Ex Vivo Lung Perfusion: A Review of Current and Future Application in Lung Transplantation.

The number of waitlisted lung transplant candidates exceeds the availability of donor organs. Barriers to utilization of donor lungs include suboptimal lung allograft function, long ischemic times due to geographical distance between donor and recipient, and a wide array of other logistical and medical challenges. Ex vivo lung perfusion (EVLP) is a modality that allows donor lungs to be evaluated in a closed circuit outside of the body and extends lung donor assessment prior to final acceptance for transplantation.

Lung Disease-Related Pulmonary Hypertension.

Patients with advanced lung disease can develop pulmonary hypertension and succumb to right ventricular failure/cor pulmonale. Patients with pulmonary hypertension owing to chronic lung disease, or World Health Organization group 3 pulmonary hypertension, are more limited and carry a high risk of mortality. Adjunctive therapies remain the cornerstones of treatment.

Inhaled Nitric Oxide via High-Flow Nasal Cannula in Patients with Acute Respiratory Failure Related to COVID-19.

Introduction: Limited evidence exists regarding use of inhaled nitric oxide (iNO) in spontaneously breathing patients. We evaluated the effectiveness of continuous iNO via high-flow nasal cannula (HFNC) in COVID-19 respiratory failure.

Methods: We performed a multicenter cohort study of patients with respiratory failure from COVID-19 managed with HFNC.

Incidence and prognostic significance of pleural effusions in pulmonary arterial hypertension.

It has been suggested pleural effusions may develop in right heart failure in the absence of left heart disease. The incidence and prognostic significance of pleural effusions in pulmonary arterial hypertension is uncertain. Patients with pulmonary arterial hypertension followed at our tertiary care center were reviewed.

Development and Validation of a Clinical Diagnostic Scoring System for the Diagnosis of Idiopathic Pulmonary Fibrosis.

Interpreting the radiologic data in conjunction with an objective clinical score could help to harmonize idiopathic pulmonary fibrosis (IPF) diagnosis and improve accuracy. We sought to establish and validate a multivariable objective scoring model based on clinical parameters by stratifying the risk of patients having IPF diagnosed versus having other forms of interstitial lung disease (ILD) diagnosis. A clinical score was derived from review of patients evaluated at the Inova Fairfax ILD Program and validated in three distinct cohorts.

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes.

Research Question: There is no widely accepted grading system for IPF disease severity, although physiologic impairment based on pulmonary function testing is frequently employed. We sought to describe clinical and functional characteristics as well as outcomes of patients with severe physiologic impairment.

Patients And Methods: IPF patients with severe physiologic impairment defined by FVC ≤ 50% and/or DLco ≤ 30% predicted evaluated in the Inova Advanced Lung Disease Program between 2011 and 2019 were included.

Outcomes of mechanically ventilated patients with COVID-19 associated respiratory failure.

Purpose: The outcomes of patients requiring invasive mechanical ventilation for COVID-19 remain poorly defined. We sought to determine clinical characteristics and outcomes of patients with COVID-19 managed with invasive mechanical ventilation in an appropriately resourced US health care system.

Methods: Outcomes of COVID-19 infected patients requiring mechanical ventilation treated within the Inova Health System between March 5, 2020 and April 26, 2020 were evaluated through an electronic medical record review.

HRCT evaluation of patients with interstitial lung disease: comparison of the 2018 and 2011 diagnostic guidelines.

Background And Aims: Chest high-resolution computed tomography (HRCT) is the central diagnostic tool in discerning idiopathic pulmonary fibrosis (IPF) from other interstitial lung disease (ILDs). In 2018, new guidelines were published and the nomenclature for HRCT interpretation was changed. We sought to evaluate how clinicians' interpretation would change based on reading HRCTs under the framework of the old new categorization.

Association Between Anticoagulation and Survival in Interstitial Lung Disease: An Analysis of the Pulmonary Fibrosis Foundation Patient Registry.

Background: Aberrations in the coagulation system have been implicated in the pathogenesis of interstitial lung disease (ILD). Anticoagulants have been proposed as a potential therapy in ILD; however, a randomized controlled trial examining warfarin as a treatment for IPF was terminated early due to increased death rates. This has led some to speculate that warfarin specifically may be harmful in ILD, and use of direct oral anticoagulants (DOACs) could result in superior outcomes.

Surgical Lung Biopsy for Interstitial Lung Disease. Safety and Feasibility at a Tertiary Referral Center.

Video-assisted thoracoscopic surgery (VATS) remains the gold standard for interstitial lung disease (ILD) characterization when histology is deemed necessary. There is diminishing use of VATS owing to increased reliance on high-resolution computed tomographic patterns, as well as concerns regarding the potential morbidity and mortality of the procedure. The goal of this study was to evaluate the safety and tolerability of VATS among a broad group of patients with ILD referred to a tertiary care center.

A 48-Year-Old South African Woman with Rheumatoid Arthritis and Lung Nodules.

We present the case of a 48-year-old South African woman with no smoking history, and seropositive rheumatoid arthritis diagnosed in 2001. She was treated with chloroquine (150 mg, 4 times per week) and methotrexate (30 mg weekly) with well-controlled symptoms until 2015, when she developed a disease flare. Her treatment regimen was changed to leflunomide (20 mg daily) monotherapy with subsequent symptom control.

Impaired Myofibroblast Dedifferentiation Contributes to Nonresolving Fibrosis in Aging.

Idiopathic pulmonary fibrosis (IPF) is a fatal age-associated disease with no cure. Although IPF is widely regarded as a disease of aging, the cellular mechanisms that contribute to this age-associated predilection remain elusive. In this study, we sought to evaluate the consequences of senescence on myofibroblast cell fate and fibrotic responses to lung injury in the context of aging.

Single-center experience with use of letermovir for CMV prophylaxis or treatment in thoracic organ transplant recipients.

Background: Cytomegalovirus (CMV) infection is common in thoracic organ transplant recipients. Valganciclovir and ganciclovir are used for both prophylaxis and treatment of this infection, but intolerance and treatment failure are common. Letermovir has been demonstrated to reduce the risk of CMV infection when used for prophylaxis in allogeneic hematopoietic cell transplantation.

Extracorporeal Membrane Oxygenation as a Bridge to Initial Medical Therapy in a Patient With Decompensated Pulmonary Arterial Hypertension Presenting With Biventricular Failure.

Extracorporeal membrane oxygenation (ECMO) can be used as a bridge to medical therapy in decompensated right heart failure due to pulmonary arterial hypertension (PAH). A middle-aged man with previously undiagnosed pulmonary hypertension was successfully bridged to intravenous prostanoid therapy with venoarterial ECMO support after presenting with cardiogenic shock and hypoxemic respiratory failure. Although the patient initially had biventricular failure, PAH was suspected due to underlying mixed connective tissue disease and disproportionate right ventricular dysfunction.

Connective tissue disease-associated interstitial lung disease and outcomes after hospitalization: A cohort study.

Background: The impact of hospitalization on patient outcomes is increasingly recognized and considered in the prognostication of many pulmonary disorders. We sought to evaluate the impact of hospitalization on survival in connective tissue disease-interstitial lung disease (CTD-ILD) patients.

Methods: A chart review of patients with CTD-ILD followed at a tertiary care center was performed.

Novel management strategies for idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) treatment was revolutionized by the advent of two novel antifibrotics, nintedanib and pirfenidone. However, neither is a panacea and other agents are still sorely needed. This review presents on-going efforts to improve outcomes for patients with IPF by targeting novel pharmacologic pathways, improving comorbidity management, and aiming for improved quality of life.

Critical Care of the Adult Patient With Cystic Fibrosis.

Cystic fibrosis (CF) is a multiorgan, genetic disease resulting from dysfunction of the CF transmembrane conductance regulator. The primary clinical manifestations are bronchiectasis, chronic pulmonary infections with recurrent exacerbations, and pancreatic insufficiency. Advances in CF care have led to improved survival, with more than one-half of those affected being adults.

Mucormycosis pulmonary abscess, containment in a patient with uncontrolled diabetes mellitus.

A 56-year-old Hispanic male with solo risk factor of uncontrolled diabetes mellitus presented with recurrent haemoptysis. Initial concern was for malignancy with postobstructive pneumonia; however, invasive testing and biopsy confirmed infectious mass of fungal aetiology requiring surgical resection followed by a prolonged course of anti-fungal therapy. Discussion centred on approach to, progression of and course of action in the management of pulmonary abscess due to mucormycosis.

Dysregulated Nox4 ubiquitination contributes to redox imbalance and age-related severity of acute lung injury.

Acute respiratory distress syndrome (ARDS) is a devastating critical illness disproportionately affecting the elderly population, with both higher incidence and mortality. The integrity of the lung endothelial cell (EC) monolayer is critical for preservation of lung function. However, mechanisms mediating EC barrier regulation in the context of aging remain unclear.

Hemoperitoneum as a Consequence of Colonoscopy.

Hemoperitoneum without evidence of organ damage is a rare complication of colonoscopy. It is most frequently seen in association with splenic rupture due to traction on the splenocolic ligament. In our case, we present a 48-year-old cirrhotic man who developed peritoneal bleeding during a diagnostic colonoscopy for iron deficiency anemia.