ZUSAMMENFASSUNGHerzinsuffizienz und COPD sind häufig und begegnen uns vielfach gemeinsam. Jede der beiden Erkrankungen führt als Komorbidität der anderen zu einer erhöhten Morbidität und Mortalität, was eine erhöhte Aufmerksamkeit erfordert. Dies gilt in der stabilen Phase und besonders während akuter Dekompensation bzw. Exazerbation. Eine exakte Diagnostik ist notwendig. Sie sollte bei Patienten mit COPD neben klinischen Parametern EKG, Echokardiografie und die Bestimmung eines natriuretischen Peptids umfassen, bei solchen mit Herzinsuffizienz zumindest eine Spirometrie. COPD und Herzinsuffizienz werden jeweils ohne Abstriche leitliniengerecht behandelt. Eine Therapie mit kardioselektiven Betablockern aus kardiologischer Indikation darf COPD-Patienten nicht verweigert werden, wie es vielfach der Fall ist. Vielmehr verbessert sie deren Prognose signifikant.

This document replaces the DGP recommendations published in 1998 and 2013. Based on recent studies and a consensus conference, the indications, choice and performance of the adequate exercise testing method and its necessary technical and staffing setting are discussed. Detailed recommendations are provided: for blood gas analysis and right heart catheterization during exercise, walk tests, spiroergometry, and stress echocardiography. The correct use of different exercise tests is discussed for specific situations in respiratory medicine: exercise induced asthma, obesity, monitoring of rehabilitation or therapeutical interventions, preoperative risk stratification, and evaluation in occupational medicine.

Today, in adult cardiology, the efficacy of beta blockers in patients with congestive heart failure is well established. Beta blockers, as a drug class, provide mortality benefits in comparison with placebo, or standard treatment, in patients with mild to severe heart failure. However, 40 years after Waagstein's first reports in adults and 20 years after our reports in infants with congenital heart defects, beta blockers are still a long way from routine clinical use in pediatric heart failure. Pediatric Cardiology has missed this milestone in heart failure treatment because the necessary clinical studies have not been carried out. However, other pediatricians were more attentive. After using propranolol in infants with a cardiomyopathy due to hemangiomatosis, several new specific pediatric indications for propranolol were unveiled and all appear to be related to its vascular effects: Hemangioma - Lymphatic Anomalies - Retinopathy of Prematurity - Refeeding Edema. Perhaps these new investigations will convince pediatric cardiologists to reevaluate propranolol treatment, especially in infants with univentricular hearts in an effort to improve their very high mortality rate. Furthermore, there have been important findings suggesting that oral propranolol induces a significant decrease in endothelial nitric oxide synthase activity and vascular endothelial growth factor levels in children, both of which are important factors in the development of pulmonary vascular disease. Moreover, based upon our long-time data, we developed our “autonomic imprinting” model, that may explain how early life stress due to infant heart failure may impair growth and cognition, and increase cardiovascular risk in later life.

Canniffe et al. report that the current prevalence of hypertension late after successful coarctation repair is 32.5% (range 25–68%) [ [1] Canniffe C. Ou P. Walsh K. et al. Hypertension after repair of aortic coarctation—a systematic review. Int J Cardiol. 2013; 167: 2456-2461 Abstract Full Text Full Text PDF PubMed Scopus (110) Google Scholar ]. A higher age at the time of surgery, a longer time of follow up and a better definition of hypertension are factors that increase the prevalence. There are insufficient long term data to adequately compare catheter based intervention to surgical procedures for native coarctation in terms of the late prevalence of hypertension. A causative role of the autonomic nervous system resulting in arterial hypertension may be overlooked. We present the first paediatric patient in whom successful interventional renal sympathetic denervation improved postcoarctation repair hypertension.

Objective To study the change of serum inflammatory factors in patients with acute coronary syndrome(ACS) and the possible mechanism of benazepril in stablizing atherosclerostic plaques.Methods Seventy ACS patients were randomly divided into benazepril treatment group (n= 40) and routine treatment group(n = 30).in addition,22 patients with stable angina pectoris (SAP) served as a SAP group and 32 subjects served as control group.Expressions of TLR4, TNF-αand MMP-9 in different groups and their correlation were compared.Results The expression levels of TLR4,TNF-αand MMP-9 were significantly higher in ACS group than in SAP group and control group and significantly lower in benazepril treatment group than in routine treatment group(P0.05,P0.01).Conclusion Benazepril can stabilize the valnerable atherosclerostic plaques in ACS patients and improve their prognosis by down-regulating the over-expression of TLR4 and reducing the TNF-αand MMP-9 secretion in its down stream.

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension have been adopted for Germany. The guidelines contain detailed recommendations for the diagnosis of pulmonary hypertension. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the invasive hemodynamic evaluation of pulmonary hypertension. This manuscript describes in detail the results and recommendations of the working group which were last updated in October 2011.

Background Inspiratory muscle weakness has been described in patients with congestive heart failure (CHF), and only recently in patients with idiopathic pulmonary arterial hypertension. However, the relationship between pulmonary hemodynamics and respiratory muscle function has not been investigated in patients with CHF. Methods and results In two tertial referral centers for CHF patients, 532 consecutive CHF patients (159 female, age 59±12 years, NYHA I–IV) were studied by right heart catheterization, maximal inspiratory mouth occlusion pressure (Pimax) and pressure 0.1 s after beginning of inspiration during tidal breathing at rest (P0.1). There was a significant correlation between Pimax and mean pulmonary artery pressure (PAPm) (r=−0.65, p=0.0023), mean pulmonary capillary wedge pressure (PCWPm) (r=−0.56; p=0.0018), PVR (r=−0.73; p=0.0031), and cardiac output (r=0.51; p=0.0022). Moreover, the ratio P0.1/Pimax showed a linear correlation with PAPm (r=0.54; p=0.0019), and with TPG (r=0.64; p=0.0014) respectively. Vital capacity was reduced in relation to increased PAPm (r=−0.54; p=0.0029). Pimax and P0.1/Pimax were independent from VC. Conclusions This study provides the first evidence of a close relation between inspiratory muscle dysfunction, increased ventilatory drive and pulmonary hypertension in a large patient cohort with CHF. Pimax and P0.1 can easily be measured in clinical routine and might become an additional parameter for the non-invasive monitoring of the hemodynamic severity of disease.

<正>充血性心力衰竭(congestive heart failure,CHF)常导致肺阻力血管重塑和张力增加,而这种适应性反应归因于肺内皮功能障碍,进而加重肺动脉高压,进一步促进右心室衰竭的发生。该文在通过结扎冠状动脉分支远端的主动脉而诱导的大鼠CHF实验模型中,研究者采用实时荧光成像技术检查内皮功能障碍及其可能的分子机

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) have been adopted for Germany. Invasive hemodynamic data obtained by right heart catheterization are essential to confirm the diagnosis, test vasoreactivity, assess severity and guide therapy in PH patients. The definition of PH is resting on a mean pulmonary artery pressure ≥ 25 mm Hg obtained by right heart catheterization. Furthermore, a pulmonary capillary wedge pressure > 15 mm Hg excludes pre-capillary PH. Vasoreactivity testing is part of the diagnostic work-up in pulmonary arterial hypertension. Recent data on the use of inhaled iloprost update these guidelines and are of special importance due to the frequent diagnostic use of iloprost in Germany. Other aspects of invasive hemodynamic data in certain PH subgroups as well as their measurement and interpretation in children are discussed. Several aspects of right heart catheterization in PH justify a detailed commentary, and in some areas an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Paediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups were initiated, one of which was specifically addressing the invasive hemodynamic evaluation of patients with PH. This commentary summarizes the results and recommendations of this working group.

Objectives The study aims to explore the relationship between expressions of toll-like receptor 4 (TLR4) on peripheral blood monocytes, serum tumor necrosis factor-alpha (TNF-α) and matrix metalloproteinase-9 (MMP-9) in patients with acute coronary syndromes(ACS), and to investigate the possible mechanisms of Benazepril stabilizing atherosclerosis plaques. Methods 70 patients selected were randomly divided into Benazepril treatment group (35 patients) and regular treatment group (35 patients). Meanwhile, Stable angina pectoris (SAP) group of 32 patients and control group of 22 patients were also set up. With the help of flow-cytometry, expressions of TLR4 on peripheral blood monocytes of the four groups were analyzed and compared to show differences, correlations and changes of the above mentioned indicators. The concentration of TNF-α and MMP-9 in serum were measured by enzyme linked immunosorbent assay (ELISA). Results (1) Expressions of TLR4, levels of TNF-α and MMP-9 were increased and the rate was rising from the control group, to SAP group and then to ACS group. All these indicators in ACS group are significantly higher than those in other groups (P < 0.05). (ACS versus SAP, control; all (P < 0.05). (2) Multi-linear regression analysis indicates that there was a positive correlation between the expression level of TLR4 and serum levels of TNF-α and MMP-9 in patients with ACS (P < 0.01). (3) There is no significant differences between the expression level of TLR4 and serum levels of TNF-α and MMP-9 in Benazepril treatment group and regular treatment group before treatment (P > 0.05) while they all fell after treatment (P < 0.05). In addition, all the indicators decreased more greatly than the regular treatment group. Conclusions TLR4 on peripheral blood monocytes and serum TNF-α and MMP-9 in patients with coronary arteriosclerosis disease may be effective markers of the vulnerable plaque. Benazepril can inhibit over-expression of TLR4 and reduce serum levels of TNF-α and MMP-9, thus stabilize the vulnerable plaques and improve the condition of the patients with ACS.

急性冠脉综合征（ACS）发病通常与不稳定性动脉粥样硬化斑块破裂、血栓形成并导致冠状动脉血流受限相关。及早发现并稳定易损斑块治疗对阻止或限制其再次发生破裂及降低临床不良事件具有重要作用。目前干预ACS患者动脉粥样硬化不稳定斑块的药物研究多局限于他汀类药物，至于血管紧张素转换酶抑制剂（ACEI）能否通过抑制ACS患者TLR4炎性细胞信号通路，从而发挥稳定斑块的作用，目前仍不甚明确。

Congestive heart failure (CHF) causes lung endothelial (EC) dysfunction, which in turn aggravates lung vascular remodeling and right ventricular failure. Here, we elucidated the mechanisms underlying lung EC dysfunction in a rat model of CHF induced by supracoronary aortic banding. Lung EC dysfunction was evident in CHF as impaired EC‐dependent vasodilation and NO synthesis in response to mechanical stress, acetylcholine or histamine, although expression of endothelial NO synthase was unmitigated. Reconstitution of cytosolic Ca2+ ([Ca2+]i) signaling by Ca2+ ionophore restored NO production. Impaired lung EC [Ca2+]i homeostasis and signaling in CHF in response to mechanical stress, histamine, acetylcholine or thapsigargin was confirmed by real‐time fluorescence imaging, yet was not attributable to downregulation of EC Ca2+ influx channels. Rather, we identified a massive remodeling of the endothelial cytoskeleton by increased β‐actin expression and F‐actin formation, which contributed critically to endothelial dysfunction in CHF, since cytoskeletal disruption by cytochalasin reconstituted endothelial [Ca2+]i signaling and NO production. Our findings show that lung EC dysfunction in CHF results from impaired EC [Ca2+]i signaling, and identify the endothelial cytoskeleton as critical regulator of EC [Ca2+]i homeostasis.Sponsored by EU IP Pulmotension and Kaiserin‐Friedrich Foundation Berlin.

We report a case of a 20-year-old white woman with the history of anorexia nervosa presenting with spontaneous pneumomediastinum (SPM). On admission, her body mass index (BMI) was 9.9 kg/m(2). Physical examination revealed subcutaneous crepitation especially in the axillae, the intercostal spaces, between the scapulae and along the spine. A chest X-ray showed extensive tissue emphysema, especially in the upper mediastinum. In a computed tomography (CT) scan, additional air was found in the upper retroperitoneal space adjacent to the stomach and to the left of the aorta. The patient recovered clinically within three weeks, and a CT scan showed a complete remission of the pneumomediastinum and subcutaneous emphysema. Based on this, case review of the literature about the frequency of pneumomediastinum in young patients with low weight is presented concerning epidemiology, etiology, symptoms, diagnosis, treatment, time to recovery and prognosis.

Aims: To investigate the long-term safety of inhaled iloprost in patients with pulmonary hypertension (pH), including idiopathic PAH (IPAH group) and other forms of pulmonary hypertension (PHother).Methods and results: Sixty-three patients (IPAH group, n = 40, PHother n = 23) were enrolled to receive inhaled iloprost either from baseline or after 3 months in a prospective, open-label 2-year study. Iloprost was inhaled 6-9 times daily with a night pause employing a jet nebulizer delivering an inhaled single dose of 4 mu g at the mouthpiece. In the case of side effects the single dose was reduced to 2 mu g. Sixty patients received at least 1 dose of inhaled iloprost. Thirty-six patients completed at least 630 days of therapy (25 IPAH, 11 PHother), 19 patients dropped out prematurely and 8 patients died (3 IPAH, 5 PHother). There were no drug-induced toxicities and only mild to moderate side effects. The most common side effects were coughing and flushing. Two-year survival was estimated at 85% (IPAH group 91%, PHother 78%). A modified analysis was performed to correct for differential drop-out. It included follow-up data from the premature discontinuations and revealed a 2-year survival of 87% [95% CI, 76%-98%] in the IPAH group while the predicted survival was 63%. The iloprost dose increased by 16% over 2 years.Conclusion: Inhaled iloprost is well tolerated as long-term therapy and no substantial dose increase is required. Although uncontrolled, the data suggest a long-term clinical benefit from continued therapy with inhaled iloprost. (C) 2010 Elsevier Ltd. All rights reserved.