Today, invasive and non-invasive home mechanical ventilation have become a well-established treatment option. Consequently, in 2010 the German Society of Pneumology and Mechanical Ventilation (DGP) has leadingly published the guidelines on "Non-Invasive and Invasive Mechanical Ventilation for Treatment of Chronic Respiratory Failure". However, continuing technical evolutions, new scientific insights, and health care developments require an extensive revision of the guidelines.For this reason, the updated guidelines are now published. Thereby, the existing chapters, namely technical issues, organizational structures in Germany, qualification criteria, disease specific recommendations including special features in pediatrics as well as ethical aspects and palliative care, have been updated according to the current literature and the health care developments in Germany. New chapters added to the guidelines include the topics of home mechanical ventilation in paraplegic patients and in those with failure of prolonged weaning.In the current guidelines different societies as well as professional and expert associations have been involved when compared to the 2010 guidelines. Importantly, disease-specific aspects are now covered by the German Interdisciplinary Society of Home Mechanical Ventilation (DIGAB). In addition, societies and associations directly involved in the care of patients receiving home mechanical ventilation have been included in the current process. Importantly, associations responsible for decisions on costs in the health care system and patient organizations have now been involved.The currently updated guidelines are valid for the next three years, following their first online publication on the home page of the Association of the Scientific Medical Societies in German (AWMF) in the beginning of July 2017. A subsequent revision of the guidelines remains the aim for the future.

/pdf/guidelines-for-non-invasive-and-invasive-home-mechanical-4r6dr1z2nh.pdf

[Guidelines for Non-Invasive and Invasive Home Mechanical Ventilation for Treatment of Chronic Respiratory Failure - Update 2017].

The prevalence, prognosis, clinical presentation, pathophysiology, diagnosis, and treatment of the central sleep apnoea syndrome (CSAS) are reviewed and its relationship with congestive heart failure (CHF) is discussed. Adequately powered trials are needed with survival and health status as end points to establish whether correction of sleep related breathing abnormalities improves the outcome in patients with CHF.

https://thorax.bmj.com/content/thoraxjnl/57/6/547.full.pdf

Central sleep apnoea syndrome in patients with chronic heart disease: a critical review of the current literature

Background

There is only sparse data on the use of non-invasive ventilation (NIV) in acute respiratory failure (ARF) in infants and children. For this setting we investigated feasibility and efficacy of NIV and aimed to identify early predictors for treatment failure.



Patients and Methods

Retrospective chart review was performed for all patients treated with NIV for ARF from 2003 to 2010 on an 8-bed pediatric intensive care unit of a tertiary university hospital.



Results

Seventy-four patients were treated with NIV. One patient did not tolerate mask ventilation and needed immediate invasive ventilation. Intubation rate of the remaining patients was 23% and mortality 15%. Institution of NIV led to significant improvement of both respiratory and heart rate in all patients within the first hour and to further stabilization within the next 8–10 hr. In patients with NIV success blood gases improved significantly 1–2 hr after starting NIV. Multivariate analysis identified low pH after 1–2 hr to be an individual risk factor for NIV failure. Other factors tested were age, underlying disease, acute respiratory insufficiency versus post-extubation failure (PEF), and 1–2 hr after starting NIV oxygen saturation, respiratory rate, PCO2, and FiO2. Patients with PEF tended to show better outcomes compared to those with acute respiratory insufficiency.



Conclusion

NIV can be effective in infants and children with ARF. Low pH 1–2 hr after start of NIV is associated with NIV failure. It may therefore be useful in the decision to continue or stop mask ventilation. Pediatr. Pulmonol. 2011; 46:1114–1120. © 2011 Wiley Periodicals, Inc.

Non‐invasive ventilation on a pediatric intensive care unit: Feasibility, efficacy, and predictors of success

Tracheostomy and home ventilation in children.

BACKGROUND: Exercise training as a component of pulmonary rehabilitation improves health-related quality of life (HRQL) and exercise capacity in people with chronic obstructive pulmonary disease (COPD). However, some individuals may have difficulty performing exercise at an adequate intensity. Non-invasive ventilation (NIV) during exercise improves exercise capacity and dyspnoea during a single exercise session. Consequently, NIV during exercise training may allow individuals to exercise at a higher intensity, which could lead to greater improvement in exercise capacity, HRQL and physical activity. OBJECTIVES: To determine whether NIV during exercise training (as part of pulmonary rehabilitation) affects exercise capacity, HRQL and physical activity in people with COPD compared with exercise training alone or exercise training with sham NIV. SEARCH METHODS: We searched the following databases between January 1987 and November 2013 inclusive: The Cochrane Airways Group specialised register of trials, AMED, CENTRAL, CINAHL, EMBASE, LILACS, MEDLINE, PEDro, PsycINFO and PubMed. SELECTION CRITERIA: Randomised controlled trials that compared NIV during exercise training versus exercise training alone or exercise training with sham NIV in people with COPD were considered for inclusion in this review. DATA COLLECTION AND ANALYSIS: Two review authors independently selected trials for inclusion in the review, extracted data and assessed risk of bias. Primary outcomes were exercise capacity, HRQL and physical activity; secondary outcomes were training intensity, physiological changes related to exercise training, dyspnoea, dropouts, adverse events and cost. MAIN RESULTS: Six studies involving 126 participants who completed the study protocols were included. Most studies recruited participants with severe to very severe COPD (mean forced expiratory volume in one second (FEV1) ranged from 26% to 48% predicted). There was an increase in percentage change peak and endurance exercise capacity with NIV during training (mean difference in peak exercise capacity 17%, 95% confidence interval (CI) 7% to 27%, 60 participants, low-quality evidence; mean difference in endurance exercise capacity 59%, 95% CI 4% to 114%, 48 participants, low-quality evidence). However, there was no clear evidence of a difference between interventions for all other measures of exercise capacity. The results for HRQL assessed using the St George's Respiratory Questionnaire do not rule out an effect of NIV (total score mean 2.5 points, 95% CI -2.3 to 7.2, 48 participants, moderate-quality evidence). Physical activity was not assessed in any study. There was an increase in training intensity with NIV during training of 13% (95% CI 1% to 27%, 67 participants, moderate-quality evidence), and isoload lactate was lower with NIV (mean difference -0.97 mmol/L, 95% CI -1.58mmol/L to -0.36 mmol/L, 37 participants, moderate-quality evidence). The effect of NIV on dyspnoea or the number of dropouts between interventions was uncertain, although again results were imprecise. No adverse events and no information regarding cost were reported. Only one study blinded participants, whereas three studies used blinded assessors. Adequate allocation concealment was reported in four studies. AUTHORS' CONCLUSIONS: The small number of included studies with small numbers of participants, as well as the high risk of bias within some of the included studies, limited our ability to draw strong evidence-based conclusions. Although NIV during lower limb exercise training may allow people with COPD to exercise at a higher training intensity and to achieve a greater physiological training effect compared with exercise training alone or exercise training with sham NIV, the effect on exercise capacity is unclear. Some evidence suggests that NIV during exercise training improves the percentage change in peak and endurance exercise capacity; however, these findings are not consistent across other measures of exercise capacity. There is no clear evidence that HRQL is better or worse with NIV during training. It is currently unknown whether the demonstrated benefits of NIV during exercise training are clinically worthwhile or cost-effective.

Non-invasive ventilation during exercise training for people with chronic obstructive pulmonary disease

[Non-invasive ventilation in acute respiratory insufficiency].

The term "Ondine's curse" is normally used for congenital central hypoventilation in children. We report on 10 adult patients with this disorder (average age 46 y), who were treated from 1990 to 1996. They were hypercapnic while awake (mean 57 Torr) and during sleep (mean 87 Torr). The CO2-rebreathing response was negative. During exercise test minute ventilation volume did not rise adequately (mean pCO2 : 63 Torr, mean pH: 7.21). Five of these patients underwent emergency intubation before diagnosis, 2 presented with hypoventilation syndrome and 2 with decompensated cor pulmonale, 1 was diagnosed without complaints. 8 patients needed intermittent positive pressure ventilation therapy, 7 of them noninvasive, 1 via tracheal canula. One female patient has already died, the others are under stable clinical condition with the underlying central disorder remaining unchanged. Only 2 patients showed central defects visible in magnetic resonance tomography.

["Ondine's curse" in adults].

PATIENTS AND METHODS From 1988 to 2/1997 we had introduced intermittent positive pressure ventilation (IPPV) in 298 patients. In most cases non-invasive nasal mask ventilation was possible, in 21 patients (7%) a tracheostoma was necessary. These 21 patients were analysed retrospectively due to age, sex, diagnose, ventilation mode, course of illness, home care and costs. RESULTS We had 13 male and 8 female patients, aged 49 years on average (min. 2, max. 84). 90% had neuromuscular diseases especially muscle dystrophies. Ventilation therapy was performed volume controlled with the cannula unblocked during daytime and blocked at night. Eighteen patients had industrial cannulas (72% Shiley, 28% Rusch), 3 patients used silver cannulas. Daily ventilation amounted 24 hours in 7 patients, 6 to 14 hours in 14 patients. During the observed time 7 patients remained in stable health situation, in 9 patients the underlying disease was progressive and 5 of them died. IPPV was performed 50.7 months on an average, in living patients 68.8 months, in died 7.6 months. Fifteen patients lived at home, 5 were cared in nursing home, 1 patient stayed in hospital. Outside the hospital the bigger part of costs was paid by sick funds and care funds, the smaller part by social welfare offices. Often costs were divided. Total costs for caring about 24 hours ventilated patient at home amounted up to 21,000 German marks each month.

[Long-term breathing via tracheostoma].

Introduction Noninvasive intermittent ventilation is usually performed in patients with severe ventilatory pump disorder. From 1988 to 3/1995 we treated 163 patients with the aim of home mechanical ventilation (HMV). Patients and results In March 1993 115 of these 163 patients practiced HMV, 22 had already died and 26 had rejected or broken off ventilation therapy. The 115 patients were classified in three main diagnostic groups: Scoliosis or chest wall disease (n = 76), COPD (n = 11) and neuromuscular disease (n = 28). The mean pCO2 at rest of all patients before ventilation therapy was 56 (+/- 12) Torr and fell to 46 (+/- 5) Torr in the course of therapy. The maximum statical inspiration pressure PImax rose from average 3, 8 (+/- 2, 3) to 4, 9 (+/- 2, 0) kPa. There was a probability of surviving two years after onset of ventilation therapy of 85% in the scoliosis group, of 60% in the neuromuscular group and of 30% in the COPD group. Conclusions According to results of others home ventilation therapy was very successful in patients with chest wall disease. In some patients with neuromuscular disorder quality of life could be improved and life prolonged. Only half of the COPD patients could be treated successfully, whereas the other half had no benefit from noninvasive ventilation therapy.

Noninvasive intermittent ventilation. A prospective data collection in patients with hypoventilation syndrome

PATIENTS We retrospectively analysed the course of 33 patients who had rejected nasal IPPV-therapy (1988 to February 1996). RESULTS The death-rate was higher (48%) compared to nasal IPPV patients in the same time (18%). The patients were divided in 3 main diagnostic groups (COPD, restrictive thoracic wall, neuromuscle disease). We observed the highest death-rate in COPD patients (66%) and the lowest death-rate in the group with scoliosis or chest wall disease (23%). This is the same result tendencially as in patients with nasal IPPV (mortality-rate COPD 66%, restrictive chest wall 6%).

Karg O

Papers

[Non-invasive ventilation in acute respiratory insufficiency].

["Ondine's curse" in adults].

[Long-term breathing via tracheostoma].

Noninvasive intermittent ventilation. A prospective data collection in patients with hypoventilation syndrome

[Patient-related rejection of nasal IPPV therapy. Patients, reasons, follow-up].