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Bronchoalveolar lavage (BAL) is a key clinical and research tool in lung transplantation (LTx). However, BAL collection and processing are not standardized across LTx centers. This International Society for Heart and Lung Transplantation-supported consensus document on BAL standardization aims to clarify definitions and propose common approaches to improve clinical and research practice standards. The following 9 areas are covered: (1) bronchoscopy procedure and BAL collection, (2) sample handling, (3) sample processing for microbiology, (4) cytology, (5) research, (6) microbiome, (7) sample inventory/tracking, (8) donor bronchoscopy, and (9) pediatric considerations. This consensus document aims to harmonize clinical and research practices for BAL collection and processing in LTx. The overarching goal is to enhance standardization and multicenter collaboration within the international LTx community and enable improvement and development of new BAL-based diagnostics. ispartof: JOURNAL OF HEART AND LUNG TRANSPLANTATION vol:39 issue:11 pages:1171-1190 ispartof: location:United States status: published

The purpose of this study is to evaluate the association between type-1 laryngeal clefts and pathogenic bacterial growth in the lower airway in children.Retrospective chart review.A retrospective chart review was conducted for all children who underwent direct laryngoscopy, flexible bronchoscopy with bronchoalveolar lavage (BAL), and esophagogastroduodenoscopy, under a single anesthetic event from 2015 until 2018 at an academic tertiary referral center. Type-1 laryngeal clefts were diagnosed as an interarytenoid depth at or below the level of the vocal folds, on direct laryngoscopy, via palpation by a fellowship-trained pediatric otolaryngologist. Pathogenic bacterial growth in the lower airway was defined as presence of BAL culture growth of nonrespiratory flora.A total of 217 patients were identified. Type-1 laryngeal cleft was significantly associated with chronic cough (P = .0016) and cough with feeds (P .0001). However, an abnormal video fluoroscopic swallow study was not found to be significantly associated with type-1 laryngeal cleft (P = .92) or pathogenic bacterial growth in the lower airway (P = 0.19). Overall, 122 (56%) patients were diagnosed with type-1 laryngeal cleft, 75 (35%) had pathogenic bacterial growth in the lower airway and 50 (23%) had both type-1 laryngeal cleft and pathogenic bacterial growth in the lower airway. Type-1 laryngeal cleft was significantly associated with pathogenic bacterial growth in the lower airway on both univariate analysis (P = .0307) and multivariate analysis (P = .0298, odds ratio 1.922, 95% confidence interval 1.066-3.467).Children with type-1 laryngeal clefts are at higher risk of having pathogenic bacterial growth in the lower airway.4 Laryngoscope, 132:1825-1828, 2022.

Children with End Stage Lung Disease (ESLD) are part of the growing population of individuals with life-limiting conditions of childhood. These patients present with a diverse set of pulmonary, cardiovascular, neuromuscular, and developmental conditions. This paper first examines five cases of children with cystic fibrosis, bronchopulmonary dysplasia, neuromuscular disease, pulmonary hypertension, and lung transplantation from Texas Children’s Hospital. We discuss the expected clinical course of each condition, then review the integration of primary and specialized palliative care into the management of each diagnosis. This paper then reviews the management of two children with end staged lung disease at Hospital Civil de Guadalajara, providing an additional perspective for approaching palliative care in low-income countries.

Background Despite successes in lung transplantation, with infection as the leading cause of death in the first year following lung transplantation, there remains a lag in survival compared with other solid organ transplants. Infections that occur early after transplantation may impact short- and long-term outcomes in pediatric lung transplant recipients (LTRs). Methods We performed a retrospective review of pediatric LTRs at a large quaternary-care hospital from January 2009 to March 2016 to evaluate both epidemiologic features of infection in the first 30 days post-transplantation and mortality outcomes. The 30 days were divided into early (0–7 days) and late (8–30 days) periods. Results Among the 98 LTRs, there were 51 episodes of infections. Cystic fibrosis (CF) was associated with early bacterial infections (P = .004) while non-CF was associated with late viral (P = .02) infections. Infection after transplantation was associated with worse survival by Kaplan-Meier analysis (P value log rank test = .007). Viral infection in the late period was significantly associated with 3-year mortality after multivariable analysis (P = .02). Conclusions Infections in pediatric LTRs were frequent in the first 30 days after transplant, despite perioperative antimicrobial coverage. The association of 3-year mortality with late viral infections suggests a possible important role in post-transplant lung physiology and graft function. Understanding the epidemiology of early post-lung transplant infections can help guide post-operative management and interventions to reduce their incidence and the early- and long-term impact in this population.

Purpose Pleuroparenchymal fibroelastosis (PPFE) is a rare, progressive, restrictive lung disease characterized by hypercarbic respiratory failure. In pediatrics, it has been described in patients with a history of malignancy who have received a bone marrow transplant, chemotherapy, or radiotherapy. It is characterized by pleural thickening, fibrosis, subpleural elastosis, and intraalveolar collagen deposits. Survival is poor and the only therapy is lung transplantation. Little is known about the outcomes after lung transplant for PPFE in pediatric patients. Methods A single-center retrospective chart review of patients that underwent bilateral lung transplant for PPFE between 2010 and 2020 was done. Descriptive and outcomes data were collected and compared to overall survival after lung transplant for our center. Results Nine patients were transplanted for PPFE in the 10-year period. Six were male. The average age at referral for transplant was 12.5 (7 - 16) years. The average at transplant was 13.8 (9 - 16) years. All patients had a history of malignancy; the most common was neuroblastoma (6/9) followed by 1 rhabdomyosarcoma, 1 acute lymphoid leukemia, and 1 acute myeloid leukemia. Seven patients received radiation therapy (non thoracic). Five patients had a history of autologous bone marrow transplantation. The average time from chemotherapy to referral to lung transplant was 9 years (6 - 12). Six patients required donor lung resections. One required delayed chest closure for 48 hours. Currently, 4 patients are alive, 4 deceased, and 1 lost to follow-up due to the transfer of care. The longest surviving patient is 3.7 years post transplant. The median survival after transplantation of the 4 deceased patients was 2.2 (1.3 - 6.3) years. Median survival post lung transplant for any reason is 6.6 years at our center. Conclusion Our experience with PPFE has been reflective of what has been published in the literature. All patients had a previous malignancy. Our limited data indicate worse outcomes compared to our overall center survival. Six patients required donor lung resizing due to the severely restrictive and small thoracic cavity size, resulting in increased morbidity. There is a need for further research to determine if lung transplantation for PPFE is an indicated therapy given the apparent risk of poor outcomes and higher morbidity.

Introduction Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. In PVOD the pulmonary venous vessels are obliterated resulting in obstructive pulmonary vascular disease, increase in pulmonary vascular resistance, right heart failure and death. Pulmonary hypertension is the most common reason for lung transplant in infants. Lung transplant is the only therapy for PVOD. Prolonged intubation, deconditioning and extra corporeal life support are associated with poor outcomes after lung transplant. We present a case of PVOD in an infant who had successful lung transplant using a novel veno-arterial extracorporeal membrane oxygenation (VA ECMO) configuration. Case Report A previously healthy 15-month-old male presented with cough and tachypnea. Cardiomegaly and pulmonary edema were seen on chest x-ray. Echocardiogram revealed severe pulmonary hypertension. Cardiac catheterization study and chest computed tomography revealed PVOD. He was unable to be extubated after cardiac catheterization and was transferred to our institution for lung transplant evaluation. He had severely depressed right ventricular function, requiring vasopressors to maintain hemodynamic stability. Respiratory status was tenuous, requiring sedation and paralysis. He was evaluated and listed for lung transplant. The patient was centrally cannulated for VA ECMO using cannulae for a ventricular assist device (Berlin ExCor) that were exteriorized on the abdomen. “Venous” inflow cannula was placed in the main pulmonary artery to provide afterload reduction for the right ventricle. After starting VA ECMO, vasopressor support, respiratory support, paralysis and sedation were weaned. He was woken up, extubated; and intensive physical and occupational rehabilitation began. He regained strength, was able to sit, stand and take steps. He also regained his milestones, was able to babble, wave and eat simple foods. The patient remained on VA ECMO for 61 days until he successfully underwent lung transplantation. Summary Our case highlights the successful use of a novel VA ECMO configuration as a bridge to lung transplant in an infant with PVOD. To our knowledge this is the youngest patient to undergo awake VA ECMO reported. The use of a unique cannulation allowed for rehabilitation while on VA ECMO. Though challenging, VA ECMO remains an option as a bridge to lung transplant in infants with PVOD.

Chronic pulmonary aspiration presents a challenging problem to medical providers. Flexible bronchoscopy, coupled with bronchoalveolar lavage, can provide diagnostic clues for those patients suspected of aspirating. The gross appearance of the lungs, as viewed endoscopically, can demonstrate anatomic abnormalities or inflammation. Several biomarkers in bronchoalveolar lavage fluid can provide more evidence, although no definitive test currently exists.

Objective To describe a series of patients with pathogenic variants in FLNA and progressive lung disease necessitating lung transplantation. Study Design We conducted a retrospective chart review of 6 female infants with heterozygous presumed loss-of-function pathogenic variants in FLNA whose initial presentation was early and progressive respiratory failure. Results Each patient received lung transplantation at an average age of 11 months (range, 5-15 months). All patients had pulmonary arterial hypertension and chronic respiratory failure requiring tracheostomy and escalating levels of ventilator support before transplantation. All 6 patients survived initial lung transplantation; however, 1 patient died after a subsequent heart-lung transplant. The remaining 5 patients are living unrestricted lives on chronic immunosuppression at most recent follow-up (range, 19 months to 11.3 years post-transplantation). However, in all patients, severe ascending aortic dilation has been observed with aortic regurgitation. Conclusions Respiratory failure secondary to progressive obstructive lung disease during infancy may be the presenting phenotype of FLNA -associated periventricular nodular heterotopia. We describe a cohort of patients with progressive respiratory failure related to a pathogenic variant in FLNA and present lung transplantation as a viable therapeutic option for this group of patients.

Although infection is the leading cause of death in the first year following pediatric lung transplantation, there are limited data on risk factors for early infection. Sepsis remains under-recognized and under-reported in the early post-operative period for lung transplant recipients (LTR). We evaluated the incidence of infection and sepsis, and identified risk factors for infection in the early post-operative period in pediatric LTRs. A retrospective review of medical records of LTRs at a large quaternary-care hospital from January 2009 to March 2016 was conducted. Microbiology results on days 0-7 after transplant were obtained. Sepsis was defined using the 2005 International Pediatric Consensus Conferencecriteria. Risk factors included history of recipient and donor infection, history of multi-drug resistant (MDR) infection, nutritional status, and surgical times. Among the 98 LTRs, there were 22 (22%) with post-operative infection. Prolonged donor ischemic time ≥7 hours, cardiopulmonary bypass(CPB) time ≥340 minutes, history of MDR infection and diagnosis of cystic fibrosis were significantly associated with infection. With multivariable regression analysis, only prolonged donor ischemic time remained significant (OR 4.4, 95% CI: 1.34-14.48). Further research is needed to determine whether processes to reduce donor ischemic time could result in decreased post-transplant morbidity.

Long-term success in pediatric lung transplantation is limited by infection and bronchiolitis obliterans syndrome (BOS). The bilateral sequential lung transplantation (BSLT) technique may result in airway ischemia leading to bronchial stenosis, dehiscence, or loss of small airways. En bloc lung transplant (EBLT) with bronchial artery revascularization (BAR) minimizes airway ischemia, thus promoting superior airway healing. BAR also allows for safe tracheal anastomosis, circumventing the need for bilateral bronchial anastomoses in small children.This was a retrospective review of bilateral transplantations from 2005 to 2014. Both techniques were used in parallel. Redo and multiorgan transplants were excluded.There were 119 recipients comprising 88 BSLTs and 31 EBLTs. Follow-up time was 3 years (interquartile range, 1-5 years). Donor ischemic and cardiopulmonary bypass times were not different between techniques (p = 0.48 and p = 0.18, respectively). Degree of graft dysfunction and cellular rejection scores were not different (p = 0.83 and p = 0.93, respectively). There were 3 hospital deaths after BSLT and 2 after EBLT (p = 0.60). Overall survival was 61% for the BSLT group and 77% for the EBLT group (p = 0.54). Freedom from BOS was 71% in the BSLT group and 94% in the EBLT group (p = 0.08). On routine bronchoscopy, 57% BSLT and 16% EBLT patients had 1 or more airway ischemic findings (p0.0001). Multivariate analysis showed BSLT was associated with higher ischemic injury (relative risk, 2.86; 95 confidence interval, 1.3-6.5; p = 0.01) and non-airway complications (relative risk, 4.62; 95% confidence interval, 1.1-20.2; p = 0.04) but not airway reinterventions (p = 0.07). Airway dehiscence occurred in 3 BSLT patients.Pediatric EBLT with BAR can be safely performed without increasing operative or graft ischemic times. Airway ischemia and non-airway complications were significantly reduced when BAR was combined with tracheal anastomosis, potentially diminishing morbidity caused by anastomotic healing complications.

In this review, we discuss the recent advances in our understanding of the cause, pathogenesis, presentation, diagnosis, treatment, and prognosis of interstitial lung disease (ILD) in children.The classification of ILD syndromes in children greater than 2 years of age is based largely on adult classification schemes. In children less than 2 years of age, classification has been developed and evaluated pathologically. Entities can be categorized into developmental disorders, growth abnormalities, and surfactant dysfunction disorders based on pathologic findings. Two distinctive entities, neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis, present early in life with characteristic findings. These two disorders appear to have a favorable prognosis. Diagnosis of ILD syndromes is based on the summation of history and physical findings and both noninvasive and invasive studies. Newer approaches are being evaluated to decrease the need for lung biopsy.Children's interstitial lung diseases are rare diffuse lung diseases resulting from a variety of pathogenic processes that include genetic factors, association with systemic disease processes, and inflammatory or fibrotic responses to stimuli. There are unique causes and presentations seen in infancy. Diagnosis in these disorders is made by the summation of clinical, radiologic, and pathologic findings.