ISHLT members have recognized the importance of a consensus statement on the evaluation and management of patients with chronic thromboembolic pulmonary hypertension. The creation of this document ...required multiple steps, including the engagement of the ISHLT councils, approval by the Standards and Guidelines Committee, identification and selection of experts in the field, and the development of 6 working groups. Each working group provided a separate section based on an extensive literature search. These sections were then coalesced into a single document that was circulated to all members of the working groups. Key points were summarized at the end of each section. Due to the limited number of comparative trials in this field, the document was written as a literature review with expert opinion rather than based on level of evidence.
Globally, lung cancer is the leading cause of cancer death. Previous trials demonstrated that low-dose computed tomography lung cancer screening of high-risk individuals can reduce lung cancer ...mortality by 20% or more. Lung cancer screening has been approved by major guidelines in the United States, and over 4,000 sites offer screening. Adoption of lung screening outside the United States has, until recently, been slow. Between June 2017 and May 2019, the Ontario Lung Cancer Screening Pilot successfully recruited 7,768 individuals at high risk identified by using the PLCOm2012noRace lung cancer risk prediction model. In total, 4,451 participants were successfully screened, retained and provided with high-quality follow-up, including appropriate treatment. In the Ontario Lung Cancer Screening Pilot, the lung cancer detection rate and the proportion of early-stage cancers were 2.4% and 79.2%, respectively; serious harms were infrequent; and sensitivity to detect lung cancers was 95.3% or more. With abnormal scans defined as ones leading to diagnostic investigation, specificity was 95.5% (positive predictive value, 35.1%), and adherence to annual recall and early surveillance scans and clinical investigations were high (>85%). The Ontario Lung Cancer Screening Pilot provides insights into how a risk-based organized lung screening program can be implemented in a large, diverse, populous geographic area within a universal healthcare system.
Clinical practice guidelines separately describe radiologic patterns of usual interstitial pneumonia (UIP) and fibrotic hypersensitivity pneumonitis (fHP), without direction on whether or how to ...apply these approaches concurrently within a single patient.
How can we integrate guideline-defined radiologic patterns to diagnose interstitial lung disease (ILD) and what are the pitfalls associated with described patterns that require reassessment in future guidelines?
Patients from the Canadian Registry for Pulmonary Fibrosis underwent detailed reevaluation in standardized multidisciplinary discussion. CT scan features were quantified by chest radiologists masked to clinical data, and guideline-defined patterns were assigned. Clinical data then were provided to the radiologist and an ILD clinician, who jointly determined the leading diagnosis.
Clinical-radiologic diagnosis in 1,593 patients was idiopathic pulmonary fibrosis (IPF) in 26%, fHP in 12%, connective tissue disease-associated ILD (CTD-ILD) in 34%, idiopathic pneumonia with autoimmune features in 12%, and unclassifiable ILD in 10%. Typical and probable UIP patterns corresponded to a diagnosis of IPF in 66% and 57% of patients, respectively. Typical fHP pattern corresponded to an fHP clinical diagnosis in 65% of patients, whereas compatible fHP was nonspecific and associated with CTD-ILD or IPAF in 48% of patients. No pattern ruled out CTD-ILD. Gas trapping affecting > 5% of lung parenchyma on expiratory imaging was an important feature broadly separating compatible and typical fHP from other patterns (sensitivity, 0.77; specificity, 0.91).
An integrated approach to guideline-defined UIP and fHP patterns is feasible and supports > 5% gas trapping as an important branch point. Typical or probable UIP and typical fHP patterns have moderate predictive values for a corresponding diagnosis of IPF and fHP, although occasionally confounded by CTD-ILD; compatible fHP is nonspecific.
Interstitial lung disease (ILD) in connective tissue diseases (CTD) have highly variable morphology. We aimed to identify imaging features and their impact on ILD progression, mortality and ...immunosuppression response.
Patients with CTD-ILD had high-resolution chest computed tomography (HRCT) reviewed by expert radiologists blinded to clinical data for overall imaging pattern (usual interstitial pneumonia UIP; non-specific interstitial pneumonia NSIP; organizing pneumonia OP; fibrotic hypersensitivity pneumonitis fHP; and other). Transplant-free survival and change in percent-predicted forced vital capacity (FVC) were compared using Cox and linear mixed effects models adjusted for age, sex, smoking, and baseline FVC. FVC decline after immunosuppression was compared with pre-treatment.
Of 645 CTD-ILD patients, the frequent CTDs were systemic sclerosis (n = 215), rheumatoid arthritis (n = 127), and inflammatory myopathies (n = 100). NSIP was the most common pattern (54%), followed by UIP (20%), fHP (9%), and OP (5%). Compared with UIP, FVC decline was slower for NSIP (1.1%/year, 95%CI 0.2, 1.9) and OP (3.5%/year, 95%CI 2.0, 4.9), and mortality was lower for NSIP (HR 0.65, 95%CI 0.45, 0.93) and OP (HR 0.18, 95%CI 0.05, 0.57), but higher in fHP (HR 1.58, 95%CI 1.01, 2.40). The extent of fibrosis also predicted FVC decline and mortality. After immunosuppression, FVC decline was slower compared with pre-treatment in NSIP (by 2.1%/year, 95%CI 1.4, 2.8), with no change for UIP or fHP.
Multiple radiologic patterns are possible in CTD-ILD, including a fHP pattern. NSIP and OP were associated with better outcomes and response to immunosuppression, while fHP had worse survival compared with UIP.
Purpose: To compare the accuracy of ultra-low-dose (uLDCT) to standard-of-care low-dose chest CT (LDCT) in the detection of fungal infection in immunocompromised (IC) patients. Method and Materials: ...One hundred IC patients had paired chest CT scans performed with LDCT followed by uLDCT. The images were independently reviewed by three chest radiologists who assessed the image quality (IQ), diagnostic confidence, and detection of major (macro nodules, halo sign, cavitation, consolidation) and minor (4–10 mm nodules, ground-glass opacity) criteria for fungal disease using a five-point Likert score. Discrepant findings were adjudicated by a fourth chest radiologist. Box–whisker plots were used to analyze IQ and diagnostic confidence. Inter-rater reliability was assessed using interclass correlation coefficients (ICCs). The statistical difference between LDCT and uLDCT results was assessed using Wilcoxon paired test. Results: Lung reconstructions had IQ and diagnostic confidence scores (mean ± std) of 4.52 ± 0.47 and 4.63 ± 0.51 for LDCT and 3.85 ± 0.77 and 4.01 ± 0.88 for uLDCT. The images were clinically acceptable except for uLDCT in obese patients (BMI ≥ 30 kg/msup.2), which had an IQ ranking from poor to excellent (scores 1 to 5). The accuracy in detecting major and minor radiological findings with uLDCT was 96% and 84% for all the patients. The inter-rater agreements were either moderate, good, or excellent, with ICC values of 0.51–0.96. There was no significant statistical difference between the uLDCT and LDCT ICC values (p = 0.25). The effective dose for uLDCT was one quarter that of LDCT (CTDIsub.vol = 0.9 mGy vs. 3.7 mGy). Conclusions: Thoracic uLDCT, at a 75% dose reduction, can replace LDCT for the detection of fungal disease in IC patients with BMI < 30.0 kg/msup.2.
We hypothesize that the incidence of screen-detected lung cancer (LC), in participants with previously negative scans, will be highest in the cohort with the highest baseline risk score.
Individuals ...with negative baseline screening results from the Princess Margaret International Early Lung Cancer Action Program before 2009 underwent low-dose computed tomography rescreening from 2015 to 2018. Individuals were contacted in order of descending risk, as determined by the Prostate, Lung, Colorectal, and Ovarian Cancer Screening Trial's PLCOM2012 6-year LC risk-prediction model, and then categorized into three risk cohorts according to their baseline risks. The incidence of LC in each risk cohort was determined and compared. Chi-square testing was used for categorical variables and one-way analysis of variance on ranks was used for continuous variables.
Of the 1261 participants we attempted to re-contact, 359 participants returned for a rescreening scan (mean of 7.6 years between scans). Participants were divided into low (<2%), moderate (≥2% to <3.5%), and high baseline risk (≥3.5%) cohorts. On average, those in the high-risk cohort compared to the moderate- and low-risk cohorts were older (66 years versus 62 and 59 years) and had a greater smoking history (54 pack-years versus 47 and 29 pack-years). The incidence of cancer in the high-risk cohort was significantly higher than in the moderate-risk cohort (11% versus 1.7%, p = 0.002).
There was a significantly higher incidence of LC in the high-risk cohort than in the moderate-risk cohort. The cut-point between the high- and moderate-risk was determined to be greater than or equal to 3.5% of the 6-year baseline risk.
RATIONALE: Appropriate management of low-dose computed tomography (LDCT) screening detected lung nodules will have significant implications for health care resource utilization and minimizing harm ...from radiation exposure related to imaging studies, invasive procedures and clinically significant distress.
We aimed to: provide a practical, evidence-based best practice framework for healthcare professionals (HP) to manage screening LDCT detected lung nodules and identify areas that require future studies.
The Canadian Partnership Against Cancer and Pan-Canadian Lung Cancer Screening Network (PLCSN) undertook a scientific review of the assessment and management of screening LDCT detected lung nodules. Key messages were derived by consensus through a series of stakeholder meetings to obtain full consensus.
MAIN RESULTS: 1) A high standard of LDCT image quality is of importance to determine nodule type, size and growth; 2) Personalized approach to manage screen detected lung nodules based on malignancy probability is a promising approach to decrease resource utilization and minimize risk of screening; 3) Radiologist reports should provide specific guidance for expert and non-expert health care providers regarding the most appropriate next step with a separate lay-language report for screenees tailored to the general result category along with a recommended next step; 4) Diagnostic work-up in centers with multidisciplinary specialized expertise in minimally invasive sampling of pulmonary nodules is recommended to achieve the best possible yield and lowest complications rate; and 5) Common quality indicators in lung nodule management protocols across health jurisdictions provide the opportunity to evaluate and refine management protocols.
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