Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline.

BACKGROUND: Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations regarding four specific questions related to the diagnosis of LAM and management of pneumothoraces in patients with LAM. METHODS: Systematic reviews were performed and then discussed by a multidisciplinary panel. For each intervention, the panel considered its confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences, patient values and preferences, cost, and feasibility. Evidence-based recommendations were then formulated, written, and graded using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach. RESULTS: For women who have cystic changes on high-resolution computed tomography of the chest characteristic of LAM, but who have no additional confirmatory features of LAM (i.e., clinical, radiologic, or serologic), the guideline panel made conditional recommendations against making a clinical diagnosis of LAM on the basis of the high-resolution computed tomography findings alone and for considering transbronchial lung biopsy as a diagnostic tool. The guideline panel also made conditional recommendations for offering pleurodesis after an initial pneumothorax rather than postponing the procedure until the first recurrence and against pleurodesis being used as a reason to exclude patients from lung transplantation. CONCLUSIONS: Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of LAM. METHODS: Systematic reviews were performed to summarize evidence pertinent to our questions. The evidence was summarized and discussed by a multidisciplinary panel. Evidence-based recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach. RESULTS: After considering the panel's confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were formulated for or against specific interventions. These included recommendations for sirolimus treatment and vascular endothelial growth factor D testing and recommendations against doxycycline and hormonal therapy. CONCLUSIONS: Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

Management of the Potential Organ Donor in the ICU: Society of Critical Care Medicine/American College of Chest Physicians/Association of Organ Procurement Organizations Consensus Statement.

This document was developed through the collaborative efforts of the Society of Critical Care Medicine, the American College of Chest Physicians, and the Association of Organ Procurement Organizations. Under the auspices of these societies, a multidisciplinary, multi-institutional task force was convened, incorporating expertise in critical care medicine, organ donor management, and transplantation. Members of the task force were divided into 13 subcommittees, each focused on one of the following general or organ-specific areas: death determination using neurologic criteria, donation after circulatory death determination, authorization process, general contraindications to donation, hemodynamic management, endocrine dysfunction and hormone replacement therapy, pediatric donor management, cardiac donation, lung donation, liver donation, kidney donation, small bowel donation, and pancreas donation. Subcommittees were charged with generating a series of management-related questions related to their topic. For each question, subcommittees provided a summary of relevant literature and specific recommendations. The specific recommendations were approved by all members of the task force and then assembled into a complete document. Because the available literature was overwhelmingly comprised of observational studies and case series, representing low-quality evidence, a decision was made that the document would assume the form of a consensus statement rather than a formally graded guideline. The goal of this document is to provide critical care practitioners with essential information and practical recommendations related to management of the potential organ donor, based on the available literature and expert consensus.

Lung transplantation for obstructive lung diseases.

Chronic obstructive pulmonary disease (COPD) represents one of the most common indications for lung transplantation, accounting for approximately one third of all procedures performed worldwide to date. Despite this extensive experience, questions remain about the appropriate timing of transplantation in the natural history of COPD, the optimal procedure to perform, and the survival benefit achieved. Less commonly encountered obstructive lung disorders for which transplantation is occasionally performed include emphysema due to α-1-antitrypsin deficiency, lymphangioleiomyomatosis, and Langerhans cell histiocytosis. Like COPD, the application of transplantation to these rare disorders also poses several questions. This article explores issues that arise when lung transplantation is utilized for treatment of both common and uncommon obstructive lung disorders.

Lung transplantation.

First performed in 1963, lung transplantation is approaching the half-century mark. With more than 32,000 procedures having been performed worldwide, lung transplantation has become the standard of care for select patients with advanced lung diseases of various nonmalignant etiologies. Indications for transplantation have broadened over the years, and selection criteria have become less restrictive. A relatively scarce donor pool limits wider application of this therapy, but this is being addressed in part through relaxation of donor selection criteria, donor management protocols that preserve and optimize lung function, and development of ex vivo perfusion techniques to "recondition" suboptimal organs. Bilateral lung transplantation has become the procedure of choice for most indications, although its preferential use in patients with idiopathic pulmonary fibrosis remains controversial. Post-transplantation survival has steadily improved, but significant constraints on long-term survival persist as evidenced by a median survival rate that currently stands at 5.7 years. This has brought into focus the question of whether and for whom transplantation actually confers a survival advantage, a question that in the absence of randomized trials can only be answered with statistical modeling. Primary graft dysfunction, infection, and bronchiolitis obliterans syndrome are common complications encountered by the lung transplant recipient and are major impediments to long-term survival. This review provides an overview of the current status of lung transplantation, highlighting both the many advances that have taken place and the challenges that remain.

Providing Outpatient Telehealth Services in the United States: Before and During Coronavirus Disease 2019.

Before coronavirus disease 2019 (COVID-19), telehealth evaluation and management (E/M) services were not widely used in the United States and often were restricted to rural areas or locations with poor access to care. Most Medicare beneficiaries could not receive telehealth services in their homes. In response to the COVID-19 pandemic, Medicare, Medicaid, and commercial insurers relaxed restrictions on both coverage and reimbursement of telehealth services. These changes, together with the need for social distancing, transformed the delivery of outpatient E/M services through an increase in telehealth use. In some cases, the transition from in-person outpatient care to telehealth occurred overnight. Billing and claim submission for telehealth services is complicated; has changed over the course of the pandemic; and varies with each insurance carrier, making telehealth adoption burdensome. Despite these challenges, telehealth is beneficial for health-care providers and patients. Without additional legislation at the federal and state levels, it is likely that telehealth use will continue to decline after the COVID-19 public health emergency.

Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation.

Tens of thousands of patients with advanced lung diseases may be eligible to be considered as potential candidates for lung transplant around the world each year. The timing of referral, evaluation, determination of candidacy, and listing of candidates continues to pose challenges and even ethical dilemmas. To address these challenges, the International Society for Heart and Lung Transplantation appointed an international group of members to review the literature, to consider recent advances in the management of advanced lung diseases, and to update prior consensus documents on the selection of lung transplant candidates. The purpose of this updated consensus document is to assist providers throughout the world who are caring for patients with pulmonary disease to identify potential candidates for lung transplant, to optimize the timing of the referral of these patients to lung transplant centers, and to provide transplant centers with a framework for evaluating and selecting candidates. In addition to addressing general considerations and providing disease specific recommendations for referral and listing, this updated consensus document includes an ethical framework, a recognition of the variability in acceptance of risk between transplant centers, and establishes a system to account for how a combination of risk factors may be taken into consideration in candidate selection for lung transplantation.

Severe venous and lymphatic obstruction after single-chamber pacemaker implantation in a patient with chest radiation therapy.

A 73-year-old woman with a history of paroxysmal atrial fibrillation, sinus node dysfunction, bilateral breast cancer, and extensive chest radiation developed progressive edema, dyspnea, and recurrent pleural effusions soon after single-chamber pacemaker implantation. Thoracentesis yielded a diagnosis of chylothorax, and progressive refractory anasarca developed. A computed tomography angiogram suggested obstruction of the superior vena cava and left subclavian vein despite outpatient therapeutic anticoagulation. Autopsy confirmed venous thrombosis, along with mediastinal fibrosis. The presumed etiology of the chylothorax and anasarca was obstruction of the atretic central venous structures following pacemaker implantation, critically impairing the already tenuous venous and lymphatic drainage. (PACE 2010; 520-524).

Lung transplantation for chronic obstructive pulmonary disease: special considerations.

Since the introduction of lung transplantation nearly half a century ago, more procedures have been performed for chronic obstructive pulmonary disease (COPD) than for any other single indication. Because COPD tends to progress slowly and long-term survival is possible even in the advanced stages, the time at which transplantation should be offered remains unclear. Current recommendations rely on use of the BODE index to provide guidance on listing. Although both single lung transplantation (SLT) and bilateral lung transplantation (BLT) are suitable procedures for the COPD population, BLT has become the preferred procedure, particularly for patients under age 60, for whom it appears to offer superior survival and functional benefits. Whether lung transplantation truly extends survival for patients with COPD is uncertain. Preliminary answers have come from use of survivorship models that suggest a subset of COPD patients do derive a survival benefit, and that the size of this subgroup can be enhanced by selecting patients with extremely severe airflow obstruction and preferentially utilizing BLT. Those undergoing SLT are uniquely at risk for complications related to the remaining native lung-bronchogenic carcinoma and progressive hyperinflation-which are fortunately rare.

Multisociety task force recommendations of competencies in Pulmonary and Critical Care Medicine.

RATIONALE: Numerous accrediting organizations are calling for competency-based medical education that would help define specific specialties and serve as a foundation for ongoing assessment throughout a practitioner's career. Pulmonary Medicine and Critical Care Medicine are two distinct subspecialties, yet many individual physicians have expertise in both because of overlapping content. Establishing specific competencies for these subspecialties identifies educational goals for trainees and guides practitioners through their lifelong learning. OBJECTIVES: To define specific competencies for graduates of fellowships in Pulmonary Medicine and Internal Medicine-based Critical Care. METHODS: A Task Force composed of representatives from key stakeholder societies convened to identify and define specific competencies for both disciplines. Beginning with a detailed list of existing competencies from diverse sources, the Task Force categorized each item into one of six core competency headings. Each individual item was reviewed by committee members individually, in group meetings, and conference calls. Nominal group methods were used for most items to retain the views and opinions of the minority perspective. Controversial items underwent additional whole group discussions with iterative modified-Delphi techniques. Consensus was ultimately determined by a simple majority vote. MEASUREMENTS AND MAIN RESULTS: The Task Force identified and defined 327 specific competencies for Internal Medicine-based Critical Care and 276 for Pulmonary Medicine, each with a designation as either: (1) relevant, but competency is not essential or (2) competency essential to the specialty. CONCLUSIONS: Specific competencies in Pulmonary and Critical Care Medicine can be identified and defined using a multisociety collaborative approach. These recommendations serve as a starting point and set the stage for future modification to facilitate maximum quality of care as the specialties evolve.

Risk factors for early primary graft dysfunction after lung transplantation: a registry study.

BACKGROUND: Primary graft dysfunction (PGD) is a leading cause of early morbidity and mortality in lung transplantation. We sought to identify risk factors for PGD using the United Network for Organ Sharing/International Society for Heart and Lung Transplant (UNOS/ISHLT) Registry. METHODS: A total of 6984 lung transplants between 1994 and 2002 were available for analysis. Potential risk factors were tested for association with PGD and multivariable logistic regression was applied to adjust for confounding. RESULTS: The overall incidence of PGD was 10.7% (95% CI 9.9-11.4). In multivariable analyses, factors independently associated with PGD were donor age >45 yr (p < 0.001); donor head trauma (p = 0.03); recipient body mass index >25 kg/m(2) (p = 0.005); recipient female gender (p = 0.001); use of Eurocollins preservation solution (p = 0.001); single lung transplant (p = 0.005); increased ischemic time (p < 0.001); and elevated recipient pulmonary artery systolic pressure at transplant (p < 0.001). Recipient transplant diagnosis was strongly associated with PGD, with primary or secondary pulmonary hypertension (p < 0.001 for both), and idiopathic (p < 0.001) or secondary pulmonary fibrosis (p = 0.011) as significant and independent risk factors for PGD. CONCLUSIONS: Risk factors for PGD in the UNOS/ISHLT registry are consistent with prior smaller studies. Recipient, donor, and therapy variables are independently associated with PGD, as defined in a large registry.

Does lung transplantation confer a survival benefit?

PURPOSE OF REVIEW: There are reasonably compelling data documenting the beneficial impact of lung transplantation on functional status, hemodynamics, and quality of life. Demonstration of a survival benefit has been more problematic, largely due to significant constraints on long-term survival. This article reviews the current literature addressing the question of whether transplantation is truly a life-extending procedure. RECENT FINDINGS: Because randomized trials are nonexistent, the question has been approached by comparing observed posttransplant survival to wait list survival, or by simulating survival with and without transplantation by statistical modeling. Studies uniformly suggest that transplantation extends survival for patients with idiopathic pulmonary fibrosis. For patients with chronic obstructive pulmonary disease, studies are conflicting and suggest that survival benefit is largely restricted to a subset of patients with extremely severe airflow obstruction who undergo bilateral lung transplantation. Cystic fibrosis (CF) adults with a predicted 5-year survival of less than 50%, and without Burkholderia cepacia or arthropathy appear most likely to realize a survival benefit. Whether transplantation extends survival for pediatric CF patients remains controversial. SUMMARY: Questions about the survival benefit of lung transplantation linger. In the absence of definitive answers, it may be most appropriate to view transplant as a procedure that enhances the quality but not necessarily the duration of life.

Noninfectious Pulmonary Complications of Liver, Heart, and Kidney Transplantation: An Update.

Despite significant advances in surgical techniques, perioperative care, and immunosuppressive therapy, solid organ transplantation still carries considerable risk of complications. Pulmonary complications, in particular, are a major cause of morbidity and mortality. Although infectious complications prevail, the lungs are also vulnerable to a variety of noninfectious complications related to the transplant surgery and adverse effects of the immunosuppressive regimen. This article focuses on noninfectious pulmonary complications associated with the 3 most commonly performed solid organ transplant procedures: liver, kidney, and heart.

Low prevalence of prior streptococcus pneumoniae vaccination among potential lung transplant candidates.

INTRODUCTION: Appropriate vaccination status in the pre-solid organ transplant period is critically important. METHODS: To determine if lung transplant candidates are adequately vaccinated for Streptococcus pneumoniae, a cross-sectional study was performed. Electronic records of patients referred to our institution for transplantation evaluation between July 2002 and January 2004 were reviewed. RESULTS: Only 98 of 157 patients (62.4%; 95% confidence interval [CI], 54.8 to 70.1%) evaluated for lung transplantation reported prior receipt of S pneumoniae vaccine. COPD was the only factor significantly associated with vaccination. Patients with COPD were more likely to have received vaccination compared to patients with other diagnoses (odds ratio, 4.66; 95% CI, 2.26 to 9.60). CONCLUSIONS: S pneumoniae vaccination rates among potential lung transplant candidates fall substantially short of current recommendations for universal immunization. Transplant programs should thoroughly review vaccination status and develop strategies to ensure that candidates receive all appropriate vaccines before transplantation.

Birt-Hogg-Dubé Syndrome.

Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the Folliculin gene and is characterized by the formation of fibrofolliculomas, early onset renal cancers, pulmonary cysts, and spontaneous pneumothoraces. The exact pathogenesis of tumor and lung cyst formation in BHD remains unclear. There is great phenotypic variability in the clinical features of BHD, and patients can present with any combination of skin, pulmonary, or renal findings. More than 80% of adult patients with BHD have pulmonary cysts on high-resolution computed tomography scan of the chest.

Complications of Lung Transplantation: A Roentgenographic Perspective.

Lung transplantation is now an established treatment for a broad spectrum of end-stage pulmonary diseases. According to the International Society for Heart and Lung Transplantation Registry, more than 50,000 lung transplants have been performed worldwide, with nearly 11,000 lung transplant recipients alive in the United States. With the increasing application of lung transplantation, pulmonologists must be cognizant of common complications unique to the postlung transplant period and the associated radiologic findings. The aim of this review is to describe clinical manifestations and prototypical radiographic features of both common and rare complications encountered in lung transplant recipients.

Impact of primary graft failure on outcomes following lung transplantation.

STUDY OBJECTIVES: Primary graft failure (PGF) is a severe acute lung injury syndrome that occurs following lung transplantation. We compared the clinical outcomes of patients who developed PGF with those who did not. METHODS: We conducted a retrospective cohort study including 255 consecutive lung transplant procedures. PGF was defined as (1) diffuse alveolar opacities developing within 72 h of transplantation, (2) an arterial partial pressure of oxygen/fraction of inspired oxygen (PaO2/FiO2) ratio of < 200 beyond 48 h postoperatively, and (3) no other secondary cause of graft dysfunction. PGF was tested for acceptance with 30-day and all-cause hospital mortality rates, overall survival, hospital length of stay (HLOS), duration of mechanical ventilation, and best 6-min walk test (6MWT) distance achieved within 12 months. SETTING: Academic medical center. RESULTS: The overall incidence of PGF was 11.8% (95% confidence interval [CI], 7.9 to 15.9%). The all-cause mortality rate at 30 days was 63.3% in patients with PGF and 8.8% in patients without PGF (relative risk [RR], 7.15; 95% CI, 4.34 to 11.80%; p < 0.001). A total of 73.3% of patients with PGF died during hospitalization vs 14.2% of patients without PGF (RR, 5.18%; 95% CI, 3.51 to 7.63; p < 0.001). The median HLOS in 30-day survivors was 47 days in patients with PGF vs 15 days in those without PGF (p < 0.001), and the mean duration of mechanical ventilation was 15 days in patients with PGF vs 1 day in those without PGF (p < 0.001). By 12 months, a total of 28.5% of survivors with PGF achieved a normal age-appropriate 6MWT distance vs 71.4% of survivors without PGF at 12 months (p = 0.014). The median best 6MWT distance achieved within the first 12 months was 1,196 feet in patients with PGF vs 1,546 feet in those without PGF (p = 0.009). CONCLUSIONS: PGF has a significant impact on mortality, HLOS, and duration of mechanical ventilation following lung transplantation. Survivors of PGF have a protracted recovery with impaired physical function up to 1 year following transplantation.