A first look at the reliability, validity and responsiveness of L-PF-35 dyspnea domain scores in fibrotic hypersensitivity pneumonitis.

BACKGROUND: Dyspnea impairs quality of life (QOL) in patients with fibrotic hypersensitivity pneumonitis (FHP). The Living with Pulmonary Fibrosis questionnaire (L-PF) assesses symptoms, their impacts and PF-related QOL in patients with any form of PF. Its scores have not undergone validation analyses in an FHP cohort. METHODS: We used data from the Pirfenidone in FHP trial to examine reliability, validity and responsiveness of the L-PF-35 Dyspnea domain score (Dyspnea) and to estimate its meaningful within-patient change (MWPC) threshold for worsening. Lack of suitable anchors precluded conducting analyses for other L-PF-35 scores. RESULTS: At baseline, Dyspnea's internal consistency (Cronbach's coefficient alpha) was 0.85; there were significant correlations with all four anchors (University of California San Diego Shortness of Breath Questionnaire scores r = 0.81, St. George's Activity domain score r = 0.82, percent predicted forced vital capacity r = 0.37, and percent predicted diffusing capacity of the lung for carbon monoxide r = 0.37). Dyspnea was significantly different between anchor subgroups (e.g., lowest percent predicted forced vital capacity (FVC%) vs. highest, 33.5 ± 18.5 vs. 11.1 ± 9.8, p = 0.01). There were significant correlations between changes in Dyspnea and changes in anchor scores at all trial time points. Longitudinal models further confirmed responsiveness. The MWPC threshold estimate for worsening was 6.6 points (range 5-8). CONCLUSION: The L-PF-35 Dyspnea domain appears to possess acceptable psychometric properties for assessing dyspnea in patients with FHP. Because instrument validation is never accomplished with one study, additional research is needed to build on the foundation these analyses provide. TRIAL REGISTRATION: The data for the analyses presented in this manuscript were generated in a trial registered on ClinicalTrials.gov; the identifier was NCT02958917.

Weight loss with GLP-1 analogues in preparation for transplantation.

This case describes a woman in her 20s with a 6-month history of progressive exertional dyspnoea and cough. Examination revealed hypoxia on room air, sinus tachycardia, finger clubbing and bibasal inspiratory crackles. Inflammatory markers were mildly elevated and empirical antimicrobial therapy was commenced. A multidisciplinary discussion consensus diagnosis of acute interstitial pneumonitis was made based on the findings of high-resolution CT of the chest, macrophage predominant bronchoalveolar lavage cell differential and surgical lung biopsy. There was clinical and radiological deterioration despite glucocorticoids and antifibrotic therapy. A body mass index of 37.5 kg/m2 precluded her from lung transplant assessment and consideration. Following consultation with the weight management service, she was commenced on glucagon-like peptide 1 (GLP-1) analogue therapy. She had a remarkable response within 6 months, was listed for lung transplantation, and within 18 months of her initial presentation, a double lung transplantation was performed.

A 49-Year-Old Man With Fever and Dyspnea After Endobronchial Ultrasound-Guided Transbronchial Fine Needle Aspiration Biopsy.

CASE PRESENTATION: A 49-year-old man, a farmer, had been experiencing coughing, phlegm, and difficulty breathing for 2 months. He underwent a CT scan at a local hospital that showed a mediastinal mass. Bronchoscopy showed no obstruction in the tracheal lumen, and an endobronchial ultrasound-guided transbronchial fine needle aspiration (EBUS-TBNA) biopsy was performed on the mediastinal mass. The cytologic smear of the mediastinal mass showed a few atypical epithelial cells; the possibility of a tumor could not be ruled out. The patient visited our thoracic surgery outpatient department; based on the advice of the thoracic surgeon, the patient underwent another endobronchial ultrasound-guided transbronchial fine needle aspiration biopsy of the mediastinal mass 4 days before this admission. The patient went home and waited for the results. Two days later, the patient experienced a fever and palpitations accompanied by chills, yellow phlegm, and orthopnea. The patient visited our ED, underwent tracheal intubation, and was admitted to our ICU. The patient had had occasional coughing and phlegm for the past 10 years, which were not taken seriously or investigated. The patient does not smoke or drink alcohol, and there is no history of cancer in the family.

Accuracy of urgency allocation in patients with shortness of breath calling out-of-hours primary care: a cross-sectional study.

BACKGROUND: In out-of-hours primary care (OHS-PC), semi-automatic decision support tools are often used during telephone triage. In the Netherlands, the Netherlands Triage Standard (NTS) is used. The NTS is mainly expert-based and evidence on the diagnostic accuracy of the NTS' urgency allocation against clinically relevant outcomes for patients calling with shortness of breath (SOB) is lacking. METHODS: We included data from adults (≥18 years) who contacted two large Dutch OHS-PC centres for SOB between 1 September 2020 and 31 August 2021 and whose follow-up data about final diagnosis could be retrieved from their own general practitioner (GP). The diagnostic accuracy (sensitivity and specificity with corresponding 95% confidence intervals (CI)) of the NTS' urgency levels (high (U1/U2) versus low (U3/U4/U5) and 'final' urgency levels (including overruling of the urgency by triage nurses or supervising general practitioners (GPs)) was determined with life-threatening events (LTEs) as the reference. LTEs included, amongst others, acute coronary syndrome, pulmonary embolism, acute heart failure and severe pneumonia. RESULTS: Out of 2012 eligible triage calls, we could include 1833 adults with SOB who called the OHS-PC, mean age 53.3 (SD 21.5) years, 55.5% female, and 16.6% showed to have had a LTE. Most often severe COVID-19 infection (6.0%), acute heart failure (2.6%), severe COPD exacerbation (2.1%) or severe pneumonia (1.9%). The NTS urgency level had a sensitivity of 0.56 (95% CI 0.50-0.61) and specificity of 0.61 (95% CI 0.58-0.63). Overruling of the NTS' urgency allocation by triage nurses and/or supervising GPs did not impact sensitivity (0.56 vs. 0.54, p = 0.458) but slightly improved specificity (0.61 vs. 0.65, p < 0.001). CONCLUSIONS: The semi-automatic decision support tool NTS performs poorly with respect to safety (sensitivity) and efficiency (specificity) of urgency allocation in adults calling Dutch OHS-PC with SOB. There is room for improvement of telephone triage in patients calling OHS-PC with SOB. TRIAL REGISTRATION: The Netherlands Trial Register, number: NL9682 .

A retrospective study of breathlessness supportive therapy on chronic refractory breathlessness in a palliative care unit.

INTRODUCTION: Chronic refractory breathlessness is a debilitating symptom which negatively affects quality of life with profound impact on physical and psychosocial functioning of patients and/or carers. Multidisciplinary based interventions which focus on non-pharmacological approach have shown to be effective. We developed a breathlessness intervention service called breathlessness supportive therapy (BST) in a palliative care unit with limited resources. The aim is to evaluate the feasibility of developing a BST service and to study the characteristics and outcome of patients with chronic refractory breathlessness. MATERIALS AND METHODS: This is a retrospective study of patients with chronic refractory breathlessness and Modified Medical Research Council (mMRC) dyspnoea scale grade ≥ 2 who attended the BST clinic over 1 year period. BST consists of two clinic sessions 2 weeks apart. Data was retrieved from patients' medical notes and analysis done using Microsoft Excel. RESULTS: A total of 21 patients were identified. Median age was 69 years with 52% of females. 72% had non-malignant diagnoses. Median Charlson's Comorbidity Index score was 6.5. Median mMRC dyspnoea scale was 3. 47.6% had long term oxygen usage. Median Australian Karnofsky Performance Scale (AKPS) was 65 and the median baseline breathlessness visual analogue scale (VAS) was 2. 62% completed two sessions, the remaining 38% completed only one session. Mean time from BST intervention to death was 18.26 weeks, median was 22 weeks. 72% died at home, whilst 28% died in the hospital. All the patients scored 4 (somewhat agree) and 5 (strongly agree) on the overall feedback score. CONCLUSIONS: Development of a breathlessness intervention service is feasible in a resource limited setting and generally accepted by most patients. More research and prospective studies are needed to evaluate the effectiveness of BST in the future.

Integrative Assessment of Cardiopulmonary Fitness Using Cardiopulmonary Exercise Test With Supine Bicycle Echocardiography in Patients Presenting Dyspnea.

BACKGROUND: Cardiopulmonary exercise test (CPET) with supine bicycle echocardiography (SBE) enables comprehensive physiologic assessment during exercise. We characterized cardiopulmonary fitness by integrating CPET-SBE parameters and evaluated its prognostic value in patients presenting with dyspnea. METHODS AND RESULTS: We retrospectively reviewed 473 consecutive patients who underwent CPET-SBE for dyspnea evaluation. A dimensionality reduction process was applied, transforming 24 clinical and CPET-SBE parameters into a 2-dimensional feature map, followed by patient clustering based on the data distribution. Clinical and exercise features were compared among the clusters in addition to the 5-year risk of clinical outcome (a composite of cardiovascular death and heart failure hospitalization). Maximum exercise effort (R >1) was achieved in 95% of cases. Through dimensionality reduction, 3 patient clusters were derived: Group 1 (n=157), 2 (n=104), and 3 (n=212). Median age and female proportion increased from Group 1 to 2, and 3, although resting echocardiography parameters showed no significant abnormalities among the groups. There was a worsening trend in the exercise response from Group 1 to 2 and 3, including left ventricular diastolic function, oxygen consumption, and ventilatory efficiency. During follow-up (median 6.0 [1.6-10.4] years), clinical outcome increased from Group 1 to 2 and 3 (5-year rate 3.7% versus 7.0% versus 13.0%, respectively; log-rank P=0.02), with higher risk in Group 2 (hazard ratio, 1.94 [95% CI, 0.52-7.22]) and Group 3 (3.92 [1.34-11.42]) compared with Group 1. CONCLUSIONS: Comprehensive evaluation using CPET-SBE can reveal distinct characteristics of cardiopulmonary fitness in patients presenting with dyspnea, potentially enhancing outcome prediction.

Exertional breathlessness related to medical conditions in middle-aged people: the population-based SCAPIS study of more than 25,000 men and women.

BACKGROUND: Breathlessness is common in the population and can be related to a range of medical conditions. We aimed to evaluate the burden of breathlessness related to different medical conditions in a middle-aged population. METHODS: Cross-sectional analysis of the population-based Swedish CArdioPulmonary bioImage Study of adults aged 50-64 years. Breathlessness (modified Medical Research Council [mMRC] ≥ 2) was evaluated in relation to self-reported symptoms, stress, depression; physician-diagnosed conditions; measured body mass index (BMI), spirometry, venous haemoglobin concentration, coronary artery calcification and stenosis [computer tomography (CT) angiography], and pulmonary emphysema (high-resolution CT). For each condition, the prevalence and breathlessness population attributable fraction (PAF) were calculated, overall and by sex, smoking history, and presence/absence of self-reported cardiorespiratory disease. RESULTS: We included 25,948 people aged 57.5 ± [SD] 4.4; 51% women; 37% former and 12% current smokers; 43% overweight (BMI 25.0-29.9), 21% obese (BMI ≥ 30); 25% with respiratory disease, 14% depression, 9% cardiac disease, and 3% anemia. Breathlessness was present in 3.7%. Medical conditions most strongly related to the breathlessness prevalence were (PAF 95%CI): overweight and obesity (59.6-66.0%), stress (31.6-76.8%), respiratory disease (20.1-37.1%), depression (17.1-26.6%), cardiac disease (6.3-12.7%), anemia (0.8-3.3%), and peripheral arterial disease (0.3-0.8%). Stress was the main factor in women and current smokers. CONCLUSION: Breathlessness mainly relates to overweight/obesity and stress and to a lesser extent to comorbidities like respiratory, depressive, and cardiac disorders among middle-aged people in a high-income setting-supporting the importance of lifestyle interventions to reduce the burden of breathlessness in the population.

Chronotropic incompetence demonstrated with cardiopulmonary exercise test.

In his case report, a 74-year-old physically fit man was evaluated repeatedly for several years in the cardiology department due to dyspnoea on exertion (DOE). Several standard cardiac and pulmonary tests were performed but did not provide sufficient cause for the DOE. Lastly, the patient was evaluated with a cardiopulmonary exercise test (CPET) with simultaneous in- and expiratory gas sampling. The test revealed a low aerobic capacity due to chronotropic incompetence (CI), thus explaining the DOE. Subsequently, the patient was treated with a rate-responsive pacemaker. CPET-is an ideal test for diagnosing CI.

Is the 2016 ESC diagnostic algorithm useful for assessing the prevalence of chronic heart failure in population-based studies?

BACKGROUND: Chronic heart failure (CHF) is a major healthcare problem. However, there are no epidemiological studies assessing the prevalence of CHF in the general population with diagnosis based on algorithms recommended for clinical practice. AIM: The aim of the HF-Pomorskie survey was to assess the prevalence of three basic components of the 2016 ESC diagnostic algorithm for CHF (symptoms, N-terminal pro B-type natriuretic peptide [NT-proBNP], and abnormalities on echocardiography) and to determine whether this algorithm may be applicable to studies in general population samples. METHODS: The study was performed in a representative sample of 313 adults (170 women and 143 men) aged between 20 and 90 years (mean 55.2 years [15.3]) in Northern Poland. A questionnaire to determine New York Heart Association [NYHA] class, laboratory tests including NT-proBNP, as well as transthoracic echocardiography and spirometry examinations were performed in all subjects. RESULTS: Dyspnea (NYHA class II-IV) was reported by 13.7% of recruited participants. Dyspnea and elevated levels of NT-proBNP (>125 pg/ml) were found in 7.7% of all examined subjects, while dyspnea, elevated NT-proBNP levels accompanied by systolic or diastolic abnormalities on echocardiography occurred in 4.8%. In the group without dyspnea (86.3% of all examined subjects), every sixth subject had an elevated level of NT-proBNP. On the other hand, 5.8% of studied subjects reported a previous diagnosis of CHF, which was confirmed using the current ESC algorithm in 78% of them. CONCLUSIONS: The prevalence of CHF assessed by the 2016 ESC diagnostic algorithm in the representative sample of adults was equal to 4.8%. The clinical algorithm for the diagnosis of CHF is fully applicable to the representative surveys in the general population. However, due to logistic and economic factors, echocardiography examination and NT-proBNP determination can be limited to patients reporting dyspnea or previous diagnosis of CHF.

Dyspnea-Related Dimensions And Self-Efficacy: Associations With Well-Being in Advanced Lung Cancer.

CONTEXT: Dyspnea is a complex, multidimensional symptom comprising sensory-perceptual, affective, and functional domains that commonly persists in patients with lung cancer and impairs mental health and quality of life (QOL). However, data are lacking on how dyspnea's dimensions or self-efficacy to manage dyspnea are associated with patient outcomes. OBJECTIVES: To assess the associations of dyspnea dimensions (dyspnea-related sensory-perceptual experience, affective distress, and functional impact) and dyspnea self-efficacy with depression, anxiety, and QOL in patients with advanced lung cancer reporting dyspnea. METHODS: We conducted a secondary analysis of baseline clinical trial data testing a supportive care intervention for dyspnea. Patients with advanced lung cancer reporting at least moderate dyspnea (≥2 on the Modified Medical Research Council Dyspnea Scale) self-reported dyspnea and patient outcome measures. Hierarchical regressions tested the associations of the dyspnea dimensions with depressive and anxiety symptoms (Hospital Anxiety and Depression Scale) and QOL (Functional Assessment of Cancer Therapy-Lung) while adjusting for variables known to affect these outcomes. RESULTS: The sensory-perceptual experience of dyspnea (effort) was associated with worse depressive symptoms (b = 0.21, P < 0.01) and QOL (b = -0.53, P = 0.01). Dyspnea self-efficacy was associated with improved depressive (b = -1.26, P < 0.01) and anxiety symptoms (b = -1.72, P < 0.01) and QOL (b = 3.66, P < 0.01). The affective and functional dimensions of dyspnea were not associated with the patient outcomes in the final models. CONCLUSIONS: Dyspnea-related sensory-perceptual experience and self-efficacy were associated with mental health and QOL outcomes in patients with lung cancer. Examining the individual contributions of dyspnea's multiple dimensions provides a nuanced understanding of its patient impact.

Cardiopulmonary exercise testing in younger patients with persistent dyspnea following acute, outpatient COVID-19 infection.

Studies using cardiopulmonary exercise testing (CPET) to evaluate persistent dyspnea following infection with COVID-19 have focused on older patients with co-morbid diseases who are post-hospitalization. Less attention has been given to younger patients with post-COVID-19 dyspnea treated as outpatients for their acute infection. We sought to determine causes of persistent dyspnea in younger patients recovering from acute COVID-19 infection that did not require hospitalization. We collected data on all post-COVID-19 patients who underwent CPET in our clinic in the calendar year 2021. Data on cardiac function and respiratory response were abstracted, and diagnoses were assigned using established criteria. CPET data on 45 patients (238.3 ± 124 days post-test positivity) with a median age of 27.0 (22.0-40.0) were available for analysis. All but two (95.6%) were active-duty service members. The group showed substantial loss of aerobic capacity-average VO2 peak (L/min) was 84.2 ± 23% predicted and 25 (55.2%) were below the threshold for normal. Spirometry, diffusion capacity, high-resolution computed tomography, and echocardiogram were largely normal and were not correlated with VO2 peak. The two most common contributors to dyspnea and exercise limitation following comprehensive evaluation were deconditioning and dysfunctional breathing (DB). Younger active-duty military patients with persistent dyspnea following outpatient COVID-19 infection show a substantial reduction in aerobic capacity that is not driven by structural cardiopulmonary disease. Deconditioning and DB breathing are common contributors to their exercise limitation. The chronicity and severity of symptoms accompanied by DB could be consistent with an underlying myopathy in some patients, a disorder that cannot be differentiated from deconditioning using non-invasive CPET.

Feasibility of Constant Work Rate Testing to Detect Exercise-Induced Laryngeal Obstruction.

INTRODUCTION: Exercise-induced laryngeal obstruction (EILO) is a condition in which laryngeal structures inappropriately obstruct the upper airway during exercise. The standard diagnostic test for EILO is the continuous laryngoscopy during exercise (CLE) test, usually performed with an incremental work rate protocol regardless of the nature of the triggering event. Typically, laryngeal obstruction occurs only briefly at the end of an incremental test, near peak work capacity. We aimed to investigate constant work rate (CWR) protocols for CLE testing to expand diagnostic test modalities and improve the understanding of EILO. METHODS: In this prospective, self-controlled feasibility study, 10 patients with EILO performed both an incremental and a CWR CLE test at 70%, 80%, and 90% of maximal exercise capacity. Laryngoscopic video data were recorded and compared, and we evaluated the ability of CWR to reproduce the symptoms and laryngeal obstruction seen in incremental testing. RESULTS: In 70%-90% of cases, CWR testing induced at least the same severity of obstruction as incremental testing and CLE scores remained comparable across test modalities. CWR at 70% allowed observation of laryngeal obstruction for a significantly longer duration than in incremental testing (158 s; 95% confidence interval, 25-291 s; P = 0.027). Dyspnea intensity appeared higher during CWR testing compared with incremental testing. CONCLUSIONS: Submaximal CWR CLE testing is feasible and able to induce EILO equivalent to the standard incremental CLE test. This is the first step toward tailored CLE exercise protocols, and further studies are now needed to establish the utility of CWR in clinical and research settings.

Epiglottic retroversion as a cause of upper airway obstruction: A case report.

RATIONALE: Epiglottic retroversion is the abnormal movement of the epiglottis to the rima glottis, resulting in blockage of inspiratory airflow. Acute upper airway obstruction caused by epiglottic retroversion can lead to sudden respiratory failure. Epiglottic retroversion has occasionally been reported in horses and dogs; however it is extremely rare in humans. Herein, we report a case of epiglottic retroversion causing recurrent upper airway obstruction in human. PATIENT CONCERNS: We present the case of a 74-year-old man who was diagnosed with epiglottic retroversion without evidence of epiglottis. The patient presented with recurrent episodes of abnormal breathing sounds and dyspnea. Inspiratory stridor was evident whenever the patient experienced dyspnea. DIAGNOSIS: Epiglottic retroversion was diagnosed as the cause of upper airway obstruction using fiber-optic bronchoscopy. INTERVENTIONS: The patient underwent tracheostomy to prevent acute respiratory failure because the recurrent episodes of stridor and dyspnea did not improve. OUTCOMES: The episodic dyspnea and oxygen desaturation did not relapse after tracheostomy and he could be discharged home. LESSONS: This case highlights the importance of considering epiglottic retroversion as a cause of acute upper airway obstruction.