Die Beatmung bei chronisch ventilatorischer Insuffizienz hat sich in den letzten 20 Jahren durch die Entwicklung der nicht invasiven Beatmung zu einer wichtigen Therapieform entwickelt. Sehr unterschiedliche Erkrankungen können zu einer ventilatorischen Insuffizienz führen und werden mit einer Heimbeatmung behandelt. Bei den neuromuskulären Erkrankungen liegt eine primäre Verminderung der Atemmuskelkraft vor. Mit Fortschreiten der Erkrankung entwickelt sich fast regelhaft eine ventilatorische Insuffizienz, die sich zunächst nachts manifestiert. Bei nachgewiesener Tageshyperkapnie ist bei den häufigen Erkrankungen Amyotrophe Lateralsklerose und Muskeldystrophie Duchenne die Lebenserwartung ohne Beatmungstherapie sehr beschränkt und kann durch die nicht invasive Beatmung bei guter Lebensqualität deutlich verlängert werden, solange kein ausgeprägter bulbärer Befall vorliegt. Dies gilt auch für Thoraxwanderkrankungen wie die Kyphoskoliose und das sogenannte Post-Tbc-Syndrom. Hier sind neben Verbesserungen des Gasaustausches und der Lebensqualität auch ein Anstieg der körperlichen Belastbarkeit und ein Abfall des pulmonalen Hypertonus dokumentiert. Nur wenige Daten liegen zur langfristigen Wirksamkeit beim Obesitas-Hypoventilations-Syndrom (OHS) vor, allerdings legen die retrospektiven Studien einen deutlichen Überlebensvorteil nahe. Der kleinere Teil der Patienten kann effektiv mit nCPAP behandelt werden, die meisten benötigen jedoch eine Ventilationstherapie. Die größte Gruppe bilden die Patienten mit einer schwergradigen COPD. Hier wird die Indikation zur Heimbeatmung aufgrund der Studienlage kontrovers diskutiert. Nach Analyse der Studiendaten ergibt sich ein Trend zu besseren Behandlungsergebnissen, wenn die Patienten vor Beginn der Therapie stärker hyperkapnisch sind und wenn höhere effektive Ventilationsdrücke verwendet werden. Unter diesen Voraussetzungen sind Verbesserungen der Hyperkapnie, der körperlichen Belastbarkeit, des Schlafes und der Lebensqualität dokumentiert. Eine Verringerung der Mortalität konnte bisher nicht nachgewiesen werden.
Abstract
Mechanical ventilation has become an important treatment option in chronic ventilatory failure. There are different diseases which lead to ventilatory failure and to home mechanical ventilation (HMV). A primary loss of in- and expiratory muscle strength is the reason for respiratory deterioration in neuromuscular disease. In most of these diseases ventilatory failure develops because of the progressive character of muscular damage. Initially, ventilatory failure can be found during night-time. In the case of hypercapnia at daytime, life expectancy is strongly reduced, especially in amyotrophic lateral sclerosis and Duchenne muscular dystrophy. HMV leads to a prolongation of life and to an increase in quality of life, if bulbar involvement is not severe. Impressive clinical improvements under HMV have been found in restrictive disorders of the rib cage like kyphoscoliosis or posttuberculosis sequelae, with an increase of quality of life, walking distance and a decrease in pulmonary hypertension. Only few data are published about long-term results of HMV in Obesity Hypoventilation. In terms of retrospective analyses of clinical data HMV seems to improve survival in this population. Some patients only need CPAP treatment, but most patients have to be treated with ventilatory support. The application of HMV in patients with chronic ventilatory failure due to chronic obstructive pulmonary disease (COPD) is growing, but there are controversial results in randomised clinical trials. Analysis of these data suggest better results of HMV in patients with severe hypercapnia, with the application of higher effective ventilatory pressure and a ventilator mode with a significant reduction in the work of breathing. Under such conditions HMV leads to a reduction of hypercapnia, an improvement in sleep quality, walking distance and quality of life, but until now there is no evidence in reduction of mortality in COPD.
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