OBJECTIVE: Noninvasive ventilation (NIV) has been shown to improve survival and symptom burden in patients with amyotrophic lateral sclerosis (ALS). However, limited data exist regarding the clinical and physiological parameters at the time of NIV initiation. This study aimed to describe the clinical characteristics and respiratory physiological markers in a cohort of ALS patients with chronic respiratory failure.
METHODS: This is a single-center retrospective cohort study of patients with ALS assessed for NIV initiation between February 2012 and January 2021. NIV was initiated based on insurance eligibility criteria: daytime hypercapnia, defined by partial pressure of carbon dioxide (PaCO2) >45 mm Hg using diurnal transcutaneous CO2 (TcCO2) as a surrogate, a maximal inspiratory pressure (MIP) <60 cmH2O or forced vital capacity (FVC) <50% predicted normal.
RESULTS: We identified 335 patients with ALS and chronic respiratory failure referred to an outpatient home ventilation clinic for NIV initiation. The mean age was 64 years ±11; 151 (45%) were female, 326 (97%) were white, and 100 (29%) had bulbar-onset ALS. At the time of NIV initiation, the mean FVC was 64% ± 19%, the mean MIP; 41 cmH2O ± 17, and diurnal TcCO2; 40 ± 6 mmHg. The most common reasons for NIV initiation were MIP <60 cmH2O (58%) and multiple concomitant indications (28%). Within 1 year of NIV initiation, 126 (37%) patients were deceased.
CONCLUSIONS: We found that impairment in inspiratory force was the most common reason for NIV initiation and often preceded significant declines in FVC.

Blastomycosis can result in lung injury with high mortality rates. The literature on veno-venous extracorporeal membrane oxygenation (VV-ECMO) used as a rescue therapy is limited to case reports and small case series collected over extended time periods. This report describes the clinical course and post-hospitalization outcomes among patients with blastomycosis-induced respiratory failure requiring VV-ECMO in the most recent time frame. The data were collected retrospectively from the health records of eight patients with blastomycosis-induced respiratory failure admitted to a tertiary care center between 2019 and 2023. The mean time from the start of mechanical ventilation to ECMO initiation was 57 h. All patients survived to ECMO decannulation, and seven of them survived to hospital discharge. All six patients whose post-discharge follow-up information was available were weaned from mechanical ventilation and lived at home while two required supplemental oxygen. This includes a case where the provision of adequate ECMO support was challenging due to the patient\'s morbid obesity. The most common residual imaging abnormalities included pulmonary infiltrates and pneumatoceles. The study demonstrates the feasibility of VV-ECMO as a rescue therapy in patients with blastomycosis-related refractory respiratory failure. Rapid initiation of ECMO support in eligible patients may have contributed to the good outcomes.

OBJECTIVE: Acute hypoxaemic respiratory failure (AHRF) is a common reason for intensive care unit (ICU) admission. However, patient characteristics, outcomes, and trends over time are unclear. We describe the epidemiology and outcomes of patients with AHRF over time.
METHODS: In this binational, registry-based study from 2005 to 2022, we included all adults admitted to an Australian or New Zealand ICU with an arterial blood gas within the first 24 h of ICU stay. AHRF was defined as a partial pressure of oxygen/inspired oxygen ratio (PaO2/FiO2) ≤ 300. The primary outcome was adjusted in-hospital mortality, categorised based on PaO2/FiO2 (mild: 200-300, moderate: 100-200, and severe < 100, and non-linearly). We investigated how adjusted mortality evolved based on temporal trends (by year of admission), sex, age, admission diagnosis and the receipt of mechanical ventilation.
RESULTS: Of 1,560,221 patients, 826,106 (52.9%) were admitted with or developed AHRF within the first 24 h of ICU stay. Of these 826,106 patients, 51.4% had mild, 39.3% had moderate, and 9.3% had severe AHRF. Compared to patients without AHRF (5.3%), patients with mild (8%), moderate (14.2%) and severe (29.9%) AHRF had higher in-hospital mortality rates. As PaO2/FiO2 ratio decreased, adjusted in-hospital mortality progressively increased, particularly below an inflection point at a PaO2/FiO2 ratio of 200. The adjusted in-hospital mortality for all patients decreased over time (13.3% in 2005 to 8.2% in 2022), and this trend was similar in patients with and without AHRF.
CONCLUSIONS: The healthcare burden due to AHRF may be larger than expected, and mortality rates remain high in severe AHRF. Although mortality has decreased over time, this may reflect improvements in ICU care in general, rather than specifically in AHRF. More research is required to earlier identify AHRF and stratify these patients at risk of deterioration early, and to validate our findings.

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As the medical complexity of pregnant patients increases, the rate of maternal morbidity has risen. Maternal cardiovascular disease is a leading cause of maternal morbidity and mortality followed closely by sepsis and infection, both of which may be associated with respiratory failure. There has been an expansion in the application of extracorporeal life support in pregnant and peripartum patients which requires obstetric anesthesiologists to understand the indications, obstetric and medical considerations, relative advantages and potential complications of this invasive technology in this population. Obstetricians and anesthesiologists who care for women on the labor floor must strive to recognize at-risk and deteriorating patients, facilitate escalation of care when appropriate, and engage consultant teams to consider the need for extracorporeal support in high-risk circumstances. This article reviews the epidemiology, indications, specific considerations, potential complications, and outcomes of extracorporeal life support in pregnant and peripartum patients.

Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but fatal complication of a malignant tumor that causes rapidly progressive pulmonary hypertension (PH). We report the case of a 70-year-old Japanese man who died of respiratory failure during chemotherapy for gastric cancer and was diagnosed with PTTM at autopsy. The autopsy revealed PTTM-specific histological findings, such as tumor emboli with fibrin-rich clots and fibrocellular intimal proliferation in the vessels. The cancer cells were immunohistochemically positive for vascular endothelial growth factor and platelet-derived growth factor, whereas the thickened intima of the pulmonary arteries was positive for versican (VCAN). As VCAN is an extracellular matrix proteoglycan that is dramatically increased in vascular lesions of pulmonary arterial hypertension, this case demonstrates that VCAN is also involved in the pathophysiology of PTTM.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) through the femoral artery and vein can lead to significant vascular complications. We retrospectively studied the acute vascular complications of Veno-Arterial Extracorporeal Membrane Oxygenation (VA-ECMO) in COVID-19 patients compared to non-COVID patients during the period from January 2020 to July 2023.
RESULTS: Seventy-eight patients underwent VA-ECMO for various indications from January 2020 to July 2023. The studied patients had a mean age of 59.6 ± 6.9 years for non-COVID patients (38 patients), and 62.2 ± 7.6 years for COVID patients (40 patients), with a P = 0.268. In non-COVID patients, The baseline characteristics were similar in both groups. The primary indications for ECMO were cardiac diseases, followed by respiratory failure (78.9% vs 10.5%). Conversely, in COVID patients, respiratory failure due to COVID-19 infection was the main indication (45% vs 40%). The overall incidence of general complications, including cerebrovascular stroke, acute kidney injury, intracardiac thrombi, and wound infection, was comparable in both groups (31.6% vs 45%). The overall incidence of vascular complications in both groups was 33.3%. Ipsilateral acute lower limb ischemia occurred in 5.3% vs 10% of non-COVID and COVID patients, respectively. Thrombosis of the distal perfusion catheter (DPC) occurred in 10.5% vs 15%, respectively.
CONCLUSIONS: During the COVID-19 pandemic, an increasing number of patients required VA-ECMO due to associated respiratory failure. Patients undergoing VA-ECMO are at high risk of developing various vascular complications. COVID-19 significantly increases the risk of acute limb ischemia and distal perfusion catheter thrombosis in both upper and lower limbs. However, other VA-ECMO-related vascular complications are comparable between COVID-19 and non-COVID patients.

BACKGROUND: Right ventricular dysfunction is associated with mortality in patients with acute respiratory distress syndrome (ARDS) but information in veno-venous extracorporeal membrane oxygenation (ECMO) settings is limited. Study objectives were to examine factors associated with right ventricular (RV) systolic dysfunction (RVSD) and RV dilation in ECMO patients with ARDS, to compare outcomes in those with and without RVSD and RV dilation defined by qualitative and quantitative parameters, and to describe RVSD evolution during ECMO.
METHODS: Retrospective observational study of adult ARDS patients supported with ECMO at a tertiary care hospital.
RESULTS: Of a total of 62 patients, 56% had RVSD and 61% had RV dilation by qualitative assessment. Male gender, COVID-19, hypercarbia, and pneumothorax were associated with RVSD and RV dilation. In-hospital mortality was significantly higher in patients with RV dilation vs. no dilation (42% vs. 17%, p = .05) but comparisons for patients with and without RVSD (37% vs. 26%, respectively) did not reach statistical significance. Findings were similar when RV size and function were quantified by right to left ventricle end-diastolic area ratio and fractional area change (39% vs. 21% and 36% vs. 20% respectively; p = NS). Of 39 patients with multiple echocardiograms, 9 of 18 with initially normal RV function developed RVSD while RV function normalized in 10 of 21 patients who began ECMO with RVSD.
CONCLUSIONS: Study results suggest an association of RV dilation and RVSD with worse outcomes and a dynamic nature of RV function necessitating close monitoring during the ECMO course.

UNASSIGNED: Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a rare autosomal recessive hereditary disease. Immunoglobulin μ-binding protein 2 (IGHMBP2) gene mutations are the main cause of SMARD1.
UNASSIGNED: Here we describe a female infant with SMARD1 carrying heterozygous mutations in IGHMBP2 genes, c.1334A > C(p.His445Pro) and c.1666C > G(p.His556Asp), which were inherited from both parents. Clinical presentations included frequent respiratory infections, respiratory failure, distal limb muscle weakness, and fat pad found at the distal toe.
UNASSIGNED: c.1666C > G(p.His556Asp) is a novel site mutation in IGHMBP2. This case expanded knowledge on the genetic profile of SMARD1 and it provides a basis for genetic testing of parents and for genetic counseling to assess the risk of fetal disease.

In recent years the number of patients treated in intensive care units by extracorporeal membrane oxygenation (ECMO) due to severe respiratory failure or cardiogenic shock has steadily increased [1]. Consequently, the number of invasive procedures and operations in these patients has also increased. A fundamental understanding of these systems and the clinical indications is therefore helpful for the practicing (non-cardiac) surgeon. This review article focuses on peripheral ECMO procedures: venovenous (V-V) ECMO for patients with respiratory failure and venoarterial (V-A) ECMO for circulatory support in cardiogenic shock.
UNASSIGNED: In den letzten Jahren haben sich die Zahlen von Patienten, die mit einer extrakorporalen Membranoxygenierung (ECMO) aufgrund einer schweren respiratorischen Insuffizienz oder eines kardiogenen Schocks auf Intensivstationen behandelt werden, stetig erhöht [1]. Damit steigt auch zwangsläufig die Anzahl an invasiven Prozeduren und Operationen bei dieser Patientenklientel. Ein prinzipielles Verständnis der Funktionsweise und der Indikationen für den Einsatz dieser Systeme ist damit auch für den (nicht kardio‑)chirurgisch tätigen Arzt hilfreich. Der Schwerpunkt dieses Übersichtsartikels liegt auf den peripheren ECMO-Verfahren: venovenöse (V-V-)ECMO bei Patienten mit respiratorischer Insuffizienz und venoarterielle (V-A-)ECMO zur Kreislaufunterstützung bei kardiogenem Schock.