Chronic thromboembolic pulmonary hypertension (CTEPH) is a subgroup of pulmonary hypertension that differs from all other forms of PH in terms of its pathophysiology, patient characteristics and treatment. For implementation of the European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension in Germany, the Cologne Consensus Conference 2016 was held and last updated in spring of 2018. One of the working groups was dedicated to CTEPH, practical and controversial issues were commented and updated. In every patient with suspected PH, CTEPH or chronic thromboembolic disease (CTED, i.e. symptomatic residual vasculopathy without pulmonary hypertension) should be excluded. Primary treatment is surgical pulmonary endarterectomy (PEA) in a multidisciplinary CTEPH centre. Inoperable patients or patients with persistent or recurrent CTEPH after PEA are candidates for targeted drug therapy. There is increasing experience with balloon pulmonary angioplasty (BPA) for inoperable patients; this option, like PEA, is reserved for specialised centres with expertise in this treatment method.

In the 2009 European Guidelines on the diagnosis and treatment of pulmonary hypertension (PH), one section covers aspects of pathophysiology, diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The practical implementation of the guidelines for this disease is of crucial importance, because CTEPH is a subset of PH which can potentially be cured by pulmonary endarterectomy (PEA). Nowadays, CTEPH is commonly underdiagnosed and not properly managed. Any patient with unexplained PH should be evaluated for the presence of CTEPH, and a ventilation/perfusion (V/Q) lung scan is recommended as screening method of choice. If the V/Q scan or CT angiography reveals signs of CTEPH, the patient should be referred to a specialized center with expertise in the medical and surgical management of this disease. Every case has to be reviewed by an experienced PEA surgeon for the assessment of operability. In this updated recommendation, important contents of the European guidelines were commented, and more recent information regarding diagnosis and treatment was added.

Several forms of pulmonary disease occur among patients treated with amiodarone, i.e. chronic interstitial pneumonitis, organizing pneumonia, ARDS, a solitary pulmonary mass of fibrosis. The prevalence is estimated to be about 5%. Two major hypotheses of amiodarone-induced pulmonary injury include direct cytotoxicity and a hypersensitivity reaction. Given the frequency and potential severity of amiodarone-induced pulmonary toxicity, early detection is desirable. Unfortunately, there are no adequate predictors of pulmonary toxicity due to amiodarone. Patients who should benefit from amiodarone should be carefully selected and the lowest effective dosage of amiodarone should be taken. Amiodarone-induced pulmonary toxicity is a diagnosis of exclusion. Pulmonary evaluation with chest X-ray and pulmonary function testing, including diffusion capacity for carbon monoxide is recommended when amiodarone is started. A documented decline in the diffusing capacity of greater than 20% is useful in suggesting the need for closer monitoring or for further diagnostic testing. Although the optimal frequency of follow-up has not been determined, most cases of amiodarone-induced lung injury develop during the first 2 years of treatment and disease onset usually is slow. Pulmonary function tests and imaging may be performed every 3-6 months, depending on the presumed individual risk. Treatment of amiodarone pulmonary toxicity consists primarily of stopping amiodarone. Corticosteroid therapy can be life-saving for severe cases and for patients with less severe disease in whom withdrawal of amiodarone is not desirable. Due to its accumulation in fatty tissues and long elimination half-life, pulmonary toxicity may initially progress despite drug discontinuation and may recur after steroid withdrawal. The prognosis of amiodarone lung disease is generally favourable.

BackgroundIncreased ventilatory response has been shown to have a high prognostic value in patients with chronic heart failure. Our aim was therefore to determine the ventilatory efficiency in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension by cardiopulmonary exercise testing (CPET) identifying PH-patients with increased risk for death within 24 months after evaluation.Methods116 patients (age: 64 ± 1 years) with a mean pulmonary arterial pressure of 35 ± 1 mmHg underwent CPET and right heart catheterization. During a follow-up of 24 months, we compared the initial characteristics of survivors (n = 87) with nonsurvivors (n = 29).ResultsSignificant differences (p ≤ 0.005) between survivors and nonsurvivors existed in ventilatory equivalents for oxygen (42.1 ± 2.1 versus 56.9 ± 2.6) and for carbon dioxide (Ve/VCO2) (47.5 ± 2.2 versus 64.4 ± 2.3). Patients with peak oxygen uptake ≤ 10.4 ml/min/kg had a 1.5-fold, Ve/VCO2 ≥ 55 a 7.8-fold, alveolar-arterial oxygen difference ≥ 55 mmHg a 2.9-fold, and with Ve/VCO2 slope ≥ 60 a 5.8-fold increased risk of mortality in the next 24 months.ConclusionsOur results demonstrate that abnormalities in exercise ventilation powerfully predict outcomes in PH. Consideration should be given to add clinical guidelines to reflect the prognostic importance of ventilatory efficiency parameters in addition to peak VO2.

Background: Herpes simplex virus (HSV) replication can be detected in the respiratory secretions of a high proportion of ventilated intensive care unit (ICU) patients. However, the clinical significance remains poorly defined. We investigated whether patients with ventilator-associated pneumonia not responding to antibiotics and in whom high levels of HSV could be detected in respiratory secretions benefit from acyclovir treatment. Methods: Respiratory secretions (bronchoalveolar lavage fluid or tracheal aspirates) were tested for HSV replication by quantitative real-time PCR. ICU survival times, clinical parameters, and radiographic findings were retrospectively compared between untreated and acyclovir treated patients with high (> 10 5 HSV copies/mL) and low (10 3 -10 5 HSV copies/mL) viral load.Results: Fifty-seven low and 69 high viral load patients were identified. Fewer patients with high viral load responded to antibiotic treatment (12% compared to 40% of low load patients, p = 0.001). Acyclovir improved median ICU survival (8 vs 22 days, p = 0.014) and was associated with a significantly reduced hazard ratio for ICU death (HR = 0.31, 95% CI 0.11-0.92, p = 0.035) in high load patients only. Moreover, circulatory and pulmonary oxygenation function of high load patients improved significantly over the course of acyclovir treatment: mean norepinephrine doses decreased from 0.05 to 0.02 μg/kg body weight/min between days 0 and 6 of treatment (p = 0.049), and median PaO 2 /FiO 2 ratio increased from 187 to 241 between day 3 and day 7 of treatment (p = 0.02). Chest radiographic findings also improved significantly (p < 0.001). Conclusions: In patients with ventilator-associated pneumonia, antibiotic treatment failure, and high levels of HSV replication, acyclovir treatment was associated with a significantly longer time to death in the ICU and improved circulatory and pulmonary function. This suggests a causative role for HSV in this highly selected group of patients.