[Proposals from a French expert panel for respiratory care in ALS patients]. / Propositions formulées par un groupe d'experts français concernant la prise en charge respiratoire chez les patients atteints de sclérose latérale amyotrophique.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive diaphragm weakness and deteriorating lung function. Bulbar involvement and cough weakness contribute to respiratory morbidity and mortality. ALS-related respiratory failure significantly affects quality of life and is the leading cause of death. Non-invasive ventilation (NIV), which is the main recognized treatment for alleviating the symptoms of respiratory failure, prolongs survival and improves quality of life. However, the optimal timing for the initiation of NIV is still a matter of debate. NIV is a complex intervention. Multiple factors influence the efficacy of NIV and patient adherence. The aim of this work was to develop practical evidence-based advices to standardize the respiratory care of ALS patients in French tertiary care centres. METHODS: For each proposal, a French expert panel systematically searched an indexed bibliography and prepared a written literature review that was then shared and discussed. A combined draft was prepared by the chairman for further discussion. All of the proposals were unanimously approved by the expert panel. RESULTS: The French expert panel updated the criteria for initiating NIV in ALS patients. The most recent criteria were established in 2005. Practical advice for NIV initiation were included and the value of each tool available for NIV monitoring was reviewed. A strategy to optimize NIV parameters was suggested. Revisions were also suggested for the use of mechanically assisted cough devices in ALS patients. CONCLUSION: Our French expert panel proposes an evidence-based review to update the respiratory care recommendations for ALS patients in daily practice.

[High-flow nasal oxygen therapy and hypercapnic acute respiratory failure]. / Oxygénothérapie nasale à haut débit et insuffisance respiratoire aiguë hypercapnique.

Humidified high-flow nasal oxygen therapy (HFNO) has, in recent years, come to assume a key role in the management of hypoxemic acute respiratory failure (ARF). While non-invasive ventilation (NIV) currently represents the first-line ventilatory strategy in patients exhibiting hypercapnic ARF, the operating principles and physiological effects of HFNO could be interesting and useful in the initial management of hypercapnic ARF and/or after extubation, particularly in acute exacerbations of chronic obstructive pulmonary disease. Under these conditions, HFNO could be used either alone continuously or in combination with NIV during breaks in spontaneous breathing, depending on the severity and etiology of the underlying hypercapnic ARF.

[Non-invasive ventilation in acute respiratory failure of oncology-hematology patients: What are its current benefits and limitations?] / Ventilation non invasive dans l'insuffisance respiratoire aiguë des patients d'onco-hématologie : quelle place en 2023 ?

Acute respiratory failure (ARF) is a leading cause, along with sepsis, of admission to the intensive care unit (ICU) of patients with active cancer. Presenting variable clinical severity, ARF in onco-hematological patients has differing etiologies, primarily represented by possibly opportunistic acute infectious pneumonia (de novo hypoxemic ARF), and decompensation in chronic cardiac or respiratory diseases (e.g., acute pulmonary edema or exacerbated chronic obstructive pulmonary disease). In these patients, orotracheal intubation is associated with a doubled risk of in-hospital mortality. Consequently, over the last three decades, numerous researchers have attempted to demonstrate and pinpoint the precise role of non-invasive ventilation (NIV) in the specific context of ARF in onco-hematological patients. While the benefits of NIV in the management of acute pulmonary edema or alveolar hypoventilation (hypercapnic ARF) are well-demonstrated, its positioning in de novo hypoxemic ARF is debatable, and has recently been called into question. In the early 2000s, based on randomized controlled trials, NIV was recommended as first-line treatment, one reason being that it allowed significantly reduced use of orotracheal intubation. In the latest randomized studies, however, the benefits of NIV in terms of survival orotracheal intubation have not been observed; as a result, it is no longer recommended in the management of de novo hypoxemic ARF in onco-haematological patients.

[Telemonitoring of patients with chronic respiratory failure in France]. / La télésurveillance du patient insuffisant respiratoire chronique en France : l'opportunité d'organiser une prise en charge efficiente.

The year 2023 is marked by the officialization in French law of medical telemonitoring. Adult patients suffering from severe chronic respiratory failure (CRF) and being treated non-invasive ventilation (NIV) and/or oxygen therapy in a home setting are eligible for telemonitoring, of which the costs are to be covered by French health insurance. Telemonitoring allows a medical professional to remotely interpret the data necessary for follow-up and, if necessary, to make decisions regarding management of a given patient. Its objectives are, at the very least, to stabilize the disease through appropriate monitoring, to improve efficiency and quality of care, and to improve that patient's quality of life. The objective of this synthesis is to review the current state of remote monitoring of CRF patients by identifying, through a narrative analysis of the literature, its current benefits and limitations, and to compare present-day telemonitoring with the guidelines of the official French health authority (Haute Autorité de santé) for its nationwide application.

[Impact of AMARA VIEW™ interface on upper airway during nocturnal non invasive ventilation]. / Impact de l'interface AMARA VIEW™ sur les voies aériennes supérieures au cours de la ventilation non invasive nocturne.

INTRODUCTION: Nasal masks can result in mouth leaks with implications on sleep quality. To reduce these leaks, oronasal masks are proposed. It has been shown that an oronasal mask can induce obstructive events in the upper airways (UA). Because of its fit around the nose, the Amara View™ facial mask may have less consequence on UA obstruction. METHODS: This retrospective study assessed the impact of the Amara View™ mask on UA. The study was conducted on patients with chronic respiratory failure treated by home NIV with standard facial mask. Replacement of the standard facial mask by an Amara View™ mask was performed when the residual apnea hypopnea index recorded from the ventilator software exceeded 5/h. All patients underwent nocturnal respiratory polygraphy when on NIV with a standard facial mask and with an Amara View™ mask. Respiratory polygraphic traces were interpreted following the analysis methods published by the SomnoNIV Group. RESULTS: Seven patients were studied. Although nocturnal oxygen saturation was similar between both masks, the index of UA obstruction without ventilatory blunting was significantly lower during NIV with the Amara View™ mask compared to the standard facial mask (P=0.01). CONCLUSIONS: Nocturnal NIV using an Amara View™ facial interface may avoid UA obstruction without ventilatory command blunting.

[Variation in ventilatory response to CO2 in patients treated by non-invasive ventilation]. / Variation de la réponse ventilatoire au CO2 chez des patients appareillés par ventilation non invasive.

INTRODUCTION: There are many mechanisms for improving the clinical and blood gas status of patients with the obesity hypoventilation syndrome (OHS) or chronic obstructive pulmonary disease (COPD) by non-invasive ventilation (NIV) at home. Our objective was to set up a pilot study to evaluate the potential modification of the sensitivity of the respiratory centers to CO2 by NIV in paired new COPD and OHS patients. METHODS: We assessed the sensitivity of the respiratory centers to CO2 by the Read method in 3 COPD patients and 3 OHS patients newly treated by NIV and again 3 months later. We compared their results to those of 6 control subjects. RESULTS: All the patients included had altered ventilatory responses to CO2 with slopes of less of than 1 L.min-1.mmHg-1. Mean coefficients of variation were significantly higher in patients than in healthy subjects (P=0.007). Patients who improved their CO2 sensitivity slope were those most observant of NIV. CONCLUSION: This work showed significant changes in the ventilatory response to hypercapnia in patients with either OHS or COPD after NIV therapy. The significance of these changes deserves to be studied.

[Non-invasive home ventilation in patients with severe hypercapnic chronic obstructive pulmonary disease: The SOMNOVENT' study]. / Ventilation non invasive à domicile chez des patients BPCO sévères hypercapniques (étude SOMNOVENT').

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality, especially in cases of chronic hypercapnic respiratory failure. Following a prolonged debate, the indication and benefits of noninvasive ventilation (NIV) have been recently established. Although improved ventilation and reduction in hyperinflation appear to underlie the positive effect on NIV in COPD, only a few studies have focused on specific ventilatory algorithms for improving PaCO2. METHODS: The main objective of this study is to analyze the impact of Löwenstein's ventilatory algorithms, supposed to allow a better management of hyperinflation and its consequences on alveolar ventilation and blood gas parameters. This is an interventional study in routine care, prospective, single blind, randomized with cross over. The primary endpoint will be the transcutaneous partial pressure of nocturnal carbon dioxide. Secondary endpoints will be: abnormal respiratory events occurring during nocturnal NIV; the objective quality of sleep via polysomnography; the tolerance of ventilation and the subjective quality of sleep evaluated by auto questionnaires. EXPECTED RESULTS: The results of this study will clarify whether is it necessary to explore more the impact of the ventilatory modes developed by Löwenstein, dedicated to hypercapnic COPD patients, requiring a long-term NIV.

[Obesity hypoventilation syndrome and initial compliance with non-invasive ventilation]. / Syndrome obésité hypoventilation et observance initiale de la ventilation non invasive.

INTRODUCTION: The obesity hypoventilation syndrome (OHS) has an increasing prevalence. Compliance with first-line non-invasive ventilation has not been evaluated, taking into account patients' initial comorbidities. This study consisted of identification of the factors associated with compliance with non-invasive ventilation during the first six months of use. METHODS: A monocentric retrospective study, gathering patients from the pneumology department of Gabriel-Montpied hospital in Clermont-Ferrand, from April 2010 to October 2019. The analysis was carried-out through the collection of computerised medical records (age, mode of entry, patient comorbidities) and compliance reports (average daily hours of use) provided by the regional service provider for the Auvergne area (AIRRA). RESULTS: Being hospitalized for an acute exacerbation and being older than seventy-five years were factors associated with an improved compliance to non-invasive ventilation, with an increase of 1.47h/d and 2.73h/d (P value: 0.018 and 0.02, respectively). Moreover, patients with obstructive sleep apnea hypopnea syndrome and recipients of therapeutic education may prove more compliant over time. CONCLUSION: Age greater than seventy-five years and being hospitalized for an acute exacerbation are predictors of better use of non-invasive ventilation in OHS.

[Factors associated with the type of mask used during nocturnal NIV in patients with neuromuscular disorders]. / Facteurs associés au masque de ventilation non invasive nocturne chez les patients neuromusculaires adultes.

In subjects with neuromuscular diseases (NMD), the choice of facemask is essential for successful long-term noninvasive ventilation (NIV). While nasal masks usually represent the first line of treatment, almost a third of our subjects with NMD use an oro-nasal interface. Factors associated with the choice of mask remain poorly understood. We provide an original analysis of a previous prospective, multi-centric, Franco-Belgian survey investigating the factors associated with the type of nocturnal mask used in 116 adult NMD subjects treated with NIV. In these patients oro-nasal mask use was more often associated with non-Duchenne muscular dystrophy, older subjects, higher body mass index, better upper limb autonomy allowing independent mask removal and shorter periods of ventilation. Controlled prospective studies are needed to compare the efficacy and tolerance of different interfaces in this specific population.

[Long-Term Non-Invasive Ventilation for Chronic Respiratory Failure]. / Respiratorische Heimdialyse.

Long-Term Non-Invasive Ventilation for Chronic Respiratory Failure Abstract. Non-invasive long-term ventilation (NIV) may be helpful for selected patients with severe COPD and chronic respiratory lailure (chronic hypercapnia and hypoxemia). The patient described in the case report became normocapnic under long-term NIV and both her sleep and quality of life improved significantly. She also suffered fewer COPD exacerbations and was better able to cope with them herself with the help of NIV.

Outcomes of non-invasive ventilation in 'very old' patients with acute respiratory failure: a retrospective study.

BACKGROUND: Non-invasive ventilation (NIV) is increasingly used to support very old (aged ≥85 years) patients with acute respiratory failure (ARF). This retrospective observational study evaluated the impact of NIV on the prognosis of very old patients who have been admitted to the intermediate care unit (IMC) of the Emergency Department of the University Hospital Policlinico-Vittorio Emanuele of Catania for ARF. METHODS: All patients admitted to the IMC between January and December 2015 who received NIV as the treatment for respiratory failure were included in this study. Outcomes of patients aged ≥85 years were compared with lower ages. The expected intrahospital mortality was calculated through the Simplified Acute Physiology Score (SAPS) II and compared with the observed mortality. RESULTS: The mean age was 87.9±2.9 years; the M:F ratio was approximately 1:3. The average SAPS II was 50.1±13.7. The NIV failure rate was 21.7%. The mortality in the very old group was not statistically different from the younger group (20% vs 25.6%; d=5.6%; 95% CI -8% to 19%; p=0.404). The observed mortality was significantly lower than the expected mortality in both the group ≥85 (20.0% vs 43.4%, difference=23.4%; 95% CI 5.6% to 41.1%, p=0.006) and the younger group (25.6% vs 38.5%, difference=12.9%; 95% CI -0.03% to 25.8%, p=0.046). In both age groups, patients treated with NIV for chronic obstructive pulmonary disease had lower mortalities than those treated for other illnesses, although this was statistically significant only in the younger group. CONCLUSION: In very old patients, when used with correct indications, NIV was associated with mortality similar to younger patients. Patients receiving NIV had lower than expected mortality in all age groups.

Combined spinal-epidural anesthesia with non-invasive ventilation during cesarean delivery of a woman with a recent diagnosis of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis is the most common neurodegenerative upper and lower motor neuron disease in adults but is not common in women of child-bearing age. We present a case of a pregnant woman who was diagnosed with amyotrophic lateral sclerosis and developed respiratory distress at 32 weeks-of-gestation. She underwent a cesarean delivery under combined spinal-epidural anesthesia with non-invasive ventilation. This resulted in a successful outcome for both the mother and the baby.

[Home non-invasive ventilation for chronic obstructive pulmonary disease]. / Ventilation non invasive à domicile au cours de la broncho-pneumopathie chronique obstructive.

INTRODUCTION: The benefits of long-term non-invasive ventilation (NIV) in the management of chronic obstructive pulmonary disease (COPD) patients remain controversial. AIM: To analyze the characteristics of COPD patients under home NIV and to evaluate its impact among this population. METHODS: We carried out a retrospective study between January 2002 and April 2016 of COPD patients under long-term NIV at "la Rabta" and the Military Hospital. RESULTS: There were 27 patients with an average age of 64 and a sex ratio (M/F) of 0.92. Active smoking was reported in 96.3%. A persistent hypercapnia following an acute exacerbation of COPD with failure to wean the NIV was the main indication of long-term NIV. We noted a reduction in hospital admissions in the first year of 60% and in intensive care of 83.3% (P<10-3). There was no non-significant decrease of PaCO2 (4.5mmHg). There was no modification in FEV 1 and in FVC (P>0.05). The survival rate was 96.3% at 1 year, 83.3% at 2 years and a median survival of 24 months. CONCLUSIONS: Our study suggests that home NIV contributes to the stabilization of some COPD patients by reducing the hospitalizations rates for exacerbation. More prospective studies are needed to better assess the impact of NIV on survival and quality of life and to better define the COPD patients who require NIV.

[Neurofibromatosis as a cause of breathlessness]. / La neurofibromatose rend souvent essoufflé.

INTRODUCTION: Type 1 neurofibromatosis, also called "Recklinghausen's disease" is among the most frequent autosomal dominant genetic disorders, with an incidence of 1:3500 births. It mainly affects the skin and peripheral nervous system. However, in its less frequent manifestations, are tumors such as meningocele and skeletal dysplasias leading to severe clinical presentation. CASE REPORT: We report the case of a 55-year-old patient with type 1 neurofibromatosis and dyspnea due to a large left thoracic meningocele combined with a significant kyphoscoliosis, causing a severe restrictive ventilatory defect, complicated by chronic respiratory failure and pulmonary hypertension. Symptomatic treatment with non-invasive ventilation permitted an improvement of the clinical situation. CONCLUSIONS: Our observation shows the complexity of the therapeutic support of the neurofibromatosis of type 1. The contribution of non-invasive ventilation was illustrated by the arterial blood gas and clinical improvements as well as improved quality of life, with an acceptable level of inconvenience to the patient.

Ultrasound evaluation of the respiratory changes of the inferior vena cava and axillary vein diameter at rest and during positive pressure ventilation in spontaneously breathing healthy volunteers.

INTRODUCTION: Ultrasound assessment of the inferior vena cava (IVC) has gained favour in aiding fluid management decisions for controlled, mechanically ventilated patients as well as in non-mechanically ventilated, spontaneously breathing patients. Its utility in spontaneously breathing patients during positive pressure non-invasive ventilation has not yet been determined. The use of the axillary vein, as an alternative option to the IVC due to its ease of accessibility and independence from intra-abdominal pressure, has also not been evaluated. The aim of this study was to assess respiratory variation in IVC and axillary vein diameters in spontaneously breathing participants (Collapsibility Index) and with the application of increasing positive end-expiratory pressure (PEEP) via positive pressure non-invasive ventilation (Distensibility Index). METHODS: The IVC and axillary vein diameters of 28 healthy adult volunteers were measured, using ultrasound, at baseline and with increasing PEEP via non-invasive ventilation. The Collapsibility Index and Distensibility Index of these vessels were calculated and compared for each vessel. The association between increasing PEEP levels and the indices was evaluated. RESULTS: Positive pressure delivered via non-invasive ventilation produced a similar degree of diameter change in the IVC and the axillary vein, that is, the Distensibility Index was similar whether measured in the IVC or the axillary vein (P=0.21, 0.47 and 0.17 at baseline, 5 and 10 cmH2O PEEP, respectively). Individual study participants' IVC and axillary veins, however, had variable responses to PEEP; that is, there appeared to be no consistent relationship between PEEP and the diameter changes. CONCLUSION: While the axillary vein could potentially be used as an alternative vessel to the IVC to assess for volume responsiveness in controlled, mechanically ventilated patients as well as in non-mechanically ventilated, spontaneously breathing patients, neither vein should be used to guide fluid management decisions in spontaneously breathing patients during positive pressure non-invasive ventilation.

[Cohort of patients initiated to home ventilation. Observational and prospective study]. / Cohorte de patients nouvellement traités par ventilation non invasive à domicile. Étude observationnelle, prospective et multicentrique.

Cohort of patients initiated to home ventilation. Observational and prospective study. The effectiveness of home noninvasive ventilation (NIV) for chronic respiratory failure (CRF) is well established. However, few data are available about home NIV prescription and utilization according to the different etiologies of respiratory failure. The ANTADIR Federation, in partnership with the Ventilatory Support Group of the French Speaking Pulmonary Society, has set up a national, observational and multicenter cohort study. The main goal of this study is to analyze the clinical data justifying home NIV prescription in patients with chronic respiratory insufficiency. The secondary objectives will be to assess: the evolution of comorbidities or their occurrence, hospitalizations, NIV compliance, dropout and survival. The population includes patients with chronic respiratory failure newly initiated onto NIV, both in a stable state and following an acute exacerbation who qualify for long-term NIV. Data collected include: diagnosis and comorbidities, age, sex, BMI, biomarkers (hematocrit, arterial blood gases, total CO2) and functional data (FEV1, VC, TLC), nocturnal results (SaO2, PtcCO2), type of ventilator used, ventilator parameters and mask type. Follow-up data will be collected at 4 months, 1 year and 2 years and will include: hospitalizations, changes in prescription, adherence, dropouts and deaths. This work will make it possible to obtain new scientific information on long-term NIV use in France.

[Update on the respiratory management of patients with chronic neuromuscular disease]. / Mise au point dans la prise en charge respiratoire des maladies neuromusculaires chroniques.

BACKGROUND: Neuromuscular diseases include a wide range of conditions that may involve potentially life-threatening respiratory complications (infection, respiratory failure). SURVEILLANCE AND PULMONARY FUNCTION TESTS: For patients with neuromuscular diseases, clinical assessment of respiratory function and regular pulmonary function tests are needed to screen for nocturnal respiratory disorders, weakness of the diaphragm and potential restrictive disorders and/or chronic hypercapnic respiratory insufficiency, possibly with couch deficiency. MANAGEMENT OF NOCTURNAL RESPIRATORY DISORDERS AND CHRONIC RESPIRATORY FAILURE: Nocturnal respiratory assistance is an important phase of care for nocturnal respiratory disorders and chronic respiratory failure. This may involve continuous positive airway pressure, adaptative servo-ventilation or non-invasive ventilation with a facial or nasal mask. As needed, diurnal assistance may be proposed by mouthpiece ventilation. Should non-invasive ventilation prove insufficient, or if significant swallowing disorders or recurrent bronchial obstruction develop, or in case of prolonged intubation, tracheotomy may be required. LOWER AIRWAY OBSTRUCTION: In case of lower airway infection with ineffective cough, physical therapy, associated with air stacking, intermittent positive pressure breathing or mechanical in-exsufflation may be proposed. PATIENT-CENTERED MANAGEMENT: Care for swallowing disorders, nutritional counseling (cachexia, obesity), vaccinations and therapeutic education are integral elements of patient-centered management aiming to prevent the negative impact of infection and to manage respiratory failure of chronic neuromuscular disease.