, and from rat coronary microvasculature). As a pro-apoptotic challenge endothelial cells were cultured in serum-free medium and subjected to hypoxia for 2 h. We found that transient hypoxia reduced caspase 3 activation within 1 h of hypoxia. Accordingly, the number of apoptotic cells was reduced after 24 h of reoxygenation. This was true for all three cell types analyzed. Analysis of Akt and mitogen-activated protein kinase kinase (MEK)/extracellular signal-regulated kinase (ERK) pathways revealed that hypoxia induced a transient activation of ERK 2 but not of Akt. ERK 2 phosphorylation preceded the phosphorylation of pro-apoptotic molecule Bad at Ser112, an inhibitory phosphorylation site specific for ERK. The protective effects of hypoxia regarding Bad phosphorylation, caspase 3 activation, and apoptosis were abolished by MEK 1/2 inhibitors, PD98059 or UO126, as well as by antisense oligonucleotides directed against ERK 1/2. Furthermore, inhibition of this pathway inhibited hypoxia-induced increase in mitochondrial membrane potential. The present study demonstrates that transient hypoxia induces a novel survival mechanism that protects endothelial cells against apoptosis. This endogenous process involves MEK/ERK-mediated inhibition of the pro-apoptotic molecule Bad and caspase 3.

Strategies for dose-optimized imaging in pediatric cardiac dual source CT.

PURPOSE: As a result of rapid advances in CT technology, including dual source CT, cardiac CT exams at high heart rates are feasible, making pediatric cardiac CT in congenital heart disease possible. Dose concerns and patient size variability make general recommendations difficult. The aim of this study was to investigate which scanner settings are suitable for multiple weight groups to provide dose-optimized scanning. MATERIALS AND METHODS: All 12 patients underwent a contrast-enhanced gated dual source CT exam. A maximum of 2 ml/kg body weight or 60 ml contrast was applied. Scanner settings at 80 and 120 kV, as well as weight-adapted mAs settings were used in an iterative fashion. Datasets were assessed for image quality and dose-optimized scan parameters were established via class comparison. In a final step a table was created as a recommendation for cardiac CT in children corresponding to their body weight. Strategies for optimized contrast application with hand vs. manual injection were explored. RESULTS: In all children diagnostic image quality was obtained. In children < 60 kg, 80 kV can be used, in all others 100 kV is sufficient. The eff. mAs varied from 362 mAs to 30 mAs depending on body weight. Retrospectively a significant dose reduction would have been possible in 6 / 12 patients. Cardiac CT can be performed between 0.6 and 3.2 mSv in a pediatric population. CONCLUSION: The present study shows that dual source cardiac CT is a potential diagnostic alternative in children with congenital heart disease without excessive radiation exposure or contrast media application.

[Congenital aortic valve stenosis with very high pressure gradient in a pregnant patient]. / Kongenitale Aortenklappenstenose mit sehr hohem Druckgradienten bei einer schwangeren Patientin.

HISTORY AND CLINICAL FINDINGS: A 34-year-old woman was in her 24th week of her second pregnancy when she suffered a recurrence of her congenital aortic valve stenosis (CAS), previously treated by surgical commissurotomy. She was dyspneic on only mild exertion. The first pregnancy had been uneventful. INVESTIGATIONS: RR was 110/70 mmHg. The electrocardiogram showed left ventricular hypertrophy and myocardial damage. Echocardiography revealed a maximal systolic gradient of 155 mm Hg (mean gradient 104 mm Hg). The blood picture showed a pregnancy-related mild anemia and haemodilution. TREATMENT AND COURSE: The patient was hospitalized as a precaution from the 31st week of pregnancy onwards. An elective section was performed under intubation aneasthesia in the 36th week of pregnancy. The aortic valve was electively replaced 13 weeks after delivery. CONCLUSION: Patients with a CAS who had been treated palliatively with a commissurotomy should be carefully and regularly monitored to avoid recurrent stenosis. Close collaboration with the delivery team is essential.

[Computer-assisted process simulation: a suitable instrument for process optimization in hospitals]. / Computerunterstützte Prozesssimulation: Ein Beitrag zur Prozessoptimierung im OP.

The increasing financial pressure on hospitals resulting from changes in the health system demands detailed knowledge about the cost and earnings situation in the hospital. An essential part of strategic controlling now entails establishing structured cost-unit accounting. This can then be used for example through process optimization to ascertain savings potential and rationalization measures. This paper illustrates a possibility of using computer-assisted process simulation to find ways for prozess optimization. The simulation has been based on the treatment process "operative procedure" of a clinical pathway "CABG" developed in our hospital. The starting points for simulation possible prozess optimization consisted in the elimination of existing waiting times, respectively the parallel organization of certain partial processes. The software used for the simulation was Coral iGrafix Process 2003. The results of 1000 simulation processes reveal a clear reduction in the whole lead-time for the patient, both in avoiding waiting times and also in parallel process organization. In contrast to the initial situation (triangular distribution), the overall duration of the treatment section can be described approximately with normal distribution and a clear cluster of minimum overall durations. Computer-assisted process simulation is a suitable instrument for revealing and establishing possibilities for process optimization in hospitals, and therefore makes a valuable contribution to strategic controlling.

Good ecological status in a heavily urbanised river: is it feasible?

In Germany, a national instructions sheet, BWK M3, sets out how the impact of sewer system discharges on receiving water bodies is to be analysed. When applying the simplified assessment procedure defined therein and relying on traditional measures only, it becomes evident that in heavily urbanised rivers a large number of retention facilities would be needed to improve the hydraulic and qualitative conditions. However, the associated economics make the feasibility of such an immission-based approach to river restoration doubtful. In response, the Bergisch Rheinischer Wasserverband and the Ruhrverband started a common research project to find out whether and how the Rinderbach River, a heavily modified water body in terms of morphology and flow dynamics, could achieve the good ecological status required under the European Water Framework Directive. The focus of the survey was on the elaboration of an inventory, the identification of potentially attainable ecological objectives, and the discussion of measures with regard to their benefits and feasibility. The results obtained clearly show that it is possible to achieve good ecological status in already heavily urbanised rivers by making use of all existing natural potential within the river basin. They also demonstrated that a good interactive co-operation of experts in the fields of biology, urban drainage and hydraulics is indispensable.

Cost-efficiency aspects for fish passage restoration in the Ruhr River Basin.

The protection and sustainable management of the aquatic ecosystems is the central aim of the European Water Framework Directive. Due to the aspiration for good ecological status of the water bodies free fish passage will play an important part in river basin management. The Ruhr River has seen severe anthropogenic modifications due to urbanisation and industrialisation in the 19th and 20th centuries leading in the existence of approx. 1300 weirs within the Ruhr River Basin. The majority of the barriers are assessed as not passable or restricted passable. Against this background the Ruhrverband made a holistic approach towards restoring fish passage within the Ruhr catchment. Besides the scientific and technical aspects, such as the determination of potential (spawning) habitats and the development of measures in order to reach them, they also considered cost-benefit-ratio considerations for the Ruhr catchment as a whole as well as for site-specific designs of fish passage structures. Various benefits were evaluated taking into account different fields of economic interest. The model of financing all necessary measures should involve all responsible parties benefiting from the water utilizations. Such a mutual procedure delivers a fair cost distribution as well as an efficient implementation of measures.

[The importance of vacuum therapy in the treatment of sternal osteomyelitis from the plastic surgeons point of view]. / Der Stellenwert der V.A.C.-Therapie in der plastischen Deckung von fortgeschrittenen Sternumosteomyelitiden.

BACKGROUND: Since its introduction in 1997 the vacuum assisted closure therapy has gained widespread and is for its indications international accepted. It is also well established in the treatment of sternal infections a dreaded complication after median sternotomy in cardiac surgery. The well known positive effects of the vacuum therapy act as a dignified cleaning procedure between debridement and plastic coverage or as a temporarily closure method if the first debridement was properly not sufficient. METHOD AND PATIENTS: Between January 2003 and December 2005 twenty eight patients with advanced sternal infection after median sternotomy were treated by radical debridement, vacuum assisted closure therapy and definitive plastic coverage by muscle flaps. In this article three patients are exemplarily introduced. RESULTS: In all patients a sufficient plastic coverage was achieved after radical debridement an vacuum assisted closure therapy. The patients received a pedicaled muscle flap. Stable wound condition with no signs of a recurrent sternal infection were observed in all patients. DISCUSSION: Vacuum assisted closure therapy acts as a link between radical Debridement and definitive plastic coverage in prolonged sternal infection after median sternotomy. Before the invention of V.A.C.(R) dressing changes were obliged every day in patients with deep sternal infection. The V.A.C.(R) therapy reduces the frequency of this painful dressing changes significantly and on this behalf makes life more comfortable for this mostly multi-morbid patients.

Does the additional use of heparin-coated extracorporeal circuits (ECC) optimize the effect of modified ultrafiltration (MUF) in pediatric perfusion?

BACKGROUND: Modified ultrafiltration (MUF) has been shown to exert beneficial effects on the coagulation system and the capillary leak after pediatric cardiac surgery using extracorporeal circulation (ECC). The aim of this study was to investigate whether the additional use of heparin-coated circuits is a useful option for improving biocompatibility. METHODS: We randomized 28 children, using heparin-coated ECC circuits in group A (n = 14) and an uncoated equivalent set in group B (n = 14). After congenital heart surgery, MUF was performed post ECC in a standardized fashion. Blood samples were analyzed preoperatively, 10 min, 30 min, 1 h, and 48 h after ECC by flow cytometric analysis (FACSort) using surface antigens CD62/CD41b (platelets) and CD45/CD14 (monocytes). RESULTS: No significant difference was found with respect to mean age (20.6 months vs. 21.6 months), mean body weight (9.2 kg vs. 8.4 kg), mean ultrafiltration rate (9.1 ml/kg vs. 11.4 ml/kg), chest tube drainage, blood products, ICU stay, and 30-d survival. The percentage of CD62/CD41-positive platelets in group A (vs. B) increased up to 118 % at 60 min vs. 130 % ( P < 0.05) and declined to 98 % at 48 h postop. vs. 99 % (n. s.). The percentage of CD45/CD14-positive monocytes in group A (vs. B) increased up to 158 % at 60 min vs. 155 % (n. s.) and declined to 122 % (A) at 48 h postop. vs. 61 % (B) ( P > 0.05). CONCLUSIONS: Heparin coating of ECC in addition to MUF leads to a lower platelet activation. Monocyte surface markers CD45 and CD14 indicated a marked activation during ECC in both groups but additional heparin coating showed a better postoperative regeneration of monocyte markers in the late course indicating a beneficial additive effect.

Congenital coronary artery fistula draining into the right ventricle: successful surgical closure after failed transcatheter coil embolization.

Coronary artery aneurysms and fistulae are very rare congenital anomalies. They occur in 0.2-0.4 % of all congenital heart diseases. In this article, we report a case of a four-year-old girl with a right coronary artery aneurysm and fistula draining into the right ventricle. Since the transcatheter coil embolization was not successful, surgical occlusion was considered in this case. We describe and discuss the handling of the fistula and the aneurysmatic enlargement of the proximal coronary artery.

Plasmatic [corrected] factor XIII reduces severe pleural effusion in children after open-heart surgery.

Chylous effusions frequently occur after cardiac surgery due to severe damage to the lymphatic system, thus indicating that the insertion of a chest tube may be necessary. Factor XIII (FXIII) is discussed as being essential for wound healing. The aim of this retrospective study was to evaluate whether the application of a single dose of FXIII results in a reduced amount of pleural effusion, leading to an earlier release of patients from the hospital. The cases of 40 children with severe chylous effusions after open-heart surgery were examined. Twenty patients received FXIII and were compared to 20 age- and weight-matched patients who did not receive FXIII. Major parameters included the amount of effusion before and 1 and 3 days after the application of FXIII; the duration of chest tubes; the total amount of fluid loss via drainage; and the period of hospitalization. FXIII levels in plasma showed an inverse correlation with fluid loss. After application of a single dose of FXIII, a significant reduction of pleural effusion within the first 24 hours was detected. However, no difference was observed between the two groups when comparing the total amount of pleural effusions within the first 72 hours. Finally, the duration of hospitalization did not differ between the FXIII-treated and the control group. A single application of FXIII rapidly reduces the amount of chylous effusions in the early period after open-heart surgery. This effect is detectable only for 24 hours after the treatment and does not alter the further clinical outcome. Prospective clinical trials are warranted to determine if repeated application or a higher dose of FXIII may improve the clinical outcome of chylous leakages in children after open-heart surgery.

Cost unit accounting based on a clinical pathway: a practical tool for DRG implementation.

Setting up a reliable cost unit accounting system in a hospital is a fundamental necessity for economic survival, given the current general conditions in the healthcare system. Definition of a suitable cost unit is a crucial factor for success. We present here the development and use of a clinical pathway as a cost unit as an alternative to the DRG. Elective coronary artery bypass grafting was selected as an example. Development of the clinical pathway was conducted according to a modular concept that mirrored all the treatment processes across various levels and modules. Using service records and analyses the process algorithms of the clinical pathway were developed and visualized with CorelTM iGrafix Process 2003. A detailed process cost record constituted the basis of the pathway costing, in which financial evaluation of the treatment processes was performed. The result of this study was a structured clinical pathway for coronary artery bypass grafting together with a cost calculation in the form of cost unit accounting. The use of a clinical pathway as a cost unit offers considerable advantages compared to the DRG or clinical case. The variance in the diagnoses and procedures within a pathway is minimal, so the consumption of resources is homogeneous. This leads to a considerable improvement in the value of cost unit accounting as a strategic control instrument in hospitals.