[Differential Diagnosis of Pulmonary Hypertension Using the Example of Collagenosis-associated PAH in the Context of Chronic Lung and Left Heart Disease]. / Differenzialdiagnostik der pulmonalen Hypertonie am Beispiel der Kollagenose assoziierten PAH im Kontext chronischer Lungen- und Linksherzerkrankungen.

Pulmonary hypertension (PH) can be diagnosed in the context of connective tissue diseases (CTD) as well as in elderly patients with multiple comorbidities. A correct clinical differential diagnosis and classification is essential before adequate therapeutic decisions can be made. Differential diagnosis of PH in CTD comprises associated pulmonary arterial hypertension (APAH), group 2 or 3 PH (PH arising from left heart or chronic lung disease), chronic thromboembolic PH (PH) and group 5 (e. g. in the context of terminal renal insufficiency). This is also true of elderly patients in whom the decision has to be made if the increasing number of coincident diseases lead to PH or have to be interpreted as comorbidities. In this manuscript, the differential diagnosis of PH is elucidated, focusing on CTD, in the context of left heart disease and chronic lung disease. Furthermore, criteria are presented facilitating an objective approach in this context.

[Current Aspects of Definition and Diagnosis of Pulmonary Hypertension]. / Aktuelle Definition und Diagnostik der pulmonalen Hypertonie.

At the 6th World Symposium on Pulmonary Hypertension (WSPH), which took place from February 27 until March 1, 2018 in Nice, scientific progress over the past 5 years in the field of pulmonary hypertension (PH) was presented by 13 working groups. The results of the discussion were published as proceedings towards the end of 2018. One of the major changes suggested by the WSPH was the lowering of the diagnostic threshold for PH from ≥ 25 to > 20 mmHg mean pulmonary arterial pressure, measured by right heart catheterization at rest. In addition, the pulmonary vascular resistance was introduced into the definition of PH, which underlines the importance of cardiac output determination at the diagnostic right heart catheterization.In this article, we discuss the rationale and possible consequences of a changed PH definition in the context of the current literature. Further, we provide a current overview on non-invasive and invasive methods for diagnosis, differential diagnosis, and prognosis of PH, including exercise tests.

[Decompensated right heart failure, intensive care and perioperative management in patients with pulmonary hypertension]. / Dekompensierte Rechtsherzinsuffizienz, Intensiv- und Perioperativ-Management bei Patienten mit pulmonaler 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 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 management of decompensated right heart failure, intensive care management and perioperative management in patients with pulmonary hypertension. This article summarizes the results and recommendations of the working group on decompensated right heart failure, intensive care and perioperative management in patients with pulmonary hypertension.

[Pulmonary hypertension due to chronic lung disease: Recommendations of the Cologne Consensus Conference 2016]. / Pulmonale Hypertonie bei Lungenkrankheiten: Empfehlungen der Kölner Konsensus-Konferenz 2016.

The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) associated with chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, sGC stimulators) have not been sufficiently investigated in other forms of PH. Therefore, the European Guidelines do not recommend the use of these drugs in patients with chronic lung disease and PH. This recommendation, however, is not always in agreement with medical ethics as physicians feel sometimes inclined to treat other form of PH which may affect quality of life and survival of these patients in a similar manner. To this end, it is crucial to consider the severity of both PH and the underlying lung disease. 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, to discuss open and controversial issues surrounding the practical implementation of the European Guidelines. Several working groups were initiated, one of which was dedicated to the diagnosis and treatment of PH in patients with chronic lung disease. The recommendations of this working group are summarized in the present paper.

[Dyspnoea in Patients with Pulmonary Hypertension (PH) - a Survey in Spezialized German PH Centres]. / Dyspnoe bei Patienten mit Pulmonaler Hypertonie (PH) - Ergebnisse einer Befragung in deutschen PH-Zentren.

Dyspnoea is the predominant symptom in patients with pulmonary hypertension (PH) at diagnosis. However, since dyspnoea is nonspecific and often occurs in a number of common diseases, the presence of PH can easily be underdiagnosed.In addition, this symptom underlies a high variability in the subjective perception, therefore further diagnostic procedures are often delayed by the patients.A survey of the incidence and severity of dyspnoea in 372 patients with PAH was conducted by questionnaire in German centres. Age, sex distribution and the range of comorbidities corresponded to the findings of national and international registries.Approximately 99 % of patients reported the presence of dyspnoea on exertion, even at low loads.Remarkably, in 13 % of patients dyspnoea occurs as a paroxysmal symptom, which may lead to the differential diagnosis of bronchial asthma. In addition, the patients who were being followed in specialized PH centres reported an increase in dyspnoea during the last year.The results of the survey on the incidence of dyspnoea in patients with PAH are consistent with the findings of international studies.

Six-minute walk distance target in elderly patients with idiopathic pulmonary arterial hypertension - consideration of predicted values.

BACKGROUND: The number of elderly patients diagnosed with idiopathic pulmonary arterial hypertension (IPAH) is increasing. A 6-min walk distance (6MWD) > 380 m on therapy is associated with improved survival in IPAH. As exercise capacity is naturally decreasing with age, treatment goals for elderly patients might be different from those established for younger patients. METHODS: Consecutive adult patients diagnosed with IPAH between 1/2005 and 7/2012 were retrospectively included. We analysed the prognostic value of 6MWD targets (in metres and % predicted) with respect to age at diagnosis. RESULTS: The mean (± SD) 6MWD in 23 patients aged > 65 years at diagnosis compared with 22 younger patients was significantly lower at baseline (262 ± 69 vs. 356 ± 128 m; p = 0.011) and first follow up (310 ± 75 vs. 377 ± 137 m; p = 0.045), while the corresponding % predicted values were similar (62 ± 16% vs. 63 ± 21%; p = 0.854, and 74 ± 16% vs. 68 ± 21%; p = 0.267). The established treatment target of > 380 m 6MWD was met by only 13% of elderly compared with 46% of younger patients (p = 0.016), while the corresponding 6MWD of 78% predicted was exceeded by similar proportions (48% and 46%, respectively; p = 0.873). Survival of patients walking > 380 m or 78% predicted on first follow up was significantly better compared with patients not exceeding the respective threshold (100% vs. 72% and 95% vs. 67% at the end of follow up, respectively; p < 0.05 each). CONCLUSION: Expression of the 6MWD target as % predicted equalises age-related differences and has similar prognostic power compared with absolute values in patients with IPAH. As elderly patients are less likely to reach the absolute 6MWD threshold, the use of % predicted could be helpful in their individual assessment and adaption of targeted therapy.

Effects of simvastatin on pulmonary fibrosis, pulmonary hypertension and exercise capacity in bleomycin-treated rats.

AIM: Pulmonary fibrosis is often complicated by pulmonary hypertension. Statins reduce fibroblast activity in vitro and pulmonary hypertension in vivo. We investigated whether Simvastatin exerts beneficial effects on pulmonary fibrosis and pulmonary hypertension in Bleomycin-treated rats in vivo. METHODS: Rats were randomly assigned to controls, Bleomycin, Bleomycin plus Simvastatin from day 1 to 28 and Bleomycin plus Simvastatin from day 13 to 28. 28 days after Bleomycin instillation, right ventricular systolic pressure (RVSP), right ventricular mass (RV/(LV+S)), right ventricular and circulating brain natriuretic peptide (BNP) levels were determined to assess pulmonary hypertension. Pulmonary hydroxyproline content (HPC), pulmonary connective tissue growth factor (CTGF) transcription and lung compliance (LC) were analysed to characterize pulmonary fibrosis. Exercise capacity was determined by treadmill tests. RESULTS: Compared with controls, Bleomycin increased RVSP, RV/(LV+S), BNP levels, HPC and CTGF transcription and decreased LC significantly. Simvastatin administered from day 1 to 28 normalized all these parameters. Simvastatin administered from day 13 to 28 had no effect on HPC and LC, but reduced RV/(LV+S) significantly and induced a strong trend to lower RVSP and BNP levels. Exercise capacity was reduced by Bleomycin. Simvastatin significantly improved exercise intolerance in both treatment groups. CONCLUSIONS: Simvastatin prevents the development of pulmonary fibrosis, but fails to attenuate already established pulmonary fibrosis. In contrast, it ameliorates pulmonary hypertension and thereby exercise capacity in the prevention and the treatment group regardless of its effects on pulmonary fibrosis. Whether statins are a treatment option in humans with pulmonary fibrosis needs to be investigated by further study.

Qualitative echocardiography parameters for prediction of pulmonary hypertension.

BACKGROUND: Current guidelines recommend echocardiography as the preferred screening method for pulmonary hypertension (PH). The probability of PH is primarily assessed by estimating the right ventricular systolic pressure while the value of qualitative parameters is uncertain. The aim of our study was to evaluate the diagnostic yield of qualitative and quantitative parameters from routine echocardiography reports for PH diagnosis. METHODS: Consecutive patients with suspected PH who received right heart catheterisation and echocardiography within 2 weeks between April 2003 and September 2008 were retrospectively included. PH was diagnosed by a mean pulmonary artery pressure of ≥ 25 mmHg on right heart catheterisation. Qualitative and quantitative parameters from echocardiography reports were analysed with respect to their predictive value for PH diagnosis. RESULTS: Inclusion criteria were met by 272 patients. Diagnosis of PH was confirmed in 172 and excluded in 100 patients. Qualitative and quantitative information about the right ventricle was available in 93% and 85% of the echocardiography reports respectively. Qualitative descriptions of the right ventricle to be dilated predicted PH with sensitivity, specificity, negative and positive predictive value of 89%, 63%, 78% and 80% respectively. An estimated right ventricular systolic pressure of ≥ 50 mmHg on echocardiography predicted PH with sensitivity, specificity, negative and positive predictive value of 84%, 69%, 68% and 84% respectively. CONCLUSION: Qualitative parameters are frequently described in routine echocardiography reports and are valuable for the prediction of PH with a similar diagnostic accuracy as the estimation of right ventricular systolic pressure.

Endobronchial ultrasound-guided transbronchial needle aspiration in routine care - plenty of benign results and follow-up tests.

BACKGROUND: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a widespread technique for tissue sampling from hilar and mediastinal lymph nodes (LN). The diagnostic yield of this method is reported to be very high even outside clinical trials. We aimed to assess the diagnostic accuracy of EBUS-TBNA after its implementation at a University hospital. METHODS: We analysed the first 100 consecutive patients who underwent an EBUS-TBNA procedure at our institution with respect to indication and cytological results. Sensitivity and negative predictive value (NPV) were calculated on the basis of histological confirmation or follow-up. RESULTS: From 03/2007 to 03/2008, EBUS-TBNA of 218 LNs was performed on the basis of chest computed tomography. The primary indication for EBUS-TBNA was lymphadenopathy of unknown cause with (44%) or without (37%) pulmonary nodule(s). Only 19% of patients had known cancer and underwent the procedure for (re-)staging. In 73% of patients a non-diagnostic cytology was reported. A diagnosis could be established in only 27% by EBUS-TBNA including four patients with sarcoidosis. Sensitivity and NPV were low with 61.4% and 76.7%, respectively. Diagnostic yield increased over time and was better in cancer patients than in patients with incidental lymphadenopathy. CONCLUSION: Although EBUS-TBNA is reported to have a very high diagnostic yield in selected patients, the predominant finding in routine care, depending on the patient population, can be a non-diagnostic cytology result with the need for surgical procedures or follow-up studies. This should be considered in the approach to patients with mediastinal or hilar lymphadenopathy.

[Pulmonary hypertension due to chronic lung disease. Recommendations of the Cologne Consensus Conference 2010]. / Pulmonale Hypertonie bei chronischen Lungenerkrankungen. Empfehlungen der Kölner Konsensus-Konferenz 2010.

The 2009 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) in chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors) have not been sufficiently investigated in other forms of PH. Therefore, the European Guidelines do not recommend the use of these drugs in patients with chronic lung disease and PH. This recommendation, however, is not always in agreement with medical ethics as physicians feel sometimes inclined to treat other form of pulmonary hypertension which may affect quality of life and survival of these patients in a similar manner. In June 2010, a group of German experts met in Cologne, Germany, to discuss open and controversial issues surrounding the practical implementation of the European Guidelines. The conference was sponsored by the German Society of Cardiology, the German Society of Respiratory Medicine and the German Society of Pediatric Cardiology. One of the working groups was dedicated to the diagnosis and treatment of PH in patients with chronic lung disease. The recommendations of this working group are summarized in the present paper.

[Pulmonary hypertension due to chronic lung disease. Recommendations of the Cologne Consensus Conference 2010]. / Pulmonale Hypertonie bei chronischen Lungenerkrankungen: Empfehlungen der Kölner Konsensus-Konferenz 2010.

The 2009 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) in chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors) have not been sufficiently investigated in other forms of PH. Therefore, the European Guidelines do not recommend the use of these drugs in patients with chronic lung disease and PH. This recommendation, however, is not always in agreement with medical ethics as physicians feel sometimes inclined to treat other form of pulmonary hypertension which may affect quality of life and survival of these patients in a similar manner. In June 2010, a group of German experts met in Cologne, Germany, to discuss open and controversial issues surrounding the practical implementation of the European Guidelines. The conference was sponsored by the German Society of Cardiology, the German Society of Respiratory Medicine and the German Society of Pediatric Cardiology. One of the working groups was dedicated to the diagnosis and treatment of PH in patients with chronic lung disease. The recommendations of this working group are summarized in the present paper.

How judges sentence DUI offenders: an experimental study.

This study examined the impact of 1) a DUI offender's blood alcohol level at the time of arrest and 2) the number of prior drunken driving convictions on judges' sentencing patterns. These variables were manipulated using vignettes that were presented to all judges who sentence drunken drivers in Colorado. Judges were asked to determine appropriate amounts of jail time, fines, public service hours, and alcohol education course work. Results indicated that they gave more severe sentences to offenders with prior records and to those with higher blood alcohol levels. The effects were generally stronger for the first variable, prior record. These findings suggest that judges may perceive of and sentence repeat offenders differently than first-time offenders, regardless of the level of intoxication at arrest.