Predischarge death or lung transplantation in tracheostomy and ventilator dependent grade 3 bronchopulmonary dysplasia.

Background: Premature infants surviving beyond a postmenstrual age (PMA) of 36 weeks with severe or grade 3 bronchopulmonary dysplasia (sBPD) have significant predischarge mortality. The in-hospital mortality for BPD supported by invasive mechanical ventilation beyond 36 weeks PMA is not well described. The role of lung transplantation in treating severe BPD is uncertain.

The cascade screening in heritable forms of pulmonary arterial hypertension.

Heritable pulmonary artery hypertension (HPAH) is an increasingly recognized type of pulmonary arterial hypertension, in both pediatric and adult population. Intrinsic to hereditary disease, screening for genetic mutations within families is an important component of diagnosis and understanding burden of disease. Recently, consensus guidelines are published for genetic screening in PAH.

Differential donor management of pediatric vs adult organ donors and potential impact on pediatric lung transplantation.

Background: Despite clinical progress over time, a shortage of suitable donor organs continues to limit solid organ transplantation around the world. Lungs are the organs most likely to be assessed as unsuitable during donor management among all transplantable organs. Although the number of lung transplants performed in children is limited, death on the wait list remains a barrier to transplant success for many potential transplant candidates.

Improved Outcomes for Infants and Young Children Undergoing Lung Transplantation at Three Years of Age and Younger.

Since its inception, older children and adolescents have predominated in pediatric lung transplantation. Most pediatric lung transplant programs around the world have transplanted few infants and young children. Early mortality after lung transplantation and inadequate donor organs have been perceived as limitations for success in lung transplantation at this age.

Surgical and Cardiac Catheterization Outcomes of Scimitar Syndrome Patients: A Three Decade Single-Center Experience.

Scimitar syndrome (SS) is a rare congenital condition which includes partial anomalous pulmonary venous return (PAPVR) and a variable degree of pulmonary hypoplasia. We describe the clinical features, therapeutic approach and outcomes of patients who underwent cardiac catheterization and/or surgical repair of the scimitar vein at a single institution in the United States. This retrospective cohort study included all patients with SS who underwent scimitar vein surgical repair or cardiac catheterization from October 1989 through August 2021 in a tertiary care center.

Cystic fibrosis lung transplant recipients 10 years of age or younger: Predisposing factors for end-stage disease.

Background: The largest age group among children and adolescents referred for lung transplantation for cystic fibrosis (CF) have been those in the pubertal or postpubertal age range. However, over 100 younger patients with CF have undergone lung transplantation over the last three decades in the United States.

Methods: We performed a retrospective review of our experience with 18 children with CF who underwent lung transplantation in our center before the age of 11 years and compared them to our older CF lung transplant recipients and our larger CF Center population.

Application of lung volume reduction surgery for a child with filamin A (FLNA) mutations.

Diffuse lung disease in early childhood due to mutations in the filamin A gene has been recently reported. Clinical outcomes vary among individuals indicating variability in phenotype but a substantial proportion of reported cases in early life have ended up in death or lung transplantation. We recently encountered a school-aged child in whom the diagnosis of a filamin A mutation was delayed and the natural history of emphysematous lung disease was altered by serial lung volume reduction surgeries.

Diagnosis and treatment of cryptococcal osteomyelitis in a pediatric lung transplant patient.

Background: Asymptomatic pulmonary nodules may appear at any point after lung transplantation. The differential diagnosis is broad and includes serious life-threatening disease entities.

Methods: A retrospective case report of a single patient who developed a pulmonary nodule after lung transplantation.

Outcomes of COVID-19 infection in pediatric pulmonary hypertension: A single-center experience.

Background: The global COVID-19 pandemic was particularly concerning for the pediatric pulmonary hypertension (PH) population due to immature immune systems and developmental comorbidities. This study aims to describe a single-center experience of pediatric PH patients diagnosed with COVID-19 disease.

Methods: A retrospective cohort study of all pediatric patients followed by the PH Center at Texas Children's Hospital diagnosed with COVID-19 infection from April 2020 to February 2021.

Lung transplant referrals for individuals with cystic fibrosis: A pediatric perspective on the cystic fibrosis foundation consensus guidelines.

Lung transplant referral guidelines for individuals with cystic fibrosis (CF) were published recently. Most of the recommendations focus on the specific indications and barriers to transplantation in adults with CF. Although the number of children with CF and end-stage lung disease continues to decrease, the specific issues related to pediatric patients merit further elucidation.

Influence of early extubation on post-operative outcomes after pediatric lung transplantation.

Lung transplantation has become an accepted therapeutic option for a select group of children with end-stage lung disease. We evaluated the impact of early extubation in a pediatric lung transplant population and its post-operative outcomes. Single-center retrospective study.

Pulmonary growth abnormalities as etiologies for pediatric pulmonary hypertension.

Pulmonary growth abnormality (PGA) is a common type of diffuse lung disease in infants. Although the histologic and radiographic features of PGA have been described in the literature in varying detail, the clinical spectrum of disease has not. The array of case series and case reports has led to a clinical picture that could be confusing to clinicians.

Infections Within the First Month After Pediatric Lung Transplantation: Epidemiology and Impact on Outcomes.

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.

Management of severe pulmonary Langerhans cell histiocytosis in children.

Patients with pulmonary Langerhans cell histiocytosis (LCH) typically have a benign course but may have extensive cystic lung disease with rare life-threatening complications including multiple and recurrent pneumothoraces and respiratory failure. We report seven severely affected pediatric patients treated with chemotherapy, aggressive chest tube management, and pleurodesis of whom five survived. Patients with extraordinary amounts of pulmonary cystic disease and multiple pneumothoraces due to LCH can have remarkable, curative outcomes with early recognition, optimal LCH-directed therapy, and supportive care.

Oral treprostinil use in children: a multicenter, observational experience.

Pulmonary arterial hypertension is a progressive, incurable disease that occurs in adults and children alike. Therapeutic options for children are limited and infrequently described, including newer agents such as treprostinil, an oral prostanoid. Herein, we describe the pooled pediatric experience in 28 patients from four pediatric pulmonary hypertension programs over two years.

Clinico-radiologic features of pleuroparenchymal fibroelastosis in children.

Background: Pleuroparenchymal fibroelastosis (PPFE) may be underdiagnosed clinically and radiographically in children with a remote history of cancer, leading to a delay in care and unnecessary lung biopsies.

Objective: To describe the characteristic clinical and radiologic findings of PPFE in a cohort of children to facilitate recognition and noninvasive diagnosis.

Materials And Methods: Clinical presentation, history of chemotherapy or radiation therapy, lung or bone marrow transplantation, and lung function testing and outcome were retrospectively extracted from the electronic medical records of eight children treated at our institution's pulmonary medicine clinic with histopathology confirmation of PPFE from 2008 to 2018.

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis-Associated Liver Disease.

Approximately 5%-10% of patients with cystic fibrosis (CF) will develop advanced liver disease with portal hypertension, representing the third leading cause of death among patients with CF. Cystic fibrosis with advanced liver disease and portal hypertension (CFLD) represents the most significant risk to patient mortality, second only to pulmonary or lung transplant complications in patients with CF. Currently, there is no medical therapy to treat or reverse CFLD.

Risk factors for infection after pediatric lung transplantation.

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.

Predictive value of oxygenation index for outcomes in left-sided congenital diaphragmatic hernia.

Background & Objectives: Congenital Diaphragmatic Hernia (CDH) is associated with significant morbidity and mortality. This study compares the efficacy of the highest oxygenation index in the first 48 h (HiOI) versus current prenatal indices to predict survival and morbidity.

Methods: Medical records of 50 prenatally diagnosed, isolated, left-sided CDH patients treated from January 2011 to April 2016 were reviewed.

Hospital Readmissions in Children with Pulmonary Hypertension: A Multi-Institutional Analysis.

Objective: To assess the rate of and risk factors for 30-day hospital readmission in children with pulmonary hypertension.

Study Design: The Pediatric Health Information System database was analyzed for patients ≤18 years old with pulmonary hypertension (International Classification of Diseases, Ninth Revision, diagnosis codes of 416.0, 416.

Waiting list outcomes in pediatric lung transplantation: Poor results for children listed in adult transplant programs.

Background: Low case volume has been associated with lower survival after pediatric lung transplantation. Our aim was to analyze waitlist outcomes among pediatric lung transplant centers in the USA.

Methods: We studied a cohort of 1,139 pediatric candidates listed in the Organ Procurement and Transplantation Network for lung transplantation between 2002 and 2014.