Episodes Of Respiratory Failure Research Articles

October 2024 Critical Care Case of the Month: Respiratory Failure in a Patient with Ulcerative Colitis

No abstract available. Manuscript truncated after 150 words. History of Present Illness The patient is a 57-year-old woman with a history of ulcerative colitis (UC) complicated by toxic megacolon with subsequent colectomy. She presented to the emergency department with cough, shortness of breath and hypoxemia (87% on RA). PMH, SH • UC with history of toxic megacolon (4 years prior) with a total colectomy • History of a prior episode of respiratory failure a year earlier thought possibly medication induced (ustekinumab, Stelara®) which she was taking for her UC. She was treated with steroids at that time with good response. • Pyoderma gangrenosum of both ankles (attributed to UC). • Anemia of chronic disease. • She is a lifelong non-smoker. • No exposures to toxic dusts, birds, down, humidifiers, mold or other antigens associated with hypersensitivity pneumonitis. Physical Exam • Afebrile, Oxygen saturation 94% on 2 lpm supplemental oxygen. • Chest: crackles noted at left base. • CV regular rhythm, no murmur. Ext: scarring and erythema on …

Spinal Muscular Atrophy Type 1 Survival Without New Pharmacotherapies: Two Treatment Paradigms.

The aims of the study are to present noninvasive respiratory management outcomes using continuous noninvasive ventilatory support and mechanical in-exsufflation from infancy for spinal muscular atrophy type 1 and to consider bearing on new medical therapies. Noninvasive ventilatory support was begun for consecutively referred symptomatic infants with spinal muscular atrophy type 1 from 1 to 10 mos of age. Intercurrent episodes of respiratory failure were managed by intubation then extubation to continuous noninvasive ventilatory support and mechanical in-exsufflation despite failing ventilator weaning and extubation attempts. Intubations, tracheotomies, and survival were monitored. Of 153 patients with spinal muscular atrophy 1 consecutively referred since 1995, 37 became continuous noninvasive ventilatory support dependent, almost half before 10 yrs of age. Of the 37, 18 required continuous noninvasive ventilatory support for a mean 18.6 ± 3.3 yrs to a mean 25.3 (range, 18-30) yrs of age, dependent from as young as 4 mos of age with 0 to 40 ml of vital capacity. One of the 18 died from COVID-19 acute respiratory distress syndrome at age 24 after 23 yrs of continuous noninvasive ventilatory support. Extubation success rate of 85% per attempt (150/176) resulted in only one undergoing tracheotomy. Medical treatments begun during the first 6 wks of age convert spinal muscular atrophy 1 into spinal muscular atrophy 2 or 3 but cough flows remain inadequate to avoid many pneumonias that, once resolved by a treatment paradigm of extubation to continuous noninvasive ventilatory support and mechanical in-exsufflation, eliminates need to resort to tracheotomies.

Long-term Neurocognitive Morbidity After a Single Episode of Respiratory Failure in Children

Long-term Neurocognitive Morbidity After a Single Episode of Respiratory Failure in Children

Adverse Events Associated with Cardiac Catheterization in Children Supported with Ventricular Assist Devices.

Children on ventricular assist device (VAD) support can present several unique challenges, including small patient size, univentricular or biventricular congenital heart disease (1V- or 2V-CHD) and need for biventricular VAD (BiVAD) support. While cardiac catheterization can provide valuable information, it is an invasive procedure with inherent risks. We sought to evaluate the safety of catheterization in pediatric patients on VAD support. We performed a retrospective review of patients on VAD support who underwent catheterization at Lucile Packard Children's Hospital between January 1, 2014 and September 1, 2019. Using definitions adapted from Pedimacs, adverse events (AEs) after catheterization were identified, including arrhythmia; major bleeding or acute kidney injury within 24 hours; respiratory failure persisting at 24 hours; and stroke, pericardial effusion, device malfunction, bacteremia or death within 7 days. AEs were categorized as related or unrelated to catheterization. Sixty procedures were performed on 39 patients. Underlying diagnoses were dilated cardiomyopathy (48%), 1V-CHD (35%), 2V-CHD (8%), and other (8%). Devices were implantable continuous flow (72%), paracorporeal pulsatile (18%) and paracorporeal continuous flow (10%). Catheterizations were performed on patients in the ICU (60%), on inotropic support (42%), with deteriorating clinical status (37%) and on BiVAD support (12%). There were 9 AEs possibly related to catheterization including 6 episodes of respiratory failure, 2 major bleeding events, and 1 procedural arrhythmia. AE occurrence was associated with ICU status ( P = 0.01), BiVAD support ( P = 0.04) and procedural indication to evaluate worsening clinical status ( P = 0.04). Despite high medical acuity, catheterization can be performed with an acceptable AE profile in children on VAD support.

SHAPE-SENSING ROBOTIC ASSISTED BRONCHOSCOPY FOR THE DIAGNOSIS OF PERIPHERAL PULMONARY LESIONS

TOPIC: Lung Cancer TYPE: Original Investigations PURPOSE: To assess the real-world performance characteristics of a shape-sensing robotic platform for the diagnosis of peripheral pulmonary nodules. METHODS: This is a retrospective, single center, single operator cohort of consecutive cases performed utilizing shape-sensing robotic assisted bronchoscopy (SSRAB) with integrated 3.5mm robotic catheter (Ion Endoluminal System, Intuitive Surgical, Sunnyvale, CA). Cases were performed between January 2020 and April 2021. A specific anesthesia protocol was used in all patients to prevent atelectasis. Radial endobronchial ultrasound (rEBUS) was used in all cases. Cone-beam CT (CBCT, Philips Allura Xper FD20 with Lung Suite, Best, Netherlands) was used at the discretion of the physician. A procedure was considered diagnostic if there was a malignant or specific benign result. Biopsies with inflammation were considered indeterminate, followed with CT imaging and considered diagnostic if there was significant improvement or resolution of the lesion. Specimens noted to be “atypical” were considered non-diagnostic. RESULTS: A total of 230 lesions, biopsied in 192 consecutive patients were included (56% female). Multiple lesions were biopsied (maximum = 3) in a single procedure in 38 patients (20%). Bilateral lesions were biopsied in 13 patients (7%). Most lesions (64%) were in the upper lobes (n= 144, 40% RUL, 24% LUL). Median lesion size was 15mm (IQR: 10, 21). A bronchus sign was present in 38% of lesions. Lesions biopsied were solid (78%), semi-solid (12%), ground glass (7%) or cavitary (3%). Diagnostic yield was 92.2% for all lesions and 89.3% for lesions 10mm or less (n=56, smallest = 5mm). CBCT was used for 75% of lesions (n=174). Malignancy was noted in 74% (n=170) of lesions. A specific benign diagnosis was noted in 16% (n=37), and 8% (n=15) of lesions were non-diagnostic. There were 8 lesions where biopsy results were indeterminate. These patients underwent serial CT scans for follow-up: 5 lesions resolved, improved significantly or were stable for 12 months. Three lesions have follow-up pending and are considered non-diagnostic in this analysis. All indeterminate lesions had tool-in-lesion confirmation on intraprocedural CBCT scan. Sensitivity for malignancy ranges from 90.4% to 91.9% considering pending follow-up of 3 lesions. Pneumothorax was noted in 2 patients (1%), 1 of which required a chest tube (0.5%). There were no episodes of respiratory failure or significant bleeding that required intervention (including removal of the robotic catheter). CONCLUSIONS: This represents the largest cohort of patients to date using any robotic bronchoscopy technology and shows that SSRAB, when combined with CBCT and rEBUS, is safe with a high diagnostic yield. CLINICAL IMPLICATIONS: The use of SSRAB may increase the diagnostic yield for peripheral pulmonary nodules. Larger, prospective multicenter trials are forthcoming. DISCLOSURES: My spouse/partner as a Employee relationship with Medtronic Please note: >$100000 by Michael Pritchett, source=Web Response, value=Salary Consultant relationship with Medtronic Please note: $5001 - $20000 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with Johnson & Johnson Please note: $5001 - $20000 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with AstraZeneca Please note: $20001 - $100000 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with Intuitive Please note: $20001 - $100000 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with BodyVision Please note: $5001 - $20000 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with Boston Scientific Please note: $1001 - $5000 by Michael Pritchett, source=Web Response, value=Consulting fee Research relationship with Philips Please note: $5001 - $20000 by Michael Pritchett, source=Web Response, value=Grant/Research Support Consultant relationship with Biodesix Please note: $5001 - $20000 by Michael Pritchett, source=Web Response, value=Consulting fee Speaker relationship with Intuitive Please note: 2 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Speaking fees Consultant relationship with Medtronic Please note: 5 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with Intuitive Please note: 2 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Consulting fee Advisory Committee Member relationship with AstraZeneca Please note: 4 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Honoraria Advisory Committee Member relationship with Pfizer Please note: 4 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Honoraria Advisory Committee Member relationship with Biodesix Please note: 4 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Honoraria Consultant relationship with BodyVision Please note: 3 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with Philips Please note: 2 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Consulting fee Consultant relationship with Johnson & Johnson Please note: 3 years Added 06/23/2021 by Michael Pritchett, source=Web Response, value=Consulting fee No relevant relationships by Charles Schirmer, source=Web Response

HSV PNEUMONIA IN AN IMMUNOCOMPETENT PATIENT PROGRESSING TO ORGANIZING PNEUMONIA

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: HSV pneumonia has been rarely reported in immunocompetent patients. We report a case of a 75-year-old healthy female with intractable pneumonia diagnosed as HSV that progressed to organizing pneumonia CASE PRESENTATION: A 75-year-old female with a past medical history of Barrett's esophagus presented to the ER with a 3-week history of fatigue, fevers, and shortness of breath. She had no family history of autoimmune disorders, she moved from Iran 40 years ago and had no known exposure to TB. She denied smoking and had no pets or any other exposures. Labs showed leukocytosis of 14000 with neutrophilic and monocytic predominance, mild hyponatremia (132 meq/L), and a procalcitonin of 0.25 ng/ml. Chest X-ray revealed infiltrates in the right upper and lower lobes. She was started on ceftriaxone and azithromycin but continued to worsen. Antibiotics were broadened again without clinical response. Urine streptococcus, legionella antigen, B-D-glucan, Tuberculin skin test, HIV, histoplasma were all negative. Bronchoscopy was done on day 10 that revealed copious clear, yellow clear fluid in her proximal and distal airway and with similarly appearing fluid with mucus plugs on the BAL. Cultures from BAL including modified AFB for nocardia were unrevealing. Rheumatological workup including, ANA, ANCA, anti-CCP, Sjogren's antibody, myositis panel, HSP was also negative. Subsequent CT scan revealed worsening infiltrates and a right-sided pleural effusion, patient underwent thoracentesis, and cultures on pleural fluid were positive for HSV. The patient underwent a VATS biopsy (Day 22) that revealed organizing pneumonia with thickened alveoli and fibrinous plugs. HSV 1 IgG antibodies were also positive. She was started on steroids and acyclovir with significant improvement. DISCUSSION: HSV is known to infect the upper airway and can cause gingivostomatitis and pharyngitis. It can progress to pneumonitis or pneumonia in patients who are immunocompromised or mechanically ventilated. Only in rare cases does it manifest as clinically significant lower respiratory tract infections in immunocompetent individuals. Our patient had a biphasic illness, she was diagnosed based on the HSV culture on the pleural fluid, which has a specificity of 99%. Her second episode of respiratory failure with worsening infiltrates represented the development of organizing pneumonia that responded to steroids. The use of acyclovir in such patients is not supported by clinical trials and needs to be studied. Our patient finished a course of acyclovir and was treated with steroids for several weeks. CONCLUSIONS: HSV pneumonia should be considered as an etiology of intractable pneumonia when patients do not show clinical improvement on conventional therapy. This case also suggests that acute herpes infection can have serious complications like organizing pneumonia and further studies are needed to understand this association. REFERENCE #1: Mills B, Ratra A, El-Bakush A, Kambali S, Nugent K. Herpes Simplex Pneumonia in an Immunocompetent Patient With Progression to Organizing Pneumonia. J Investig Med High Impact Case Rep. 2014 Apr 9;2(2):2324709614530560. doi: 10.1177/2324709614530560. PMID: 26425602; PMCID: PMC4528890. REFERENCE #2: Martinez E, de Diego A, Paradis A, Perpiñá M, Hernandez M. Herpes simplex pneumonia in a young immunocompetent man. Eur Respir J. 1994 Jun;7(6):1185-8. PMID: 7925891. DISCLOSURES: No relevant relationships by Chahat Puri, source=Web Response No relevant relationships by Kyunghoon Rhee, source=Web Response No relevant relationships by Donald Slack, source=Web Response No relevant relationships by Jennifer Sullivan, source=Web Response No relevant relationships by Ari Zaiman, source=Web Response

PSEUDOANEURYSM OF THE ASCENDING THORACIC AORTA AS A COMPLICATION OF BILATERAL LUNG TRANSPLANT AND THE RISK OF UNDERGOING A NEW CARDIOTHORACIC SURGERY

TOPIC: Transplantation TYPE: Fellow Case Reports INTRODUCTION: Lung transplantation is an effective option for those with advanced lung disease. Acute or chronic graft failure may lead to significant morbidity and mortality. Lung retransplantation is an alternative for patients that develop allograft dysfunction. Preoperative investigation must consider complications from previous cardiothoracic procedures that may impose additional risk. We herein reported on a previously undiagnosed pseudoaneurysm of the thoracic ascending aorta (PTA) successfully treated during a bilateral lung retransplantation. CASE PRESENTATION: Patient is a 24-year-old female with history of bilateral lung transplant due to cystic fibrosis with pulmonary manifestation. She was admitted for bilateral retransplantation after progressive decline in lung function. Initial decompensation consisted of acute respiratory failure with hypoxemia secondary to Pseudomonas pneumonia, consistent with A4 pulmonary allograft rejection. She rapidly progressed to other episodes of respiratory failure, infection, dyspnea on minimal exertion, rest tachycardia, and significant decline in spirometry consistent with Bronchiolitis Obliterans Syndrome. After general anesthesia, upon intraoperative Transesophageal echocardiography, the anesthesiologist detected a previously undiagnosed PTA on the anterior surface. A redo bilateral sequential lung transplantation with PTA repair was performed under cardiopulmonary bypass (CPB) with transverse sternotomy and cannulation of right common femoral artery and vein. With the heart decompressed, the PTA, which was 2x1.5x2cm and located at the previous cannulation site, was dissected from the surrounding adhesions. The PTA was resected and repaired by partial clamping of the aorta, followed by an uneventful bilateral lung transplant. Pathology reports of the resected PTA showed fibroadipose and benign thymic tissues. Cultures were negative. The patient had an uncomplicated post-operative course and was discharged after 19 days. DISCUSSION: This is the first report of PTA as a complication of lung transplant. Postoperative PTA is rare and has been described only in cardiac procedures. It usually occurs in previous suture lines and in immunocompromised patients such as transplant recipients it is commonly related to infection in these endothelial damaged sites. It is crucial to be aware that they can be asymptomatic or misdiagnosed due to the primary clinical conditions such as lung rejection or multiple infections in the present case. In hindsight, the PTA was present in the patient's previous chest CTs and would have been easily identified if the arterial phase was performed. CONCLUSIONS: It is imperative that the multidisciplinary team involved in the care of patients undergoing lung retransplantation is vigilant about the possibility of PTA. Preoperative diagnosis can be lifesaving by avoiding rupture and exsanguination in a new cardiothoracic procedure REFERENCE #1: Glanville AR, Verleden GM, Todd JL, Benden C, Calabrese F, Gottlieb J, Hachem RR, Levine D, Meloni F, Palmer SM, Roman A, Sato M, Singer LG, Tokman S, Verleden SE, von der Thüsen J, Vos R, Snell G. Chronic lung allograft dysfunction: Definition and update of restrictive allograft syndrome-A consensus report from the Pulmonary Council of the ISHLT. J Heart Lung Transplant. 2019 May;38(5):483-492. REFERENCE #2: Behzadnia N, Ahmadi ZH, Mandegar MH, Salehi F, Sharif-Kashani B, Pourabdollah M, Ansari-Aval Z, Kianfar AA, Mirhosseini SM, Eiji M. Asymptomatic mycotic aneurysm of ascending aorta after heart transplantation: a case report. Transplant Proc. 2015 Jan-Feb;47(1):213-6. REFERENCE #3: Sullivan KL, Steiner RM, Smullens SN, Griska L, Meister SG. Pseudoaneurysm of the ascending aorta following cardiac surgery. Chest. 1988 Jan;93(1):138-43. DISCLOSURES: No relevant relationships by Thais Peclat, source=Web Response No relevant relationships by Si Pham, source=Web Response No relevant relationships by Eduardo Rodrigues, source=Web Response

COVID-19 in pediatric kidney transplantation: The Improving Renal Outcomes Collaborative

There are limited data on the impact of COVID‐19 in children with a kidney transplant (KT). We conducted a prospective cohort study through the Improving Renal Outcomes Collaborative (IROC) to collect clinical outcome data about COVID‐19 in pediatric KT patients. Twenty‐two IROC centers that care for 2732 patients submitted testing and outcomes data for 281 patients tested for SARS‐CoV‐2 by PCR. Testing indications included symptoms and/or potential exposures to COVID‐19 (N = 134, 47.7%) and/or testing per hospital policy (N = 154, 54.8%). Overall, 24 (8.5%) patients tested positive, of which 15 (63%) were symptomatic. Of the COVID‐19‐positive patients, 16 were managed as outpatients, six received non‐ICU inpatient care and two were admitted to the ICU. There were no episodes of respiratory failure, allograft loss, or death associated with COVID‐19. To estimate incidence, subanalysis was performed for 13 centers that care for 1686 patients that submitted all negative and positive COVID‐19 results. Of the 229 tested patients at these 13 centers, 10 (5 asymptomatic) patients tested positive, yielding an overall incidence of 0.6% and an incidence among tested patients of 4.4%. Pediatric KT patients in the United States had a low estimated incidence of COVID‐19 disease and excellent short‐term outcomes.

Diagnostic Yield of Digital Tomosynthesis-assisted Navigational Bronchoscopy for Indeterminate Lung Nodules.

Navigational bronchoscopy is commonly used to sample lung nodules, with a better safety profile but lower diagnostic yield than computerized tomography-guided transthoracic needle biopsy. The addition of digital tomosynthesis to electromagnetic navigation, using intraprocedural images obtained from a C-arm fluoroscope to identify target lesion location and update navigational guidance, may improve diagnostic yield. Consecutive bronchoscopies using tomosynthesis-assisted fluoroscopic electromagnetic navigational bronchoscopy (F-ENB) at a single institution over a 1-year period were included. The primary outcome was diagnostic yield. A bronchoscopy was defined as diagnostic if pathologic examination revealed malignancy or specific histological findings indicative of lesional sampling with confirmatory 6-month follow-up for benign lesions. A total of 324 patients with 363 nodules underwent F-ENB between April 25, 2018 and April 29, 2019. The average nodule size was 1.9±1.1 cm, 65% of the nodules were located in the peripheral third of the lung. A bronchus sign was present in 24% of cases. Of the 363 nodules, 299 (82.4%) had lesional findings. At 6-month follow-up, among these 299 nodules, 6 were found to be false negatives and 12 nodules were lost to follow-up. Considering all nodules lost to follow-up as false negatives, the 6-month diagnostic yield was 77.4%. Pneumothorax complicated 8 (2.5%) of cases. There was 1 episode of respiratory failure. This retrospective study suggests the diagnostic yield of F-ENB may exceed that of traditional ENB. Future prospective and comparative studies are needed to confirm these promising data.

Incidence, Risk Factors, and Effects on Outcome of Ventilator-Associated Pneumonia in Patients With Traumatic Brain Injury: Analysis of a Large, Multicenter, Prospective, Observational Longitudinal Study

No large prospective data, to our knowledge, are available on ventilator-associated pneumonia (VAP) in patients with traumatic brain injury (TBI). To evaluate the incidence, timing, and risk factors of VAP after TBI and its effect on patient outcome. This analysis is of the Collaborative European NeuroTrauma Effectiveness Research in Traumatic Brain Injury data set, from a large, multicenter, prospective, observational study including patients with TBI admitted to European ICUs, receiving mechanical ventilation for≥ 48 hours and with an ICU length of stay (LOS)≥ 72 hours. Characteristics of patients with VAP vscharacteristics of patients without VAP were compared, and outcome was assessed at 6months after injury by using the Glasgow Outcome Scale Extended. The study included 962 patients: 196 (20.4%) developed a VAP at a median interval of 5days (interquartile range [IQR], 3-7days) after intubation. Patients who developed VAP were younger (median age, 39.5 [IQR, 25-55] years vs51 [IQR, 30-66] years; P< .001), with a higher incidence of alcohol abuse (36.6%vs27.6%; P= .026) and drug abuse (10.1%vs4.2%; P= .009), more frequent thoracic trauma (53%vs43%; P= .014), and more episodes of respiratory failure during ICU stay (69.9%vs28.1%; P< .001). Age (hazard ratio [HR], 0.99; 95%CI, 0.98-0.99; P= .001), chest trauma (HR, 1.4; 95%CI, 1.03-1.90; P= .033), histamine-receptor antagonist intake (HR, 2.16; 95%CI, 1.37-3.39; P= .001), and antibiotic prophylaxis (HR, 0.69; 95%CI, 0.50-0.96; P= .026) were associated with the risk of VAP. Patients with VAP had a longer duration of mechanical ventilation (median, 15 [IQR, 10-22] days vs8 [IQR, 5-14] days; P< .001) and ICU LOS (median, 20 [IQR, 14-29] days vs13 [IQR, 8-21] days; P< .001). However, VAP was not associated with increased mortality or worse neurological outcome. Overall mortality at 6months was 22%. VAP occurs less often than previously described in patients after TBI and has a detrimental effect on ICU LOS but not on mortality and neurological outcome. ClinicalTrials.gov; No.: NCT02210221; URL: www.clinicaltrials.gov.

Dynamic Changes in Prognosis with Elapsed Time on Ventilators among Mechanically Ventilated Patients.

Rationale: Previous outcome studies of mechanical ventilation usually adopted a static timeframe to observe the outcome and reported prognosis from the standpoint of the first ventilator day. However, patients and their families may repeatedly inquire about prognosis over time after the initiation of mechanical ventilation.Objectives: We aimed to describe dynamic changes in prognosis according to the elapsed time on a ventilator among mechanically ventilated patients.Methods: For this cohort study we used the entire population dataset of Taiwan’s National Health Insurance database. We enrolled adults who newly received invasive mechanical ventilation for at least two consecutive days between March 1, 2010, and August 31, 2011. For every single ventilator day after the initiation of mechanical ventilation, we estimated the cumulative probabilities of weaning success and death in the subsequent 90 days.Results: A total of 162,200 episodes of respiratory failure requiring invasive mechanical ventilation were included. The median age of the subjects was 72 years (interquartile range 57–81 yr) and the median follow-up time was 250 days (interquartile range 30–463 d). The probability curve of weaning success against the time on ventilation showed a unidirectionally decreasing trend, with a relatively sharp slope in the initial 2 months. The probabilities of weaning success in 90 days after the 2nd, 7th, 21st, and 60th ventilator days were 68.3% (95% confidence interval [CI], 68.1–68.5%), 62.6% (95% CI, 62.2–62.9%), 46.3% (95% CI, 45.8–46.8%), and 21.0% (95% CI, 20.3–21.8%), respectively. In contrast, the death curve showed an initial increase and then a decreasing trend after the 19th ventilator day. We also reported tailored prognosis information according to the age, sex, and ventilator day of a mechanically ventilated patient.Conclusions: This study provides ventilator-day–specific prognosis information obtained from a large cohort of unselected patients on invasive mechanical ventilation. The probability of weaning success decreased with the elapsed time on mechanical ventilation, and the decline was particularly remarkable in the first 2 months of ventilatory support.

Respiratory Management of Myotonic Dystrophy

Patients with myotonic dystrophy can avoid episodes of respiratory failure by using up to continuous noninvasive ventilatory support (CNVS). Of 154 patients consecutively presenting to a neuromuscular disease clinic, 143 had types 1 or 2 and 11 had congenital myotonic dystrophy. Of the 76 who came multiple times, 41 were prescribed and used at least sleep NVS to relieve symptoms of fatigue and hypersomnolence. They had sleep and end-tidal (Pet) CO2 with highs over 50 mm Hg and many 4% or greater O2 desats per hour of sleep and all had mean sleep O2 sat levels less than 95% before beginning sleep NVS. Thirteen eventually had to extend NVS use into and eventually throughout daytime hours for CNVS. While using NVS during the day their arterial blood gases were within normal limits. When the 41 were using NVS only during sleep, 24 of the 41 also had normalization of diurnal SpO2 to ≥ 95% and decreased hypercapnia. The 41 patients have been using sleep NVS for 6.70±3.64 (range = 0.75 to 19) years per patient or for 274.7 patient-years. Seventy-two have died or been lost to follow-up. The 13 up to CNVS users have been doing so for 75.7 patient-years or a mean of 5.8±3.2 (range = 0.2 to 10) years per patient. The VC from first visit to most recent visit of all 71 decreased a mean 49.3±168.8 ml or about 1.5% per year. it decreased a mean 52±171 per year for the 41 NVS users over 274.7± 3.64 patient-years.

Adverse Events Following Cardiac Catheterization among Infants, Children, and Young Adults on Ventricular Assist Device Support

Children on ventricular assist device (VAD) support often present unique challenges, such as small size, univentricular or biventricular congenital heart disease (1V- or 2V-CHD), and need for biventricular (BiVAD) support. While cardiac catheterization (cath) can provide valuable information in the management of pediatric patients on VAD support, it is an invasive procedure with inherent risks. We sought to evaluate the safety of cath in this population. We performed a retrospective review of patients on VAD support who underwent cath at a single pediatric VAD center between 2014 and 2019. Using definitions based on Pedimacs, adverse events (AEs) following cath were identified, including respiratory failure (persistent need for intubation ≥ 48 hours post-cath), acute kidney injury (AKI), or arrhythmia within 24 hours, and stroke, pericardial effusion, device malfunction, infection, and death within 7 days. AEs were reviewed by two cardiologists, blinded to each other's assessments, and categorized as directly related or unrelated to cath. Strokes were unrelated to cath if there was documentation of baseline neurologic status after cath but prior to stroke. Descriptive statistics were employed. There were 60 caths on 39 patients. Median age was 11 years (range 2 months - 22 years) and median weight was 36 kg (3.8 - 99.2). Underlying diagnoses included dilated cardiomyopathy (DCM) (54%), 1V-CHD (31%), 2V-CHD (10%), hypertrophic cardiomyopathy (2.5%) and re-transplant (2.5%); 10% were on BiVAD support. Devices were paracorporeal pulsatile (20%), durable continuous flow (72%), and temporary continuous flow (8%). Caths were performed at a median 50 days (6 - 311) from VAD implant; 60% (36/60) were performed on patients in the intensive care unit. Of the 60 caths, there were 7 AEs following cath believed to be directly or possibly related to cath (12%, 7/60), occurring in 6 patients (2 DCM, 3 1V-CHD, 1 other): 1 major bleeding event, 1 AKI, 2 moderate pericardial effusions, and 3 episodes of respiratory failure. There were no strokes, arrhythmia, infections, or mortality attributable to cath. Despite medical complexity such as small size, complex CHD and intensive care needs, cardiac catheterization can be performed with an acceptable adverse event profile in infants, children and young adults on VAD support.

Atrial arrhythmias in chronic lung disease-associated pulmonary hypertension.

Atrial arrhythmias are common during episodes of acute respiratory failure in patients with chronic lung disease-associated pulmonary hypertension. Expert opinion suggests that management of atrial arrhythmias in patients with pulmonary hypertension should aim to restore sinus rhythm. This is clinically challenging in pulmonary hypertension patients with coexisting chronic lung disease, as there is controversy on the use of rhythm control agents; generally, in regard to either their pulmonary toxicity profile or the lack of evidence supporting their use. Rate control methods are largely focused on the use of beta blockers and calcium channel blockers. Concerns regarding their use involve their negative inotropic properties in cor pulmonale, the risk of bronchospasm associated with beta blockers, and the potential for ventilation/perfusion mismatching associated with calcium channel blockers. While digoxin has been associated with promising outcomes during acute right ventricular failure, there is limited evidence to suggest its routine use. Electrical cardioversion is associated with a high failure rate and it frequently requires multiple attempts. Radiofrequency catheter ablation is a more definitive approach, but concerns surrounding mechanical ventilation and sedation limit its applicability in decompensated pulmonary hypertension. Individual approaches are needed to address atrial arrhythmia management during acute episodes of respiratory failure.

The clinical case of limb-girdle muscle dystrophy 2Q associated with myasthenic syndrome and lung damage

Limb-girdle muscle dystrophy 2Q is one of the rarest forms of plectinopathies and is represented by an isolated muscular dystrophic syndrome, according to two previously described literature reports. There are five forms of plectinopathies, including limb-girdle muscle dystrophy 2Q, are caused by mutations in the PLEC gene, the alternative splicing of which determines the synthesis of 9 isoforms of the plectin protein (1, 1a, 1b, 1c, 1d, 1e, 1f, 1g, 3) performing cytolinker function in the neuronal, epithelial and muscle tissue. The article describes the family observation of three sick siblings with the limb-girdle muscle dystrophy 2Q phenotype due to the presence of a new homozygous mutation (NM_201378.3:c.58G&gt;T, NP_958780.1:p.Glu20Ter) in the isoform 1f PLEC revealed by whole-exome sequencing. Clinical, electromyography, visualization and histopathological features of limb-girdle muscle dystrophy 2Q were analyzed in detail. The onset of clinical manifestations in all the described siblings was observed in early childhood with moderate weakness mainly in the pelvic girdle muscles and proximal lower limbs with minimal involvement of the muscles of the shoulder girdle. A distinctive aspect is the stagnation of the myodystrophic process until 20—21 years, followed by the progression and development of episodes of respiratory failure, as well as the formation of rigidity of the cervical, thoracic spine and moderate contracture of the Achilles tendons. Typical features are marked atrophy of paravertebral muscles with the formation of pterygoid scapula and the presence of hypertrophy m. gastrocnemius, m. quadriceps femoris, m. deltoideus and m. triceps brachii. Histopathological examination m. vastus lateralis revealed myodystrophic process without inflammatory infiltration, muscle fiber cytoskeleton disorganization resulted from the plectin loss. Electrocardiography signs of the early repolarization syndrome, focal cardiosclerosis and sinus tachycardia are described. For the first time, involvement in the pathological process of pulmonary tissue in the form of noninfectious bronchiolitis, atelectasis, and the development of the myasthenic syndrome causing episodes of respiratory failure resulted in the death of two described siblings aged 29 and 31 years. Discussed pathogenetic role of PLEC 1f isoform in the development of described syndromes, expands understanding of rare nosology limb-girdle muscle dystrophy 2Q.

Acute Promyelocytic Leukemia (APL) in Very Elderly Patients: Real-Life behind Protocols

Acute Promyelocytic Leukemia (APL) in Very Elderly Patients: Real-Life behind Protocols

Surgical treatment of aortic valve disease: early results of a 360° minimally invasive approach

Over the years, with the introduction of minimally invasive techniques and technologies aimed at reducing surgical trauma, aortic valve surgery has considerably developed and improved. Our approach includes: reduced incisions (upper "J" ministernomy or anterior right minithoracotomy), "ultra fast-track" anesthesia protocols, sutureless and rapid deployment valve prostheses and miniaturized circuits of extracorporeal circulation. The aim of this study was to evaluate the clinical outcomes associated with this multidisciplinary approach. Between October 2016 and November 2018, 429 patients underwent isolated aortic valve replacement at the Cardiac Surgery Unit of the "Ospedali Riuniti" of Ancona, Italy. Overall, 91 patients (21.2%) were operated according to our minimally invasive approach. A severe aortic valve stenosis was the indication for surgery in 90.1% of patients, aortic valve insufficiency in the remaining 18.7%. There were neither in-hospital deaths nor major or minor neurological events. Atrial fibrillation was the main postoperative complications (n=26, 28.6%). Four patients (4.4%) underwent permanent pacemaker implantation due to third-degree atrioventricular block, and a surgical bleeding revision was performed in 3.3%. No episodes of respiratory failure were reported. The median length of hospital stay was 6 days (5-8 days). Our initial experience with a 360° minimally invasive approach for the treatment of patients undergoing aortic valve replacement shows encouraging clinical outcomes; this approach may lead to an improved perception of surgery both by patients and their families. However, further clinical studies are needed to evaluate the long-term results.

Noninvasive ventilation as the first choice of ventilatory support in children.

ObjectiveTo describe the use of noninvasive ventilation to prevent tracheal intubation in children in a pediatric intensive care unit and to analyze the factors related to respiratory failure.MethodsA retrospective cohort study was performed from January 2016 to May 2018. The study population included children aged 1 to 14 years who were subjected to noninvasive ventilation as the first therapeutic choice for acute respiratory failure. Biological, clinical and managerial data were analyzed by applying a model with the variables that obtained significance ≤ 0.20 in a bivariate analysis. Logistic regression was performed using the ENTER method. The level of significance was set at 5%.ResultsThe children had a mean age of 68.7 ± 42.3 months, 96.6% had respiratory disease as a primary diagnosis, and 15.8% had comorbidities. Of the 209 patients, noninvasive ventilation was the first option for ventilatory support in 86.6% of the patients, and the fraction of inspired oxygen was ≥ 0.40 in 47% of the cases. The lethality rate was 1.4%. The data for the use of noninvasive ventilation showed a high success rate of 95.3% (84.32 - 106). The Pediatric Risk of Mortality (PRISM) score and the length of stay in the intensive care unit were the significant clinical variables for the success or failure of noninvasive ventilation.ConclusionA high rate of effectiveness was found for the use of noninvasive ventilation for acute episodes of respiratory failure. A higher PRISM score on admission, comorbidities associated with respiratory symptoms and oxygen use ≥ 40% were independent factors related to noninvasive ventilation failure.

Iliac conduits remain safe in complex endovascular aortic repair

ObjectiveIliac conduits (ICs) are used for challenging iliac access during endovascular aortic aneurysm repair (EVAR) with contradictory reports of safety and increased surgical complications, morbidity, and mortality in national database reviews. The objective of this study was to examine outcomes related to IC placement before or concomitant with EVAR at a high-volume single center. MethodsA retrospective analysis of patients who underwent IC placement for endovascular aortic repair from 2006 to 2016 was conducted. Planned and unplanned, as well as staged and concomitant IC for EVAR, thoracic EVAR, and fenestrated/branched EVAR were included. Categorical factors were described using frequencies and percentages. Continuous measures were summarized in comparative tables. Comparisons between the planned and unplanned groups, concomitant and staged groups, and between those with and without a postoperative event were performed using Pearson χ2 tests, two-sample t-tests, and Wilcoxon rank-sum tests. ResultsA total of 137 patients underwent IC placement, of which 9 were bilateral procedures, for a total of 144 IC placements. IC placement was necessary in 14 EVARs (9.7%), 38 thoracic EVARs (26.4%), and 92 fenestrated/branched EVARs (63.9%). The 30-day related mortality was 2.1% (n = 3). Perioperative morbidities included return to the operating room in five patients (5.6%) for bleeding (n = 4) and graft thrombosis (n = 1), 4 myocardial infarctions (2.8%), 5 episodes of respiratory failure (3.5%), 12 wound complications (8.3%), and 7 renal injuries (4.9%), of which 3 had progression to dialysis (2.1%). Other complications included return to the operating room for endoleak reintervention (n = 3) and late graft infection (n = 1 [0.7%]). There were 112 ICs (77.8%) planned and 65 (45.1%) were staged. Staged ICs were less likely to have postoperative complications (P < .001), respiratory failure (P < .05), infectious complications (P < .05), and postoperative cerebrovascular accidents (P < .05). ICs were done to accommodate 20F to 24F sheath sizes in 131 instances (91.0%). Factors associated with unplanned IC placement were iliac rupture and an inability to advance the endograft in arteries deemed size-appropriate preoperatively (P < .001). Unplanned ICs were associated with higher estimated blood loss (P < .001), operating room transfusion volume (P < .001), and overall complication rates (P < .05). Women (P < .05), preoperative chronic kidney disease (P < .05), and concomitant IC placement (P < .001) were associated with higher overall complication rates. Operative time, estimated blood loss, and operating room transfusion volume were associated with increased rate of complications in comparison between all subgroups (P < .05). ConclusionsICs are a safe and viable option for high-risk patients with challenging iliac artery access for EVVAR. ICs are best performed in a planned fashion or in a staged manner, when feasible.

Too Weak to Breathe: An Odd Case of Lambert Eaton Myasthenic Syndrome

SESSION TITLE: Critical Care 3 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Lambert Eaton Myasthenic Syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction characterized by the presence of antibodies against voltage gated calcium channels (VGCC) often associated with an underlying neoplasm. CASE PRESENTATION: 52 year old Caucasian female was admitted to an outside hospital with lethargy, shortness of breath and was intubated for acute hypercapnic respiratory failure which ultimately resolved and she was extubated. Her discharge diagnosis was presumed obstructive sleep apnea. Three weeks later she presented to our hospital with the same symptoms. Physical exam was significant for diminished breath sounds bilaterally and musculoskeletal exam was normal on presentation. Arterial blood gas was 7.26/79/118/35 after 30 minutes on non-invasive positive pressure ventilation, prompting intubation. On the ventilator she was awake and alert without sedation, but failed repeated breathing trials due to apnea, low tidal volumes and a negative inspiratory force of -10 cmH2O. Her persistant, profound and unexplained respiratory muscle weakness, raised concerns for a neuromuscular disorder and neurology was consulted. Electromyogram and nerve conduction studies revealed a post exercise increase in compound muscle action potential response > 200%, her VGCC antibodies were positive and acetylcholine receptor antibodies were negative. Computerized Tomography of the chest demonstrated a right suprahilar mass with a paratracheal lymph node which was hypermetabolic on Positron Emission Tomography. Endobronchial Ultrasound with biopsy of the lymph node confirmed small cell lung carcinoma. She was treated with pyridostigmine and plasma exchange with excellent response and was extubated several days later. She received three cycles of etoposide, cisplatin, chest radiation and is currently on mestinon and 3,4 diaminopyridine for her LEMS. DISCUSSION: LEMS classically presents as proximal muscle weakness, autonomic dysfunction and areflexia. Respiratory failure is a common primary manifestation of Myasthenia Gravis, however the literature rarely documents LEMS as presenting this way. This case highlights the importance of considering occult neuromuscular diseases in cases of unexplained respiratory failure even if other classical symptoms such as muscle weakness are absent. Our patient had repeated episodes of respiratory failure requiring intubation that was initially misdiagnosed, leading to missed treatment opportunities, a poor quality of life and an unchecked pulmonary malignancy. CONCLUSIONS: LEMS often presents early in the neoplastic course, thus diagnosis has important implications. Treatment of LEMS can be effective in relieving the neuromuscular symptoms and early detection of the underlying malignancy can be lifesaving. Reference #1: Fatima. U., Shah. T., Dedhia.P., Devine. S., Lambert Eaton Syndrome: Unique Presentation of the Disease with Isolated Respiratory Muscle Weakness. CHEST. Abstract 326A. October 2013. DISCLOSURE: The following authors have nothing to disclose: Caryn Brehm, Christopher Barrios, James Caress, Daniel Files, Daniel LoVerde No Product/Research Disclosure Information