Prolonged Respiratory Failure Research Articles

Defining optimal left ventricular assist device short-term outcomes may provide insight into programmatic quality assessment

BackgroundPatients have substantial variability in perioperative outcomes after left ventricular assist device (LVAD) implant. A perioperative multidimensional tool integrating mortality, adverse events (AEs), and patient-reported outcomes to assist in quality improvement initiatives is needed. MethodsPatients undergoing HeartMate 3 LVAD implant (1/1/2017 and 1/31/2024) in the Society of Thoracic Surgeons’ Intermacs registry were studied. A Cox proportional hazard multivariable analysis incorporating AEs as time-varying covariates for mortality out to 180 days was used to generate the Intermacs Short-Term Composite Quality (INSITE) score, reflecting the adjusted hazard ratio (HR) for mortality contributed by each AE, applying global ranking methodology. In those alive and on support at 6 months, multivariable logistic regression (odds ratio, OR) was used to examine the impact of AEs on health-related quality of life (QOL) at 180 days, captured through the INSITE-QOL score. Failure to achieve ≥1 point increase in visual analog scale (VAS) from baseline was the event in the QOL analysis. ResultsOf 13,148 patients, 4,389 (33.4%) suffered at least one AE or death through 180 days. Stroke (survival: HR 13.1; QOL: HR 1.7), dialysis (survival: HR 31.4; QOL: HR 4.2), prolonged respiratory failure (survival: HR 5.7; QOL: HR 2.3), reoperation (Survival: HR 3.4; QOL: HR 1.6) and right heart failure (survival: 5.0; QOL: HR 1.4), contributed to both mortality and failure to improve QOL at 180 days (all p<0.05). The median INSITE and INSITE-QOL scores were 0.0 [0.0,1.6] and 0.0 [0.0,0.0], respectively. At 9.4% (n=17) of centers, a high INSITE score (≥13) was present in 15% of patients while the top 25% of centers had perfect INSITE-QOL scores in at least 75% of patients. ConclusionsAEs after LVAD confer differential impact on mortality and QOL, enabling development of global rank outcome scores. Given the high mortality hazard conferred by 180-day AEs, center-specific quality interventions aimed at reducing early complications provide the greatest opportunity to improve long-term survival and QOL.

Impact of preexisting interstitial lung disease on outcomes of lung cancer surgery: A monocentric retrospective study

IntroductionInterstitial lung disease (ILD) is a known risk factor for lung cancer (LC). However, the surgical risk of LC in patients with ILD remains unclear. Therefore, we conducted a single-center retrospective study to assess clinical features and outcomes of LC population who underwent surgery with or without ILD. MethodsPatients who underwent surgery for LC between January 2006 and June 2023 in our center were assessed using data extracted from the nationwide EPITHOR thoracic surgery database. Suspicion of ILD was based on patients’ records. Confirmation of ILD was then made on the patient's medical and radiological history. Patients were classified according to the pattern of ILD. The study aimed to describe the outcomes after lung cancer resection in patients with confirmed LC-ILD group compared to those without ILD (LC-non-ILD): post-operative complications, disease-free survival (DFS) and overall survival (OS). A subgroup analysis was also performed on patients with idiopathic pulmonary fibrosis and lung cancer (LC-IPF). Results4073 patients underwent surgery for LC at Assistance Publique des Hôpitaux de Marseille between January 2006 and June 2023. Of these, 4030 were in the LC-non-ILD group and 30 were LC-ILD patients. In the LC-ILD group, the predominant CT scan pattern was probable UIP (50 %). OS was not significantly lower in the LC-ILD group (45 months versus 84 months, p = 0.068). Dyspnea and tumor size were identified as potential univariate predictors of OS. No significant differences were observed on post-operative complications or their severity. The most common post-operative complications in the LC-ILD group were prolonged air leak, respiratory failure, or pneumonia. 13 patients had cancer recurrence in the LC-ILD group. ConclusionOur study provides a comprehensive analysis of a LC-ILD population features and outcome when undergoing surgery for LC. Patients with LC-ILD appeared to have a reduced OS compared with LC-non-ILD. Further investigations with larger prospective studies could be useful to confirm and develop these preliminary findings.

SCN8A Encephalopathy with a Significant Long-Term Response to Lacosamide

AbstractDevelopmental and epileptic encephalopathy associated with SCN8A variants (i.e., SCN8A encephalopathy) causes early-onset epilepsy, involuntary movements, hypotonia, and developmental delay. Sodium channel blockers are effective for treating SCN8A encephalopathy; however, the long-term effects are unknown. Herein, we report the long-term efficacy of lacosamide (LCM) treatment in a patient with SCN8A encephalopathy. Our patient, a 7-year-old girl, presented with a hyperekplexia-like excessive startle response, drug-resistant epilepsy with sinus arrest, and prolonged respiratory failure during the neonatal period. The patient was diagnosed with SCN8A encephalopathy caused by a de novo pathogenic variant of SCN8A: c.3979A &gt; G; p.Ile1327Val. The patient experienced tonic clustered seizures daily, with dramatic responses to high doses of LCM, lasting approximately 3 years.

Machine learning prediction of hospitalization costs for coronary artery bypass grafting operations

BackgroundWith the steady rise in health care expenditures, the examination of factors that may influence the costs of care has garnered much attention. Although machine learning models have previously been applied in health economics, their application within cardiac surgery remains limited. We evaluated several machine learning algorithms to model hospitalization costs for coronary artery bypass grafting. MethodsAll adult hospitalizations for isolated coronary artery bypass grafting were identified in the 2016 to 2020 Nationwide Readmissions Database. Machine learning models were trained to predict expenditures and compared with traditional linear regression. Given the significance of postoperative length of stay, we additionally developed models excluding postoperative length of stay to uncover other drivers of costs. To facilitate comparison, machine learning classification models were also trained to predict patients in the highest decile of costs. Significant factors associated with high cost were identified using SHapley Additive exPlanations beeswarm plots. ResultsAmong 444,740 hospitalizations included for analysis, the median cost of hospitalization in coronary artery bypass grafting patients was $43,103. eXtreme Gradient Boosting most accurately predicted hospitalization costs, with R2 = 0.519 over the validation set. The top predictive features in the eXtreme Gradient Boosting model included elective procedure status, prolonged mechanical ventilation, new-onset respiratory failure or myocardial infarction, and postoperative length of stay. After removing postoperative length of stay, eXtreme Gradient Boosting remained the most accurate model (R2 = 0.38). Prolonged ventilation, respiratory failure, and elective status remained important predictive parameters. ConclusionMachine learning models appear to accurately model total hospitalization costs for coronary artery bypass grafting. Future work is warranted to uncover other drivers of costs and improve the value of care in cardiac surgery.

Catastrophic complications following microvascular free tissue transfer: A 10-year review of NSQIP data

IntroductionThere is an absence of literature regarding the risks of catastrophic medical outcomes (CMOs) such as stroke, cardiac arrest, and pulmonary embolism in microvascular free tissue transfer. This study aims to determine the CMO and mortality rates, as well as risk factors, associated with microvascular reconstruction of the head and neck, extremity, and breast. MethodsThis study uses data from the American College of Surgeons National Surgical Quality Improvement Program. Cases of microvascular free tissue transfer from 2012 to 2021 were analyzed to assess the 30-day rates of CMOs, including death, as well as associated risk factors. ResultsOf the 22,839 included patients, 785 (3.44%) experienced 1043 CMOs, including 99 (0.43%) deaths. Pulmonary complications of prolonged respiratory failure and pulmonary embolism were the most common. Independent risk factors included age, male sex, underweight status, longer operation times, American Society of Anesthesiologists (ASA) class of III or above, wound classification other than clean, and underlying conditions such as diabetes, hypertension, chronic obstructive pulmonary disorder, dyspnea, metastatic cancer, and ventilator dependence. CMOs were associated with an average 10-day delay in hospital discharge. Multivariate regression analysis revealed that head and neck reconstructions were associated with increased risk of CMO (OR 4.96; p < 0.0001). ConclusionThis is the largest study to examine CMOs following microvascular free tissue transfer. Compared to previous literature spanning the period between 2006 and 2011, we observed a decreased rate of CMOs but a slight increase in 30-day mortality. Our data provide updated and comprehensive criteria for risk stratification and patient counseling. The modifiable risk factors reported in our study should be considered in elective, non-urgent cases of microvascular reconstruction.

Machine learning links unresolving secondary pneumonia to mortality in patients with severe pneumonia, including COVID-19.

BACKGROUNDDespite guidelines promoting the prevention and aggressive treatment of ventilator-associated pneumonia (VAP), the importance of VAP as a driver of outcomes in mechanically ventilated patients, including patients with severe COVID-19, remains unclear. We aimed to determine the contribution of unsuccessful treatment of VAP to mortality for patients with severe pneumonia.METHODSWe performed a single-center, prospective cohort study of 585 mechanically ventilated patients with severe pneumonia and respiratory failure, 190 of whom had COVID-19, who underwent at least 1 bronchoalveolar lavage. A panel of intensive care unit (ICU) physicians adjudicated the pneumonia episodes and endpoints on the basis of clinical and microbiological data. Given the relatively long ICU length of stay (LOS) among patients with COVID-19, we developed a machine-learning approach called CarpeDiem, which grouped similar ICU patient-days into clinical states based on electronic health record data.RESULTSCarpeDiem revealed that the long ICU LOS among patients with COVID-19 was attributable to long stays in clinical states characterized primarily by respiratory failure. While VAP was not associated with mortality overall, the mortality rate was higher for patients with 1 episode of unsuccessfully treated VAP compared with those with successfully treated VAP (76.4% versus 17.6%, P < 0.001). For all patients, including those with COVID-19, CarpeDiem demonstrated that unresolving VAP was associated with a transitions to clinical states associated with higher mortality.CONCLUSIONSUnsuccessful treatment of VAP is associated with higher mortality. The relatively long LOS for patients with COVID-19 was primarily due to prolonged respiratory failure, placing them at higher risk of VAP.FUNDINGNational Institute of Allergy and Infectious Diseases (NIAID), NIH grant U19AI135964; National Heart, Lung, and Blood Institute (NHLBI), NIH grants R01HL147575, R01HL149883, R01HL153122, R01HL153312, R01HL154686, R01HL158139, P01HL071643, and P01HL154998; National Heart, Lung, and Blood Institute (NHLBI), NIH training grants T32HL076139 and F32HL162377; National Institute on Aging (NIA), NIH grants K99AG068544, R21AG075423, and P01AG049665; National Library of Medicine (NLM), NIH grant R01LM013337; National Center for Advancing Translational Sciences (NCATS), NIH grant U01TR003528; Veterans Affairs grant I01CX001777; Chicago Biomedical Consortium grant; Northwestern University Dixon Translational Science Award; Simpson Querrey Lung Institute for Translational Science (SQLIFTS); Canning Thoracic Institute of Northwestern Medicine.

Investigation of the frequency and risk factors of pulmonary complications following cardiac surgery in the hospital

Objective: The purpose of this study was to determine the frequency of pulmonary complications after heart surgery and to assess the pre-operative, intra-operative and post-operative risk factors. Methods: This was a descriptive and analytical study that was conducted on 244 patients who underwent various cardiac surgeries from December 2021 to May 2022 in Shefa Kerman Hospital. This descriptive/analytical study on patients undergoing heart surgery at Shafa Hospital, Kerman, Iran, from December 2021 to May 2022. Pulmonary complications included atelectasis pneumonia, pleural effusion, long-term mechanical ventilation, and respiratory failure. The pre-operative, intra-operative and post-operative risk factors were investigated in the present study. SPSS software was used to analyze the data. Logistic regression analysis was employed to determine the relationship between risk factors and the incidence of lung complications. Results: In the present study, 55 patients (22.5%) had pulmonary complications, followed by prolonged mechanical ventilation (13.1%), pneumonia (11.5%), respiratory failure (4.1%), pleural effusion (11.1%) and atelectasis (6.1%). Pulmonary complications after heart surgery were associated with days of ICU stay, post-operative stroke, post-operative acute kidney injury, emergency surgery, FFP injection, high drainage rate, and mitral valve replacement surgery (all p &lt;0.001). 4% of patients died after surgery, which was statistically related to pulmonary complications (P&lt;0.0001). The results of multivariable logistic regression test showed that FFP injection, type of heart surgery, hypertension, ICU stay days are capable of predicting pulmonary complications after heart surgery. Conclusion: Mortality was found to be higher in patients with pulmonary complications. Based on the results of the regression analysis, FFP injection, type of surgery, history of hypertension and length of ICU stay were independent risk factors of pulmonary complications.

Self-Regulating Adaptive Controller for Oxygen Support to Severe Respiratory Distress Patients and Human Respiratory System Modeling

Uncontrolled breathing is the most critical and challenging situation for a healthcare person to patients. It may be due to simple cough/cold/critical disease to severe respiratory infection of the patients and resulting directly impacts the lungs and damages the alveoli which leads to shortness of breath and also impairs the oxygen exchange. The prolonged respiratory failure in such patients may cause death. In this condition, supportive care of the patients by medicine and a controlled oxygen supply is only the emergency treatment. In this paper, as a part of emergency support, the intelligent set-point modulated fuzzy PI-based model reference adaptive controller (SFPIMRAC) is delineated to control the oxygen supply to uncomforted breathing or respiratory infected patients. The effectiveness of the model reference adaptive controller (MRAC) is enhanced by assimilating the worthiness of fuzzy-based tuning and set-point modulation strategies. Since then, different conventional and intelligent controllers have attempted to regulate the supply of oxygen to respiratory distress patients. To overcome the limitations of previous techniques, researchers created the set-point modulated fuzzy PI-based model reference adaptive controller, which can react instantly to changes in oxygen demand in patients. Nonlinear mathematical formulations of the respiratory system and the exchange of oxygen with time delay are modeled and simulated for study. The efficacy of the proposed SFPIMRAC is tested, with transport delay and set-point variations in the devised respiratory model.

Spontaneous pneumomediastinum: a surrogate of P-SILI in critically ill COVID-19 patients

Spontaneous pneumomediastinum (SP) has been described early during the COVID-19 pandemic in large series of patients with severe pneumonia, but most patients were receiving invasive mechanical ventilation (IMV) at the time of SP diagnosis. In this retrospective multicenter observational study, we aimed at describing the prevalence and outcomes of SP during severe COVID-19 with pneumonia before any IMV, to rule out mechanisms induced by IMV in the development of pneumomediastinum.Among 549 patients, 21 patients (4%) developed a SP while receiving non-invasive respiratory support, after a median of 6 days [4–12] from ICU admission. The proportion of patients requiring IMV was similar. However, the time to tracheal intubation was longer in patients with SP (6 days [5–13] vs. 2 days [1–4]; P = 0.00002), with a higher first-line use of non-invasive ventilation (n = 11; 52% vs. n = 150; 28%; P = 0.02). The 21 patients who developed a SP had persisting signs of severe lung disease and respiratory failure with lower ROX index between ICU admission and occurrence of SP (3.94 [3.15–5.55] at admission vs. 3.25 [2.73–4.02] the day preceding SP; P = 0.1), which may underline potential indirect signals of Patient-self inflicted lung injury (P-SILI).In this series of critically ill COVID-19 patients, the prevalence of SP without IMV was not uncommon, affecting 4% of patients. They received more often vasopressors and had a longer ICU length of stay, as compared with their counterparts. One pathophysiological mechanism may potentially be carried out by P-SILI related to a prolonged respiratory failure, as underlined by a delayed use of IMV and the evolution of the ROX index between ICU admission and the day preceding SP.

BALANCING GVHD IMMUNOSUPPRESSION AND COVID19 VIRAL REACTIVATION IN A PATIENT WITH ATYPICAL COMPLETE DIGEORGE SYNDROME

<h3>Introduction</h3> Complete DiGeorge syndrome (DGS) accounts for less than 1% of patients with DGS, with atypical DGS being even rarer. We present a patient with atypical complete DGS who developed autoimmune phenomena attributed to autologous GVHD, and subsequent prolonged respiratory failure due to COVID19 pneumonia while on immunosuppression. <h3>Case Description</h3> A 3-year old male infant of a diabetic mother with complete DGS awaiting thymic transplantation, hypoparathyroidism, hypogammaglobulinemia on IVIG, and recent asymptomatic COVID19 infection was admitted for AKI, electrolyte disarray secondary to malabsorption, and warm autoimmune hemolytic anemia. A month prior he had a persistent eczematous rash, chronic cough, recurrent diarrhea, and thinning hair. Labs revealed uptrending total IgE to 10,000 IU/ml, no eosinophilia, an oligoclonal population of gamma-delta T cells, and increased numbers of CD4+ and CD8+ lymphocytes, which were primarily CD45RO+ central memory T cells. A large portion were activated and expressed HLA-DR. Mitogen proliferation was normal. Given these findings, he was diagnosed with autologous GVHD secondary to autoreactive oligoclonal T cells, and subsequently, atypical complete DGS. Tacrolimus and methylprednisolone were started for immunosuppression. IgE and CD3+ T cells decreased on immunosuppression, but he developed hypoxemic respiratory failure with prolonged intubation due to COVID19 pneumonia. Immunosuppression was gradually weaned. IgE and CD3+ cells increased slightly, but he showed no clinical signs of GVHD and was discharged home after a 3.5 month hospital stay. <h3>Discussion</h3> This case highlights the difficult balance between immunosuppression and viral reactivation. After starting immunosuppression for treatment of GVHD, he developed severe COVID19 pneumonia.

Changes in Orders for Life-Sustaining Treatment in Patients Requiring Prolonged Mechanical Ventilation.

Background: Growing numbers of acute critical illness survivors experience chronic critical illness (CCI) marked by prolonged dependence on life support, delirium, and/or disability. There is minimal recent data on treatment limitations in CCI. Objectives: To evaluate the natural history of changes in orders for life-sustaining treatment (OLST) in patients requiring prolonged mechanical ventilation. Design: Retrospective cohort study of 410 patients who received tracheostomy in an intensive care unit for prolonged respiratory failure. Results: Three hundred twenty-four patients had one OLST throughout the admission, with no limitations on prearrest life-sustaining treatment or cardiopulmonary resuscitation. The 86 patients who underwent at least one change in OLST were older, had longer admissions, were more likely to be deceased at hospital discharge, and were more likely to have received specialty palliative care. Thirty percent of OLST changes occurred in the last week of admission. Conclusions: OLST occur infrequently and late in patients with CCI.

Prognosis and sequelae of severe COVID-19 patients after 6 months of hospital discharge: A retrospective cohort study.

We investigated the prognosis, sequelae, and related factors of severe coronavirus disease (COVID-19) patients who required invasive mechanical ventilation 6 months after discharge from the hospital. COVID-19 patients admitted to Kishiwada Tokusyukai Hospital between April 1, 2020, and May 31, 2021, and treated with an invasive mechanical ventilator were included in this study. We conducted a telephone visit 6 months after discharge to confirm survival and asked questions about sequelae. The mortality rate 6 months after discharge was 7.4%. Tracheostomy (odds ratio [OR], 0.03; 95% confidence interval [CI], 0.003-0.26), high Acute Physiology and Chronic Health Evaluation II score (16.0 [interquartile range [IQR], 11.5-17.2] vs. 11.0 [IQR, 8.0-14.0]), prolonged hospital stay (17.0 [IQR, 12.7-24.5] vs. 10.0 [IQR, 8.0-13.0]), and prolonged ventilation duration (12.5 [IQR, 10.7-20.0] vs. 8.0 [IQR, 6.0-11.0]) were associated with the risk of death. Moreover, 49% of the patients had residual disability. The most common sequelae were hoarseness, respiratory distress on exertion (31% of symptomatic patients), and muscle weakness (22%). The prone positioning therapy (OR, 5.55; 95% CI, 1.35-32.97) was associated with hoarseness, and the use of muscle relaxants (OR, infinity; 95% CI, 1.14-infinity) was a risk factor for muscle weakness. Although the mortality rate after the acute phase of COVID-19 was not high, many patients experienced sequelae. Careful treatment should be continued after the end of acute treatment for patients with prolonged respiratory failure due to COVID-19. Muscle relaxants and prone positioning therapy may cause sequelae and should be performed carefully.

Reversibility of Hypercapnia after an Acute Exacerbation of COPD

Background: After an episode of hypercapnic AECOPD, some patients show reversible, prolonged or persistent hypercapnic respiratory failure. However, at the time of patient discharge, it is uncertain whether patients will remain hypercapnic or may return to a physiologic gas status. Methods: Data were retrospectively collected from COPD patients with an acute hypercapnic exacerbation (AECOPD). Out of 143 total COPD inpatients, complete data set was available for 82 patients in stable condition. According to the first available capillary or arterial pCO₂, patients were divided into those with persistent hypercapnia (PHG) and those with reversible hypercapnia. Results: In this study, 51% of patients with acute hypercapnic AECOPD and follow-up (FUP) visits developed normocapnia after a time period of several weeks. These patients were characterized by lower carbon dioxide partial pressure (PaCO₂), HCO₃⁻, and BE levels prior to the AECOPD event, at discharge and at FUP. pH was higher at discharge and FUP in this group. Greater disease severity and lower forced vital capacity were prominent in patients with PHG. Binary logistic regression revealed GOLD D and higher PaCO₂ at discharge as predicting factors for PHG. Conclusions: A large percentage of patients has prolonged hypercapnia following acute hypercapnic COPD exacerbation. The risk profile of patients with irreversible hypercapnia should be carefully evaluated following AECOPD in order to choose selected patients for home-noninvasive ventilation.

Transbronchial Lung Cryobiopsy in Patients with Interstitial Lung Disease: A Systematic Review.

Rationale: In 2018, a systematic review evaluating transbronchial lung cryobiopsy (TBLC) in patients with interstitial lung disease (ILD) was performed to inform American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax clinical practice guidelines on the diagnosis of idiopathic pulmonary fibrosis. Objectives: To perform a new systematic review to inform updated guidelines. Methods: Medline, Excerpta Medica Database, and the Cochrane Central Register of Controlled Trials (CCTR) were searched through June 2020. Studies that enrolled patients with ILD and reported the diagnostic yield or complication rates of TBLC were selected for inclusion. Data was extracted and then pooled across studies via meta-analysis. The quality of the evidence was appraised using the grading of recommendations, assessment, development, and evaluation approach. Results: Histopathologic diagnostic yield (number of procedures that yielded a histopathologic diagnosis divided by the total number of procedures performed) of TBLC was 80% (95% confidence interval [CI], 76-83%) in patients with ILD. TBLC was complicated by bleeding and pneumothorax in 30% (95% CI, 20-41%) and 8% (95% CI, 6-11%) of patients, respectively. Procedure-related mortality, severe bleeding, prolonged air leak, acute exacerbation, respiratory failure, and respiratory infection were rare. The quality of the evidence was very low owing to the uncontrolled study designs, lack of consecutive enrollment, and inconsistent results. Conclusions: Very low-quality evidence indicated that TBLC has a diagnostic yield of approximately 80% in patients with ILD, with manageable complications.

Defining Metrics for Short Term Success After LVAD Implant: An Analysis of the Society of Thoracic Surgeons Intermacs Registry

<h3>Purpose</h3> While clinical trials evaluating left ventricular assist device (LVAD) technology typically use composite outcomes to assess efficacy, composite outcomes including patient reported outcomes (PROs) have not been utilized as benchmarks for LVAD implant center performance improvement initiatives or quality ranking. The objective of the study was to assess the feasibility of generating a patient composite outcome measure including PROs from a real world registry. <h3>Methods</h3> Short term (ST, 180 days) adverse events (AEs) and mortality were tallied for Intermacs patients undergoing LVAD implant between 1/2012 and 12/2019. ST postoperative events included mortality on first device and frequencies of stroke, reoperation (device malfunction/other), right heart failure (RHF), prolonged respiratory failure, and/or dialysis on first device. Logistic regression was used to generate odds ratios for mortality for each AE. Separately, the EuroQOL visual analog scale (VAS) was assessed at baseline and 180 days in ST survivors. <h3>Results</h3> Of 20,115 patients, 37% suffered at least one event, most commonly death, reoperation and stroke (Table, column A). Stroke, prolonged respiratory failure, and dialysis attributed the most to ST mortality (Table, column B). Of the 16725 patients alive at 180 days, 43% completed a VAS with 82.0% showing VAS improvement. Renal failure and RHF contributed most to failure to improve VAS (Figure). <h3>Conclusion</h3> Assessment of a ST composite outcome metric after LVAD implant from a real world data source is feasible but limited by incomplete PRO reporting. ST adverse events display differential effects on mortality and PROs that can be used in development of global rank outcome scores. While reoperation is common, stroke, prolonged respiratory failure and renal failure conferred highest risks of ST deaths within Intermacs. Assessment of PROs should become a priority for LVAD centers to allow the field to generate a complete assessment of patient-centered outcomes.

The role of tracheotomy in patients with moderate to severe impairment of the lower airways

SUMMARYPatients affected by severe acute respiratory distress syndrome due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection have a high likelihood of needing prolonged intubation. As observed worldwide during the Coronavirus Disease 2019 (COVID-19) pandemic, the need for tracheotomy in patients with prolonged respiratory failure has dramatically increased. Tracheotomy in these patients offers several advantages over prolonged translaryngeal intubation: improved patient comfort may allow a reduction in intravenous administration of analgesics, sedatives and muscle relaxant drugs, enhance mobility with particular regard to respiratory muscles, and patients may achieve autonomy earlier. However, there is still debate about the optimal timing and surgical technique of tracheotomy. Similarly, debate is still open regarding the relative merits of open surgical tracheotomy (ST) versus percutaneous dilatational techniques (PDT). In general, PDT is commonly used in elective tracheotomy in adult patients in intensive care units; ST may be preferred depending upon the practitioner’s experience and patient’s characteristics. Correct timing of tracheotomy should be individualised and the indication for tracheotomy should balance the problems related to prolonged intubation and the risk of early or late complications related to the surgical procedure.

Tracheostomy Is Associated With a Decrease in Delirium and Sedation for Intubated COVID-19 Patients.

Patients with coronavirus disease 2019 (COVID-19) acute respiratory distress syndrome can experience prolonged periods of ventilation, high incidence of delirium, and require high amounts of sedation. Tracheostomy has been associated with earlier ventilator liberation, decreased sedation needs, and lower rates of delirium but optimal timing of tracheostomy remains unknown. Is tracheostomy associated with lower sedation requirements and lower incidence of delirium in patients with COVID-19 that are intubated? We retrospectively reviewed the first 32 patients at a large urban tertiary referral center that underwent tracheostomy for prolonged respiratory failure. We obtained Richmond Agitation Sedation-Scale scores and Confusion Assessment Method for Intensive Care Unit data along with amount(s) and type(s) of sedating medications given, in the 7 days before and after tracheostomy. Proportion of days delirious and sedating medications were compared in the 7 days before and after tracheostomy. There was a significant decrease in the amount of opioids and benzodiazepines in the 7-day period following tracheostomy. Opioid dosing decreased by 157.5 morphine equivalents (SD=339, P =0.01) and benzodiazepine dosing decreased by 18 mg lorazepam equivalents (SD=34, P =0.01). There was no significant difference in antipsychotic or other sedative-hyponotic drug doses. There was a significant decrease in the proportion of days of coma or delirium (mean decrease in proportion=0.16, SD=0.32, P =0.008) following tracheostomy. Tracheostomy was associated with a significant decrease amount of sedating medications and with a decrease in proportion of days delirious following tracheostomy.

Respiratory disorders in a child with multiple malformations

Concomitant congenital malformations are an actual problem in pediatrics. Association of tracheobronchial tree anomalies and congenital heart disease (CHD) could be the cause of the patient’s condition deterioration before and after cardiac surgery. We present a clinical case of a patient with multiple malformations with a long-term and poorly corrected broncho-obstructive syndrome, combined with pneumonia and hypoventilation. After surgery for tetralogy of Fallot at 3 months, the boy needed mechanical ventilation for a long time due to bilateral pneumonia complicated by bilateral pneumothorax. At 7 months, a respiratory infection required hospitalization in the intensive care unit. When pneumonia resolved, the boy was discharged home with continued oxygen support using an oxygen concentrator. The reason for the patient’s prolonged respiratory failure could be explained by prolonged hypoventilation due to narrowing of the left main bronchus and consecutive congenital pneumonia, repeated aspirations, and postoperative mechanical ventilation-associated pneumonia with bilateral pneumothorax. At the age of 1 year 3 months in the Department of Thoracic Surgery of St. Vladimir’s biodegradable stent in the left main bronchus was installed. The bronchus lumen was restored. The boy did not need oxygen support, and his physical and motor development indicators improved. Thus, in patients with tetrad of Fallot, a thorough assessment of the condition of the tracheobronchial tree is advisable in order to develop an appropriate treatment plan if needed. The collaboration of specialists in various fields of care for pediatric patients is the basis for a favorable prognosis in terms of the full growth and development of patients with multiple malformations.

Late Onset Pompe Disease with Novel Mutations and Atypical Phenotypes.

Late onset Pompe disease (LOPD) is rare and generally manifests predominantly as progressive limb girdle muscle weakness. It is linked to the pathogenic mutations in GAA gene, which leads to glycogen accumulation in various tissues. We describe the unusual clinical, biochemical, histopathological and genetic characteristics of 5 cases of LOPD. The first case had progressive anterior horn cell like disease (AHCD) that evolved later to classical limb girdle syndrome and respiratory failure, the second patient had rigid spine syndrome with gastrointestinal manifestations, the third had limb girdle weakness superimposed with episodic prolonged worsening and respiratory failure, the fourth had large fibre sensory neuropathy without primary muscle involvement and the fifth presented with classical limb girdle muscle weakness. Two homozygous missense mutations c.1461C > A (p.Phe487Leu) and c.1082C > T (p.Pro361Leu) in the GAA gene were identified in case 1 and 2 respectively. Case 3 was compound heterozygous with inframe c.1935_1940del (p.Val646_Cys647del) and an intronic splice effecting variant c.-32-13T > G. Compound heterozygous missense variants c.971C > T (p.Pro324Leu) and c.794G > A (p.Ser265Asn) were identified in case 4. Case 5 had a frameshift insertion c.1396dupG (p.Val466GlyfsTer40) and a synonymous splice affecting variant c.546G > T(p.Thr182=). We are describing for the first time from India on LOPD with unusual phenotypes identified. A high degree of clinical suspicion and diagnosing rare phenotypes of Pompe disease is imperative to consider early initiation of Enzyme Replacement Therapy (ERT).

Airway Characteristics of Patients With 22q11 Deletion Undergoing Pulmonary Artery Reconstruction Surgery: Retrospective Cohort Study.

We have previously shown that patients with a chromosome 22q11 microdeletion are at risk for prolonged respiratory failure after pulmonary artery reconstruction surgery compared with those with normal genotype. We sought to describe preexisting airway abnormalities in this patient population and examine relationships between airway abnormalities and outcomes. Single-center retrospective chart review from Society of Thoracic Surgery and Pediatric Cardiac Critical Care Consortium databases and the electronic medical record. Lucile Packard Children's Hospital at Stanford from September 2017 to February 2019. All patients undergoing pulmonary artery reconstruction surgery were considered for inclusion. We identified 127 patients meeting study inclusion criteria. Thirty-nine patients met specific criteria and underwent screening preoperative bronchoscopy including microdirect laryngoscopy and lower airway examination. Postoperative bronchoscopy was performed at the discretion of the intensive care team. Airway abnormalities were detected in 25/26 of children (96%) with a chromosome 22q11 deletion who underwent preoperative bronchoscopy. Upper and lower airway pathologies were found in 19/25 (73%) and 21/25 (81%) patients, respectively, and it was common for patients to have more than one abnormality. Presence of 22q11 deletion was associated with longer duration of mechanical ventilation (9.1 vs 4.3 d; p = 0.001), use of noninvasive positive pressure support (13 vs 6 d; p = 0.001), and longer hospital stays (30 vs 14 d; p = 0.002). These outcomes were worse when compared with patients with known airway abnormalities who did not have 22q11 deletion. Preexisting upper and lower airway pathologies are common in patients with a chromosome 22q11 deletion who undergo pulmonary artery reconstruction surgery. Despite similar postoperative hemodynamics and outcomes as their counterparts without 22q11 deletion, 22q11 deletion is associated with more postoperative respiratory complications not entirely explained by preexisting airway abnormalities.