Background: It has been shown that home mechanical ventilation improves quality of life, but it has not been widely studied which particular patient groups benefit the most from starting this type of therapy. The purpose of this prospective observational study was to evaluate quality of life change patterns 6 months after initiation of home mechanical ventilation in patients suffering from chronic respiratory failure using patient reported outcomes. Methods: We enrolled 74 chronic respiratory failure patients starting invasive or noninvasive home mechanical ventilation through the Semmelweis University Home Mechanical Ventilation Program. Quality of life was evaluated at baseline and at 6 months after initiation of home mechanical ventilation using the Severe Respiratory Insufficiency Questionnaire. Results: Overall quality of life showed 10.5% improvement 6 months after initiation of home mechanical ventilation (p < 0.001). The greatest improvement was observed in Respiratory complaint (20.4%, p = 0.015), Sleep and attendant symptoms (19.3%, p < 0.001), and Anxiety related subscales (14.4%, p < 0.001). Interface (invasive versus noninvasive ventilation) was not associated with improvement in quality of life (p = 0.660). Severely impaired patients showed the greatest improvement (CC =-0.328, p < 0.001). Initial diagnosis contributed to the observed change (p = 0.025), with chronic obstructive pulmonary disease and obesity hypoventilation syndrome patients showing the greatest improvement, while amyotrophic lateral sclerosis patients showed no improvement in quality of life. We found that patients who were started on long term ventilation in an acute setting, required oxygen supplementation and had low baseline quality of life, showed the most improvement during the six-month study period. Conclusions: Our study highlights the profound effect of home mechanical ventilation on quality of life in chronic respiratory failure patients that is indifferent of ventilation interface but is dependent on initial diagnosis and some baseline characteristics, like acute initiation, oxygen supplementation need and baseline quality of life. Trial registration: This study was approved by and registered at the ethics committee of Semmelweis University (SE TUKEB 251/2017; 20th of December, 2017).

BackgroundHome mechanical ventilation is an established treatment for chronic respiratory failure resulting in improved survival and quality of life. Technological advancement, evolving health care reimbursement systems and newly implemented national guidelines result in increased utilization worldwide. Prevalence shows great geographical variations and data on East-Central European practice has been scarce to date. The aim of the current study was to evaluate prevalence and characteristics of home mechanical ventilation in Hungary.MethodsWe conducted a nationwide study using an online survey focusing on patients receiving ventilatory support at home. The survey focused on characterization of the site (affiliation, type), experience with home mechanical ventilation, number of patients treated, indication for home mechanical ventilation (disease type), description of home mechanical ventilation (invasive/noninvasive, ventilation hours, duration of ventilation) and description of the care provided (type of follow up visits, hospitalization need, reimbursement).ResultsOur survey uncovered a total of 384 patients amounting to a prevalence of 3.9/100,000 in Hungary. 10.4% of patients received invasive, while 89.6% received noninvasive ventilation. The most frequent diagnosis was central hypopnea syndromes (60%), while pulmonary (20%), neuromuscular (11%) and chest wall disorders (7%) were less frequent indications. Daily ventilation need was less than 8 h in 74.2%, between 8 and 16 h in 15.4% and more than 16 h in 10.4% of patients reported. When comparing sites with a limited (< 50 patients) versus substantial (> 50 patients) case number, we found the former had significantly higher ratio of neuromuscular conditions, were more likely to ventilate invasively, with more than 16 h/day ventilation need and were more likely to provide home visits and readmit patients (p < 0,001).ConclusionsOur results show a reasonable current estimate and characterization of home mechanical ventilation practice in Hungary. Although a growing practice can be assumed, current prevalence is still markedly reduced compared to international data reported, the duality of current data hinting to a possible gap in diagnosis and care for more dependent patients. This points to the importance of establishing home mechanical ventilation centers, where increased experience will enable state of the art care to more dependent patients as well, increasing overall prevalence.Electronic supplementary materialThe online version of this article (10.1186/s12890-018-0754-x) contains supplementary material, which is available to authorized users.

Background: Home mechanical ventilation is a reliable treatment for patients suffering from chronic respiratory failure, improving survival and quality of life. Prevalence has been increasing worldwide as a result of evolving technical possibilities, telemedicine and improving national guidelines. Projects to establish a national guideline and registry for patients treated with home mechanical ventilation are currently under way in Hungary and our aim was to validate a quality of life questionnaire suited for evaluation and follow up in this specific patient group. The Severe Respiratory Insufficiency Questionnaire (SRI) is a quality of life tool designed to evaluate patients receiving home mechanical ventilation and has been validated both in patient groups receiving invasive and noninvasive ventilation. Methods: The Hungarian version of the SRI was created using the translation-backtranslation method, which was then tested for validity, viability and reliability in a cohort involving patients from three centers, receiving long-term home mechanical ventilation for chronic respiratory failure through an invasive or noninvasive interface. Patient data was collected (demographic data, lung function test, arterial blood gas, ventilation settings) and quality of life was measured with the previously validated SF-36 and newly created Hungarian SRI Questionnaires at two time points. Results: One hundred four patients receiving home mechanical ventilation were enrolled. The time to complete the SRI Questionnaire was 8.6 (±3.1) minutes, 69.2% questionnaires were self-administered. Exploratory factor analysis explained 73.8% of the variance of the questionnaire, but resulted in 13 scales. We found correlations between the SRI subscale scores to corresponding scales of the previously validated general quality of life survey SF-36. The Cronbach alpha coefficient was 0.928 for the Summary Scale of the SRI Questionnaire, proving high internal consistency. Reproducibility was high for most scales, resulting in a high overall correlation for the summary score (0.877, p < 0.001). Conclusions: The Hungarian version of the SRI Questionnaire is a viable, valid, reliable and reproduceable quality of life tool applicable for patients treated with home mechanical ventilation.

BackgroundPregnancy in patients with pulmonary hypertension is associated with increased risk of maternal and fetal death. Physiological changes during pregnancy, labor and the postpartum period may all lead to acute decompensation of chronic right heart failure with rapid progression to circulatory collapse. As such, guidelines discourage planned pregnancies in women suffering from pulmonary hypertension. There are, however, rare cases of pulmonary hypertension which have previously been undiagnosed and only become apparent during late stage pregnancy. These individuals require close monitoring and multidisciplinary management.Case presentationWe describe the case of a 34-year-old female who presented with acute decompensation of previously undiagnosed pulmonary hypertension during the 30th week of her second pregnancy. Echocardiography and CT scan confirmed severe pulmonary hypertension and right heart failure with no new thromboembolic component. Following stabilization of cardiorespiratory parameters with high FiO2 noninvasive ventilation, intravenous epoprostenol and levosimendan treatment, Cesarean section was performed under epidural anesthesia. Close monitoring was continued in the postoperative period and cardiovascular parameters were managed with ongoing inotropic and escalating vasodilator therapy. The findings were consistent with chronic thromboembolic pulmonary hypertension. Persistent hypoxia was found to be a result of right bronchial obstruction caused by blood clots, which resolved with bronchoscopic intervention. Ongoing postpartum management resulted in improved cardiovascular parameters and oxygenation. Epoprostenol treatment was successfully converted to subcutaneous treprostinil therapy and the patient was discharged home to care for her healthy baby girl. Optimal timing of pulmonary endarterectomy will be chosen based upon functional status and patient preference.ConclusionsThe case described is the first published report of previously undiagnosed pulmonary hypertension presenting with acute right heart failure in late pregnancy successfully managed by pharmacological therapy, noninvasive ventilation and a Cesarean performed under epidural anesthesia. The case illustrates that despite the challenges, acutely discovered right heart failure can be successfully managed with a comprehensive multidisciplinary treatment plan.

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