Lung Transplantation in Patients With Systemic Scleroderma-Description of the First Consecutive Cases in Poland: Case Series Report and a Short Literature Review.

Pulmonary complications of systemic scleroderma (SSc), such as interstitial lung disease and pulmonary hypertension (PH), are responsible for up to 60% of deaths among patients. For many years, most centers considered SSc a contraindication to lung transplantation (LTx); however, recent publications show that appropriately selected SSc candidates for LTx give results comparable to patients with idiopathic PH or idiopathic pulmonary fibrosis. This paper presents the cases of a 60-year-old male patient (patient 1) and a 42-year-old female patient (patient 2) diagnosed with SSc in 2019 and 2013, respectively. In both patients, interstitial-fibrotic changes in the lungs leading to respiratory failure were confirmed by high-resolution computed tomography as well as pulmonary hypertension (WHO group 3), which was also diagnosed during right heart catheterization. In both cases, despite pharmacotherapy, pulmonary fibrosis progressed, leading to severe respiratory failure. The patients were referred for LTx qualification. LTx was possible to consider in patients due to the lack of significant changes in other internal organs. Double LTx was successfully performed in both patients (patient 1-July 19, 2022; patient 2-September 14, 2022). They were discharged from the hospital in good condition on the 22nd and 20th postoperative day, respectively. LTx is a last-chance therapy that saves lives among patients with extreme respiratory failure in the course of SSc. It prolongs and improves the quality of life. The selection of appropriate patients is key to the success of the procedure.

A First Experience of Distant Lung Preservation for Transplantation in the Russian Federation.

Lung transplant is an effective method of treating patients with end-stage respiratory diseases, but problems such as the imbalance between the number of donor organs and the number ofrecipients needing organs still play a leading role. From a transplant point of view, a multiorgan donor is considered of greatest efficiency, so that all organs that can potentially be used should be transplanted. The combination of the vast geographical territory of Russia, the shortage of actual donors, and the relatively small number of transplant centers has led to the need to transport donor lungs by air over long distances. There were already precedents in the world for remote preservation of donor organs for transplant. In this study, we have described the unique experience of remote evaluation of donor lungs with their subsequent air transportation and transplantation, which is the first such description in Russia to our knowledge. The donor lungs for lung transplant were brought from medical institutions of the Samara region to Moscow. During remote evaluation, all information was transmitted to the transplant center by providing access to the automated information system "Organ Donation," which was used at that time by the service and contained all information about a potential donor in real time. The 2 transplant candidates had end-stage cystic fibrosis and severe respiratory failure; both patients underwent organ implantation from donors located outside their regions. In conditions of shortages of donor organs, long-distance transportation is a reasonable, feasible, and safe procedure.

Pediatric Lung Transplantations: New Possibilities and Challenges in Treatment of Children With End-Stage Respiratory Failure.

INTRODUCTION: Lung transplantation (LTx) is the last treatment option for children with end-stage respiratory failure. According to the literature, cystic fibrosis remains the most common cause of pediatric LTx. The study aimed to assess the characteristics of pediatric LTx recipients as well as the outcomes of the transplantation. METHODS: Our study is a single-center retrospective review of clinical data of all 11 patients who underwent a LTx before the age of 18 years between the years 2016 and 2020. Medical records were examined for patients' characteristics, general treatment, and complications. RESULTS: There were a total of 11 patients (8 males) with a median age 14.5 years (range: 11-17). The primary diseases that led to LTx were: cystic fibrosis in 8 patients (72.73%), hereditary hemorrhagic telangiectasia in 2 patients (18.18%), and idiopathic pulmonary arterial hypertension in 1 patient (9.09%). Median period from qualification to LTx was 235.55 days (range: 11-748). Two patients (18.18%) underwent lung retransplantation after 3 and 5 years. One patient passed away 10 months after surgery due to noncompliance. CONCLUSIONS: Pediatric lung transplantation is less common than lung transplantation in adults. It also differs in fields of donors accessibility, stronger immune system response and noncompliance that may lead to graft failure.

Center-Level Variation in Hospitalization Costs of Transcatheter Aortic Valve Replacement.

BACKGROUND: Using a nationally representative database, the present study evaluated the degree of center-level variation in the cost of transcatheter aortic valve replacement (TAVR). METHODS: All adults undergoing elective, isolated TAVR were identified in the 2016 to 2018 Nationwide Readmissions Database. Multilevel mixed-effects models were used to identify patient and hospital characteristics associated with hospitalization costs. The random intercept for each hospital was generated and considered to be the baseline cost attributable to care at each center. Hospitals in the highest decile of baseline costs were classified as high-cost hospitals. The association of high-cost hospital status with in-hospital mortality and perioperative complications was subsequently assessed. RESULTS: An estimated 119,492 patients, with a mean age of 80 years and a 45.9% prevalence of female sex, met the study criteria. Analysis of random intercepts indicated that 54.3% of variability in costs was attributable to interhospital differences rather than patient factors. Perioperative respiratory failure, neurologic complications, and acute kidney injury were associated with increased episodic expenditure but did not explain the observed center-level variation. The baseline cost associated with each hospital ranged from -$26,000 to $162,000. Notably, high-cost hospital status was not linked to annual TAVR caseload or to odds of mortality (P = .83), acute kidney injury (P = .18), respiratory failure (P = .32), or neurologic complications (P = .55). CONCLUSIONS: The present analysis identified significant variation in the cost of TAVR, which was largely attributable to center-level rather than patient factors. Hospital TAVR volume and occurrence of complications were not drivers of the observed variation.

Extracorporeal membrane oxygenation as a bridge to lung transplantation: Practice patterns and patient outcomes.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is increasingly relied on to bridge patients with respiratory failure to lung transplantation despite limited evidence for its use in this setting. This study evaluated longitudinal trends in practice patterns, patient characteristics, and outcomes in patients bridged with ECMO to lung transplant. METHODS: A retrospective review of all adult isolated lung transplant patients in the United Network for Organ Sharing database between 2000 and 2019 was performed. Patients were classified as "ECMO" if supported with ECMO at the time of listing or transplantation and "non-ECMO" otherwise. Linear regression was used to evaluate trends in patient demographics during the study period. Trends in mortality were evaluated using Cox proportional hazards modeling, with time period as the primary covariate (2000-2004, 2005-2009, 2010-2014, or 2015-2019) and age, time on the waitlist, and underlying diagnosis as covariates. RESULTS: The number of patients included were 40,866, of whom 1,387 (3.4%) were classified as ECMO and 39,479 (96.6%) as no ECMO. Average age and initial Lung Allocation Score increased significantly during the study period in both cohorts, but occurred at a slower rate in the ECMO population. The hazard of death was significantly lower in more recent years (2015-2019) for both the ECMO and non-ECMO cohorts (aHR (adjusted hazards ratio) 0.59, 95% confidence interval (CI) 0.37-0.96 and aHR 0.74, 95% CI 0.70-0.79) when compared to the early years (2000-2004) of the study period. CONCLUSIONS: Post-transplantation survival for patients bridged to transplantation with ECMO demonstrates ongoing improvement despite cannulation of progressively older and sicker patients.

Changing patterns in lung transplant for respiratory failure due to COVID-19 in the U.S.

Many patients with severe COVID-19 have been affected by acute respiratory distress syndrome, which has been associated with increased mortality, and up to 31% of these survivors had persistent interstitial lung abnormalities with impaired lung function and quality of life even after 6 to 24 months after initial disease. Lung transplantation quickly emerged as a viable therapy for select patients with respiratory failure due to COVID-19 by mid-2020. In this report, we identified 477 patients who underwent lung transplantation for COVID-19 in the U.S. between March 2020 and December 2022. The number of patients waitlisted and undergoing lung transplantation for COVID-19 increased steadily in the early part of the pandemic with a peak of 97 patients waitlisted between October and December 2021, before steadily decreasing since. Notably, the procedure is now increasingly being done for survivors of COVID-19 with pulmonary fibrosis, rather than for refractory ARDS patients. The 1-year post-transplant mortality was 13.7%.

Surgical experience of extracorporeal membrane oxygenation for neonates with severe respiratory failure.

OBJECTIVE: Extracorporeal membrane oxygenation (ECMO) has been increasingly used for severe neonatal respiratory failure refractory to conventional treatments. This paper summarizes our operation experience of neonatal ECMO via cannulation of the internal jugular vein and carotid artery. METHODS: The clinical data of 12 neonates with severe respiratory failure who underwent ECMO via the internal jugular vein and carotid artery in our hospital from January 2021 to October 2022 were collected. RESULTS: All neonates were successfully operated on. The size of arterial intubation was 8 F, and the size of venous intubation was 10 F. The operation time was 29 (22-40) minutes. ECMO was successfully removed in 8 neonates. Surgeons successfully reconstructed the internal jugular vein and carotid artery of these neonates. Arterial blood flow was unobstructed in 5 patients, mild stenosis was present in 2 patients, and moderate stenosis was present in 1 patient. Venous blood flow was unobstructed in 6 patients, mild stenosis was present in 1 patient, and moderate stenosis was present in 1 patient. The complications were as follows: 1 case had poor neck incision healing after ECMO removal. No complications, such as incisional bleeding, incisional infection, catheter-related blood infection, cannulation accidentally pulling away, vascular laceration, thrombosis, cerebral haemorrhage, cerebral infarction, or haemolysis, occurred in any of the patients. CONCLUSION: Cannulation of the internal jugular vein and carotid artery can quickly establish effective ECMO access for neonates with severe respiratory failure. Careful, skilled and delicate operation was essential. In addition, during the cannulation process, we should pay special attention to the position of cannulation, firm fixation and strict aseptic operation.

[Successful Atrial Plication in Improving Respiratory Failure due to Bilateral Giant Atria].

Giant atria may trigger respiratory failure, which often requires surgical intervention. We report a patient who presented with respiratory failure due to bilateral giant atria. The patient was a 75-year-old woman with rheumatic heart disease. She had undergone mitral valve replacement and tricuspid annuloplasty at another hospital 17 years ago but recently developed respiratory dysfunction. Compression to the lungs by enlarged atria was diagnosed as the main cause of respiratory dysfunction. Hence, the anterior-to-posterior left atrial wall was plicated by para-annular and superior-half plication, respectively, and the right atrial wall was excised into an ellipse shape. Tricuspid valvuloplasty was performed on four sets of eight artificial chordae with CV5 sutures and an annuloplasty ring. Respiratory failure was alleviated after the surgery.

Mortality Among Children with a Tracheostomy.

OBJECTIVES: To characterize the cause of death among children with a tracheostomy. STUDY DESIGN: Prospective cohort. METHODS: All pediatric patients (<18 years) who had a tracheostomy placed at a tertiary care institution between 2015 and 2020 were included. The location and cause of death were recorded along with patient demographics and age. RESULTS: A total of 271 tracheostomies were placed with 46 mortalities reviewed for a mortality rate of 16.8%. Mean age at placement was 1.7 years (SD: 3.4) and mean age at death was 2.9 years (SD: 3.5). Most tracheostomies were placed for respiratory failure (N = 33, 72%). The mean time to death after tracheostomy was 1.2 years (SD: 1.2) and 28% (N = 13) occurred during the same admission as placement. Mean time to death after hospital discharge was 1.3 years (SD: 1.3). Etiology of death was respiratory failure (33%, N = 15), cardiopulmonary arrest (15%, N = 7), unknown (43%, N = 20), or secondary to a tracheostomy-related complication for 9% (N = 4). Location of death was in intensive care units for 41% (N = 19) and 30% died at home (N = 14). Comfort care measures were taken for 37% (N = 17). Severe neurological disability (HR: 4.06, p = 0.003, 95% CI: 1.59-10.34) and congenital heart disease (HR: 2.36, p = 0.009, 95% CI: 1.24-4.48) correlated with time to death on Cox proportional hazard modeling. CONCLUSIONS: Nearly one-third of children with a tracheostomy who expire will do so during the same admission as tracheostomy placement. Although progression of underlying disease will lead to most deaths, 9% will be a result of a tracheostomy-related complication, which represents a meaningful target for quality improvement initiatives. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:403-409, 2023.

A Matched Survival Analysis of Lung Transplant Recipients With Coronavirus Disease 2019-Related Respiratory Failure.

BACKGROUND: Lung transplantation is an acceptable and potentially life-saving treatment option for coronavirus disease 2019 (COVID-19)-induced acute respiratory distress syndrome and pulmonary fibrosis. This study was conducted to determine whether recipients of lung transplantation (LT) for COVID-19-related lung disease have comparable outcomes to other recipients with a similar level of lung dysfunction. METHODS: The Organ Procurement and Transplant Network database was queried for adult LT candidates between 2006 and 2021. Recipients with COVID-19-related respiratory failure were matched 1:2 using a nearest-neighbor algorithm. Kaplan-Meier methods with log-rank tests were used to compare long-term survival. A proportional hazards model was used to calculate risk of death. RESULTS: A total of 37,333 LT candidates from all causes were compared with 334 candidates from COVID-19-related respiratory failure. COVID-19 recipients were more likely to be younger (50 vs 57 years, P < .001), male (79% vs 60%, P < .001), require extracorporeal membrane oxygenation (56.3% vs 4.0%, P < .001), and have worse lung function (lung allocation score, 82.4 vs 47.8; P < .001) at transplantation. Subsequently, 227 COVID-19 recipients were matched with 454 controls. Patients who received a transplant for COVID-19 had similar rates of mechanical ventilation, extracorporeal membrane oxygenation, postoperative complications, and functional status at discharge compared with controls. There was no difference in overall survival or risk of death from COVID-19 (hazard ratio, 0.82; 95% CI, 0.45-1.53; P = .54). CONCLUSIONS: Six-month survival for recipients of LT for COVID-19-related respiratory failure was comparable to that of other LT recipients.

Postoperative Liver Failure Secondary to Hypoxic Hepatitis in Double Lung Transplantation: A Case Report.

Hypoxic hepatitis is a diagnosis of exclusion that should be suspected in patients with systemic hypoperfusion risk factors. It has a very high mortality, close to 50%. Although respiratory failure has been described as an etiologic factor for hypoxic hepatitis, cases of liver failure secondary to hypoxic hepatitis after lung transplantation have not been reported. Here we describe the case of a 54-year-old patient who underwent double lung transplantation with intraoperative ECMO and presented postoperative liver failure with a fatal outcome, despite adequate functioning of the lung graft. We describe the clinical presentation, risk factors, intra- and postoperative course, diagnosis, and the importance of pretransplant assessment, along with a review of the literature.

Acquired laryngomalacia as a cause of post-extubation respiratory failure in patient with postoperative seizure and central pontine myelinolysis after craniotomy.

Background : Laryngomalacia is a congenital abnormality of the larynx that commonly occurs in children and rarely in adults. We report the first case of acquired laryngomalacia mainly due to postoperative seizure and central pontine myelinolysis after scheduled craniotomy. Case presentation : A 69-year-old man was admitted to the hospital for elective craniotomy for craniopharyngioma. After the surgery, he developed refractory seizure and required intubation and mechanical ventilation in the intensive-care unit (ICU). After treatment for the seizure, he was extubated. However, immediately after extubation, he developed stridor and respiratory retraction. We performed fiberoptic laryngoscopy and confirmed that the epiglottis had collapsed into the posterior wall of the pharynx during inspiration, which was suspected to be laryngomalacia. He received invasive mechanical ventilation for two days following re-extubation. After the second extubation, he developed stridor again due to acquired laryngomalacia. Six days later, his respiratory condition had worsened, and he received re-intubation and tracheostomy. After ICU discharge, central pontine myelinolysis was diagnosed by magnetic resonance imaging. Conclusions : Adult-onset laryngomalacia is a rare cause of upper airway obstruction but should be considered as a cause of postoperative extubation failure. We should not delay performing fiberoptic laryngoscopy to evaluate this pathology and provide optimal treatment. J. Med. Invest. 69 : 316-319, August, 2022.

[Chronic respiratory failure correction in cicatricial laryngeal and tracheal stenosis using helium-oxygen mixture]. / Korrektsiya khronicheskoi dykhatel'noi nedostatochnosti pri rubtsovykh stenozakh gortani i sheinogo otdela trakhei s ispol'zovaniem gelii-kislorodnoi smesi.

The article provides a review of the literature on the development of chronic respiratory failure in patients with chronic cicatricial stenosis of the larynx and cervical trachea. The authors provide data on the etiology, pathogenetic features of the course of cicatricial stenosis of the larynx and trachea, the reasons for the development of chronic respiratory failure, the effect of hypoxemia on general metabolic processes in the body and on regeneration processes, as well as on methods of their correction and improvement of the postoperative period. The methods of respiratory impact on chronic respiratory failure in these patients are considered, based on the experience of a number of researchers and technical advances in recent years.