[General and supportive therapy of pulmonary arterial hypertension]. / Allgemeine und supportive Therapie der pulmonal arteriellen Hypertonie: Empfehlungen der Kölner Konsensus Konferenz 2016.

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted and supportive treatment of pulmonary arterial hypertension (PAH). 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 2016, 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 general and supportive therapy of PAH. This article summarizes the results and recommendations of this working group.

[Targeted therapy of pulmonary arterial hypertension: Recommendations of the Cologne Consensus Conference 2016]. / Gezielte Therapie der pulmonal arteriellen Hypertonie: Empfehlungen der Kölner Konsensuskonferenz 2016.

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). 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 2016, 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 targeted therapy of PAH. This article summarizes the results and recommendations of the working group on targeted treatment of PAH.

Respiratory Muscle Assessment in Acute Guillain-Barré Syndrome.

PURPOSE: Guillain-Barré Syndrome (GBS) is a life-threatening disease due to respiratory muscle involvement. This study aimed at objectively assessing the course of respiratory muscle function in GBS subjects within the first week of admission to an intensive care unit. METHODS: Medical Research Council Sum Score (MRC-SS), vigorimetry, spirometry, and respiratory muscle function tests (inspiratory/expiratory muscle strength: PImax/PEmax, sniff nasal pressure: SnPna) were assessed twice daily. GBS Disability Score (GBS-DS) was assessed once daily. On days one (d1) and seven (d7), blood gases and twitch mouth pressure during magnetic phrenic nerve stimulation (Pmo,tw) were additionally evaluated. RESULTS: Nine subjects were included. MRC-SS, vigorimetry, PImax, and SnPna increased between d1 and d7. GBS-DS, spirometry and Pmo,tw remained unaltered. Only SnPna correlated closely with the MRC-SS on both d1 (r = 0.77, p = 0.02) and d7 (r = 0.74, p = 0.02). CONCLUSION: SnPna was the only parameter that correlated with MRC-SS, while the current gold standard of spirometry measurement did not.

[Diagnosis and Treatment of Diaphragmatic Dysfunction]. / Diagnostik und Therapie der gestörten Zwerchfellfunktion.

There are many reasons for an impairment of the diaphragmatic function potentially affecting all components of the respiratory pump. Particularly, diagnosis and treatment of unilateral and bilateral phrenic nerve paralysis are challenging. Neuromuscular disorders, trauma, iatrogenic conditions, tumor compression, but also infectious and inflammatory conditions in addition to neuralgic amyotrophy and idiopathic phrenic nerve paralysis are reasons for phrenic nerve paralysis. Primarily, diagnostic procedures include the anamnesis, physical examination, blood gas analysis, lung function testing and the diagnosis of the underlying disease. In addition, specific respiratory muscle testing and respiratory imaging are available today. Current established treatment options include respiratory muscle training, long-term non-invasive ventilation and surgical diaphragm plication in selected patients.

[Discharge medication - what do patients know about their medication on discharge?]. / Entlassungsmedikation - Was weiß der Patient bei Entlassung über seine Arzneimittel?

BACKGROUND: Patients' lack of knowledge about their discharge medication can endanger patients' safety after their hospital stay. This is especially the case with regard to medications that were newly prescribed during the hospital stay and are intended to be used after discharge or medications with an increased risk for adverse drug reactions (high-risk drugs). The aim of this study was to analyse the patients' level of knowledge about their discharge medication and to identify influence factors. METHODS: In a bicentric survey patients were interviewed prior to their discharge from an acute and a geriatric rehabilitation hospital. They were asked about their discharge medication in a structured interview. Influence factors were statistically analysed by Tobit regression. RESULTS: In total, 179 patients were interviewed. On average, patients named 48% of their discharge medication correctly (95% CI: 46-50%). Influence factors for knowledge deficits were the lack of a medication plan, an older age, the hospitalization in a rehabilitation hospital and a long hospitalization. 81% of the patients had at least one drug in their discharge medication, which was newly prescribed during the hospital stay. 11% of those drugs were named correctly, the potency was named correctly in 6%, the indication in 8%. For almost two-thirds of the patients at least one high-risk drug was recommended in the discharge letter, among them most frequently oral anticoagulants and opioid analgesics. 38% of these high-risk drugs were named correctly. CONCLUSION: Our results demonstrate an urgent need to train patients about their discharge medication, especially if medications are included that were newly prescribed during the hospital stay and recommended for further use after discharge or medications with an increased risk of adverse drug reactions. Particularly older patients and patients of a rehabilitation hospital after long hospitalization should be intensively counselled and obtain a medication plan upon discharge.

[Respiratory Muscle Training: State of the Art]. / Atemmuskeltraining: State-of-the-Art.

Specific respiratory muscle training (IMT) improves the function of the inspiratory muscles. According to literature and clinical experience, there are 3 established methods: 1.) resistive load 2.) threshold load and 3.) normocapnic hyperpnea. Each training method and the associated devices have specific characteristics. Setting up an IMT should start with specific diagnostics of respiratory muscle function and be followed by detailed individual introduction to training. The aim of this review is to take a closer look at the different training methods for the most relevant indications and to discuss these results in the context of current literature. The group of neuromuscular diseases includes muscular dystrophy, spinal muscular atrophy, amyotrophic lateral sclerosis, paralysis of the phrenic nerve, and injuries to the spinal cord. Furthermore, interstitial lung diseases, sarcoidosis, left ventricular heart failure, pulmonary arterial hypertension (PAH), kyphoscoliosis and obesity are also discussed in this context. COPD, asthma, cystic fibrosis (CF) and non-CF-bronchiectasis are among the group of obstructive lung diseases. Last but not least, we summarize current knowledge on weaning from respirator in the context of physical activity.

[Standardization of spirometry: 2015 update. Published by German Atemwegsliga, German Respiratory Society and German Society of Occupational and Environmental Medicine]. / Leitlinie zur Spirometrie. Leitlinie der Deutschen Atemwegsliga, der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin und der Deutschen Gesellschaft für Arbeitsmedizin und Umweltmedizin zur Spirometrie.

Spirometry is a simple test and considered the gold standard in lung function. An obstructive ventilatory defect is a disproportionate reduction of maximal airflow from the lung in relation to the maximal volume that can be displaced from the lung. It implies airway narrowing and is defined by a reduced FEV1/FVC ratio below the 5th percentile of the predicted value (lower limit of normal, LLN). A restrictive disorder may be suspected when vital capacity (FVC) is reduced and FEV1/FVC is normal. It is definitely proven, however, only by a decrease in TLC below the 5th percentile of predicted value (LLN). The measurement of TLC by body plethysmography is necessary to confirm or exclude a restrictive defect or hyperinflation of the lung when FVC is below the LLN. 2012 a task force of the ERS published new reference values based on 74,187 records from healthy non-smoking males and females from 26 countries. The new reference equations for the 3-95 age range are now available that include appropriate age-dependent mean values and lower limits of normal (LLN). This presentation aims at providing the reader with recommendations dealing with standardization and interpretation of spirometry.

[Pneumomediastinum related to the second stage of labour - a report on 2 cases]. / Mediastinal- und Subkutan-Emphysem in Folge einer Vaginalgeburt - ein Bericht über 2 Fälle.

We report on 2 primigravidae developing pneumomediastinum and subcutaneous emphysema associated with the second stage of labor. Both of the newborns had a comparatively high birth-weight (3,855 g and 4,245 g, respectively). In the first case, the patient felt a sudden chest pain during expulsion followed by dyspnea and swelling of the face. The birth was terminated by vacuum extraction. In the second case, a mild shoulder dystocia occurred, which could be resolved by McRoberts maneuver. The patient exhibited a swollen face and shortness of breath on the first postpartum day. Physical examination revealed crackly skin over the lower face, both sides of the neck and the front of the chest. In both cases, CT scan of the chest revealed pneumomediastinum and a subcutaneous emphysema of the upper thorax, however without showing a pneumothorax. The first patient underwent bronchoscopy and esophagogastroduodenoscopy; there were no abnormalities detected. The pneumomediastinum and subcutaneous emphysema gradually diminished spontaneously in both cases. Appropriate pain management and empirical antibiotics were applied. Pneumomediastinum during labor and birth, typically with subcutaneous emphysema in the face and neck, is a rare condition, but there are reported cases in the literature. Based on the limited available evidence, it seems to have a benign, self-limiting course. A conservative management appears -appropriate and sufficient.

[Recommendations for respiratory muscle testing]. / Empfehlungen der Deutschen Atemwegsliga zur Messung der Atemmuskelfunktion.

Based on the tremendous impact of impaired respiratory muscle function, tests on their function play a significant role in respiratory and intensive care medicine. Besides differential diagnosing e.g. during prolonged weaning and quantification of impaired respiratory muscle function, e.g. in COPD, neuro-muscular diseases or ventilator-induced diaphragmatic dysfunction, those tests qualify for follow-up assessment, e.g. phrenic nerve lesions or specific respiratory muscle training. In general, (simple) volitional and (complex) non-volitional tests are available. Volitional tests aim at screening for potential respiratory muscle impairment, while non-volitional tests - including ultrasound application - are used to further specify low values assessed by volitional tests and to assess complex clinical conditions (e.g. intubated, sedated patients). Several tests are complementary or additive to each other. Complete assessment for respiratory muscle function, therefore, frequently requires the combination of different test regimes. The current recommendations include in-depth description and practical guidelines for the different tests and approaches to assess respiratory muscle function.

[Understanding ventilator-induced diaphragmatic dysfunction (VIDD): progress and advances]. / Ventilator induzierter Zwerchfellschaden: ein Update.

There is rising evidence that ventilator-induced diaphragmatic dysfunction (VIDD) is not just an artifactual finding from animal studies, but actually occurs in humans undergoing invasive mechanical ventilation. Initial research findings in humans have demonstrated that periods of controlled invasive mechanical ventilation lasting just 18 - 69 hours can lead to a marked reduction in diaphragmatic myofibers. More recently, it has been shown that even short periods (e. g. two-hours) of controlled invasive mechanical ventilation are sufficient to initiate VIDD. The evidence available at present suggests that VIDD is most likely based on increased proteolysis of the respiratory muscles. Moreover, VIDD seems not to be part of a general muscle wasting process, as suggested by the fact that e. g. the human latissimus dorsi and the pectoralis major muscles seem not to be subjected to early muscle fiber atrophy when directly compared to the human diaphragm. Novel in vivo data have also revealed that VIDD in humans is associated with a reduction in diaphragmatic force generation after only one day of controlled invasive mechanical ventilation. This impairment was observed to progress further over the one-week investigation period. The introduction of a simple bedside ultrasound measurement of diaphragmatic function is of great importance to the clinician, as it may serve as a surrogate measure for VIDD, with high predictive value. Regarding potential therapeutic interventions against VIDD, the primary aim should be to encourage sufficient diaphragmatic use in susceptible patients so as to avoid VIDD; this approach remains in fundamental contrast to that of reducing respiratory muscle load by (invasive) mechanical ventilation.

[Learning from failure - implications for respiratory and intensive care medicine: a conceptual review]. / Aus Fehlschlägen lernen - Implikationen für die Pneumologie und Intensivmedizin: Ein konzeptionelles Review.

The clinical, social and economical impact of failure in medicine [i. e., adverse health care events (AHCE)] is overwhelming. Respiratory and intensive care medicine are strongly relevant to AHCE, particularly in cases associated with respiratory failure, mechanical ventilation and pharmacotherapy. In spite of the obvious necessity to learn from AHCE, its realisation in health-care organisations is still rare. This conceptual review therefore aims to (i) clarify the most relevant terminology, (ii) identify obstacles related to this health-care topic, and (iii) present possible strategies for solving the problems, thereby enabling respiratory and intensive care medicine to systematically and effectively learn from failure. A review of the literature (effective as of June 2013) derived from the electronic databases Medline via PubMed, EMBASE, ERIC and Google Scholar identified the following relevant obstacles (ii): a so-called blame culture associated with concealing failure, missing system analyses (vs. individual breakdown), and (economically) misdirected incentives. Possible strategies to overcome these obstacles (iii) include acknowledging the importance of leadership, a safe environment, open reporting, an effective feedback culture, and detection (e. g., trigger-tools), analysis and discussion (e. g., double loop learning) of failure. The underlying reasons for the occurrence of AHCE are based on structural, organisational and human shortcomings, and affect all categories of caregivers. Approaches to solving the problem should therefore focus primarily on the entire system, rather than on the individual alone.

Preserving oxygenation during walking in severe chronic obstructive pulmonary disease: noninvasive ventilation versus oxygen therapy.

BACKGROUND: Physical activity is known to cause significant deoxygenation in patients with severe chronic obstructive pulmonary disease (COPD). Although noninvasive positive pressure ventilation (NPPV) has been shown to improve oxygenation and physical activity in these patients, no practical approach for the application of NPPV during walking has yet been established. OBJECTIVE: To elucidate the most effective approach to preserving oxygenation during walking in patients with severe COPD receiving long-term NPPV. METHODS: Three 12-min walking tests were performed in a randomized cross-over design on 3 consecutive days, comparing the usual and double dosages of oxygen versus NPPV plus the usual dosage of oxygen. The ventilator and oxygen tank were placed in a backpack. RESULTS: Eleven patients (FEV(1) 26 +/- 9% predicted) completed the study, while 8 patients refused to walk with NPPV, due to the weight of the ventilatory device (7.3 kg with NPPV vs. 3.1 kg without). PaO(2) changes during walking differed [p = 0.01, repeated-measures (RM)-ANOVA], whereas dyspnea was unchanged. The difference in PaO(2) change was 14.0 +/- 16.6 mm Hg (unadjusted p = 0.0036, critical level = 0.017, RM-ANOVA) in favor of NPPV compared to the usual dosage of oxygen. Changes in FEV(1), tidal volume and inspiratory impedance were in favor of NPPV-aided exercise (all p < 0.05, RM-ANOVA). Walking distance was reduced under NPPV (555 +/- 227 m) compared to the usual (619 +/- 210 m) and double (622 +/- 215 m) dosages of oxygen (p = 0.024, RM-ANOVA). CONCLUSIONS: NPPV plus supplemental oxygen, but not oxygen alone, preserves oxygenation during walking in patients with severe COPD. However, dyspnea and walking distance were not improved due to the burden of carrying the heavy ventilatory equipment in a backpack.

Exercise in severe COPD: is walking different from stair-climbing?

BACKGROUND: It remains unclear whether the 6-min walking test can predict performance during stair-climbing in severe COPD patients. The present study aimed to assess different pathophysiological changes between walking and stair-climbing in these patients. METHODS: Sixteen COPD patients (mean FEV1 33+/-13% predicted) underwent a 6-min walking test and performed stair-climbing (44 steps) in a randomized, cross-over design. Blood gases, blood lactate, lung function parameters, maximal inspiratory mouth, sniff nasal and twitch mouth pressures, blood pressure, heart rate, and Borg Dyspnea Scale (BDS) were measured before and after exercise. RESULTS: The median drop of PaO2 during walking (2.6 mmHg) and stair-climbing (2.4 mmHg) was comparable (p=0.93). However, stair-climbing caused more dyspnea (median BDS 6.5 vs. 5.5, p=0.01), a higher median blood lactate (1.1 vs. 0.3 mmol/l p<0.001), a more pronounced drop in mean pH (-0.05+/-0.02 vs. -0.03+/-0.03, p=0.02) and a higher increase in mean systolic blood pressure (27+/-11 vs. 13+/-16 mmHg; p=0.009). Stair-climbing, but not walking, caused prolonged lung hyperinflation (mean TLC difference 4.4+/-4.7% predicted, p=0.003). There was no relationship between the 6-min walking distance (314+/-104 m) and the time needed for stair-climbing (55+/-33 s), nor were there any differences in inspiratory muscle strength and heart rate. CONCLUSION: Although the drop of PaO2 was comparable, stair-climbing resulted in more prolonged hyperinflation of the lungs, higher blood lactate production and more dyspnea than walking. The walking distance was not related to the time needed to manage stair-climbing. Therefore, pathophysiological changes during the 6-min walking test do not anticipate those during stair-climbing in patients with severe COPD.

Diabetic polyneuropathy is associated with respiratory muscle impairment in type 2 diabetes.

AIMS/HYPOTHESIS: Diabetes has a major negative effect on intensive care unit outcome. This has been partly attributed to impaired respiratory neuromuscular function. However, data on respiratory neuromuscular involvement in diabetes are lacking. This study therefore aimed to assess respiratory neuromuscular function related to diabetic polyneuropathy in patients with type 2 diabetes. METHODS: Respiratory neuromuscular function was assessed by the use of volitional tests and twitch mouth (TwPmo) and twitch transdiaphragmatic (TwPdi) pressures during non-volitional bilateral anterior magnetic phrenic nerve stimulation in 21 male type 2 diabetic patients without pulmonary disease and in 23 healthy, well-matched controls (forced expiratory volume in 1 s 103 +/- 11 vs 103 +/- 12% predicted; p = 0.9). RESULTS: Both volitionally assessed maximal inspiratory and expiratory mouth pressures, and sniff nasal and transdiaphragmatic pressures were comparable between diabetic patients and controls (p > 0.1 for all). TwPmo was reduced in diabetic patients compared with controls (1.3 +/- 0.5 vs 1.0 +/- 0.4 kPa; p = 0.04), while TwPdi was comparable (1.7 +/- 0.5 vs 1.6 +/- 0.7 kPa; p = 0.6). Following subgroup analysis, patients with no or mild polyneuropathy (n = 10) as assessed by neurological disability scoring had normal respiratory neuromuscular function, whereas patients with moderate or severe polyneuropathy (n = 11) presented with markedly impaired respiratory neuromuscular function as indicated by TwPmo (1.3 +/- 0.4 vs 0.8 +/- 0.3 kPa; p = 0.01) and TwPdi (1.9 +/- 0.6 vs 1.1 +/- 0.4 kPa; p < 0.01). CONCLUSIONS/INTERPRETATION: With regard to volitional tests, diabetes does not affect respiratory neuromuscular function. In contrast, the application of non-volitional phrenic nerve stimulation provides strong evidence that diabetic polyneuropathy, as simply assessed by neurological disability scoring, is associated with substantially impaired respiratory neuromuscular function in type 2 diabetic patients.

[Respiratory muscle testing: state of the art]. / Diagnostik der Atemmuskelfunktion: state of the art.

Assessing respiratory muscle function has become more and more essential both in clinical research and in the broad field of respiratory and intensive care medicine. In clinical practice, the assessment of respiratory muscle function is useful for differential diagnosis, for the quantification of respiratory muscle impairment and for follow-up assessments, for example, following respiratory muscle training or mechanical ventilation. Both volitional tests on respiratory muscle function, which are dependent on the patient making a truly maximal effort, and non-volitional tests exist. Non-invasive, less complex and most often volitional tests are suitable for screening. However, in case of pathological results these tests need to be complemented by the application of more complex, non-volitional and finally invasive techniques. This is particularly true if the cut-off value indicating no impairment is not achieved by the patient. In this case non-volitional tests are required in order to exclude pathological values resulting from sub-maximal efforts during volitional tests. In addition, isolated respiratory muscle impairments (e. g., solely diaphragmatic) might escape notice in certain tests on respiratory muscle function. For this reason, a diagnostic approach with the application of a combination of different tests should be chosen in those cases where respiratory muscle impairment is suspected. This current "state of the art" article gives a detailed description and comparative evaluation of the different volitional and non-volitional tests that are used for the assessment of respiratory muscle function.