Exercise-induced Dyspnea Research Articles

Exertional Dyspnea; Just an Untrained Child? Two Case Reports Analyzing the Role of Lung Function Testing

Background: Dyspnea is a common symptom that afflicts many patients with pulmonary disease and may be the primary manifestation of not only lung disease but also myocardial dysfunction, anemia, neuromuscular disorders, obesity, etc. Dyspnea can be induced by physical activity, which is referred to as exercise-induced dyspnea (EID). It can be caused by various etiologies, sometimes concomitant. In pediatrics, the three most common causes of exertional dyspnea are exercise-induced bronchoconstriction, inducible laryngeal obstruction, and being physically untrained Case presentation: We report two cases of adolescents who developed EID and their management approach. The first patient had an inducible laryngeal obstruction (EILO), while the second had an exercise-induced bronchoconstriction (EIB). Discussion: The diagnosis of EIB is based on clinical symptoms (e.g., exercise-related symptoms of dyspnea, cough, or wheezing) and lung function testing (LFT). This test shows a reversible airflow limitation in response to exercise. A decrease in FEV1 ≥ 10% is considered positive. A major goal is to ensure that patients with EIB continue physical activity. Therapy is based on non-pharmacologic and pharmacologic measures. Conclusion: Our aim is to add our experience to the available knowledge on the diagnosis of EID. In conclusion, when faced with a child with exertional dyspnea, before declaring that he is not trained, it is always necessary to collect an accurate medical history, examination and carry out LFT, excluding pathologies of the upper and lower respiratory tract, such as EILO and EIB.

Prevalence and characterisation of exercise-induced laryngeal obstruction in patients with exercise-induced dyspnoea.

The prevalence of exercise-induced laryngeal obstruction is largely unknown. This study aimed to evaluate the prevalence of this condition in a selected study population of patients with exercise-induced dyspnoea. A retrospective analysis was conducted of demographic data, co-morbidities, medication, symptoms, performance level of sporting activities, continuous laryngoscopy exercise test results and subsequent treatment. Data from 184 patients were analysed. The overall prevalence of exercise-induced laryngeal obstruction in the study population was 40 per cent, and the highest prevalence was among females aged under 18 years (61 per cent). However, a high prevalence among males aged under 18 years (50 per cent) and among adults regardless of gender (34 per cent) was also found. The prevalence of exercise-induced laryngeal obstruction is clinically relevant regardless of age and gender. Clinicians are encouraged to consider exercise-induced laryngeal obstruction as a possible diagnosis in patients suffering from exercise-induced respiratory symptoms. No single characteristic that can distinguish exercise-induced laryngeal obstruction from other similar conditions was identified.

Clinical and Prognostic Implications of Cardiopulmonary Exercise Stress Echocardiography in Asymptomatic Degenerative Mitral Regurgitation

The current guidelines recommend intervention in severe degenerative mitral regurgitation (MR) in symptomatic patients or asymptomatic patients with left ventricular dilatation or dysfunction. The insidious onset of symptoms may mean that patients do not report their symptoms. The role of systematic exercise testing for symptoms in MR is not clearly defined. A total of 97 patients with moderate to severe asymptomatic MR underwent exercise echocardiography combined with cardiopulmonary exercise testing. The predictors of exercise-induced dyspnea, symptom-free survival, and mitral valve intervention were identified. A total of 18 patients (19%) developed limiting dyspnea on exercise. Spontaneous symptom-free survival at 24months was significantly higher in those without exercise-induced symptoms than those with exercise-induced symptoms, p <0.0001. The only independent predictors of spontaneous symptoms at 2years were effective regurgitant orifice area (odds ratio 27.45, 95% confidence interval [CI] 1.43 to 528.40, p=0.03) and exercise-induced symptoms (odds ratio 11.56, 95% CI 1.71 to 78.09, p=0.01). The only independent predictor of surgery was indexed left ventricular systolic volumes (odds ratio 1.17, 95% CI 1.04 to 1.30, p=0.006). Where only the patients who underwent surgery due to symptoms were included, the only independent predictor was exercise-induced symptoms (odds ratio 13.94, 95% CI 1.39 to 140.27, p=0.025). In conclusion, in patients with primary asymptomatic degenerative MR, 1/5 develop revealed symptoms during exercise. This predicts a subsequent development of spontaneous symptoms and mitral valve intervention due to symptoms.

Clinical and prognostic implications of cardiopulmonary exercise stress echocardiography in asymptomatic degenerative mitral regurgitation

Abstract Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): The Saudi Arabian Cultural Bureau. Background Current guidelines recommend intervention in severe degenerative mitral regurgitation (MR) in symptomatic patients or asymptomatic patients with left ventricular dilatation or dysfunction. The insidious onset of symptoms may mean patients do not report symptoms. The role of systematic exercise testing for symptoms in MR is not clearly defined. Methods 97 patients with moderate to severe, asymptomatic MR underwent exercise echocardiography combined with cardiopulmonary exercise testing. Predictors of exercise-induced dyspnoea, symptom-free survival and mitral valve intervention were identified. Results 18 (19%) patients developed limiting dyspnoea on exercise. Spontaneous symptom free survival at 24 months was significantly higher in those without exercise-induced symptoms compared to those with exercise-induced symptoms, p &amp;lt;0.0001. The only independent predictors of spontaneous symptoms at 2 years were effective regurgitant orifice area (odds ratio 27.45 (95% CI 1.43 – 528.40), p = 0.03) and exercise-induced symptoms (odds ratio 11.56 (95% CI 1.71 – 78.09), p = 0.01). The only independent predictor of surgery was indexed left ventricular systolic volumes (Odds ratio 1.17 (95% CI 1.04 – 1.30), p = 0.006). Where only patients undergoing surgery due to symptoms were included, the only independent predictor was exercise-induced symptoms (Odds ratio 13.94 (95% CI 1.39 – 140.27), p = 0.025). Conclusion In patients with primary asymptomatic degenerative MR, one fifth develop symptoms on exercise. This predicts subsequent development of spontaneous symptoms and mitral valve intervention due to symptoms.

Visualizing exertional dyspnea in a post-COVID patient using electrical impedance tomography

Purpose and methodMany post-COVID patients suffer from dyspnea on exertion. To visualize exercise-induced dyspnea, a post-COVID patient and a healthy volunteer underwent an exercise test on a treadmill under stress relevant to everyday life monitored by electrical impedance tomography (EIT).ResultsThe lung-healthy volunteer showed an even ventilation distribution throughout the assessment, a large ventilated area, and a butterfly-like lung shape with a convex lung rim. The post-COVID patient showed clear differences in the ventilated area compared to the control subject. During exercise, a constantly changing picture of differently ventilated areas is shown. However, especially the anterior regions were under-ventilated and larger areas were partially absent from ventilation. Overall, uncoordinated breathing and an uneven distribution of ventilation dominated the findings.ConclusionEIT is suitable for visualizing disturbed ventilation of the lungs, both at rest and under stress. The potential as a diagnostic tool in dyspnea assessment should be investigated.

Dynamic lung water MRI during exercise stress.

Exercise-induced dyspnea caused by lung water is an early heart failure symptom. Dynamic lung water quantification during exercise is therefore of interest to detect early stage disease. This study developed a time-resolved 3D MRI method to quantify transient lung water dynamics during rest and exercise stress. The method was evaluated in 15 healthy subjects and 2 patients with heart failure imaged in transitions between rest and exercise, and in a porcine model of dynamic extravascular lung water accumulation through mitral regurgitation (n = 5). Time-resolved images were acquired at 0.55T using a continuous 3D stack-of-spirals proton density weighted sequence with 3.5 mm isotropic resolution, and derived using a motion corrected sliding-window reconstruction with 90-s temporal resolution in 20-s increments. A supine MRI-compatible pedal ergometer was used for exercise. Global and regional lung water density (LWD) and percent change in LWD (ΔLWD) were automatically quantified. A ΔLWD increase of 3.3 ± 1.5% was achieved in the animals. Healthy subjects developed a ΔLWD of 7.8 ± 5.0% during moderate exercise, peaked at 16 ± 6.8% during vigorous exercise, and remained unchanged over 10 min at rest (-1.4 ± 3.5%, p = 0.18). Regional LWD were higher posteriorly compared the anterior lungs (rest: 33 ± 3.7% vs 20 ± 3.1%, p < 0.0001; peak exercise: 36 ± 5.5% vs 25 ± 4.6%, p < 0.0001). Accumulation rates were slower in patients than healthy subjects (2.0 ± 0.1%/min vs 2.6 ± 0.9%/min, respectively), whereas LWD were similar at rest (28 ± 10% and 28 ± 2.9%) and peak exercise (ΔLWD 17 ± 10% vs 16 ± 6.8%). Lung water dynamics can be quantified during exercise using continuous 3D MRI and a sliding-window image reconstruction.

Perception of exercise-induced dyspnea after experimentally induced breathing discomfort.

The perception of dyspnea is influenced by both physiological and psychological factors. We sought to determine whether exertional dyspnea perception could be experimentally manipulated through prior exposure to heightened dyspnea while exercising. We hypothesized that dyspnea perception during exercise would be lower following an induced dyspnea task (IDT). Sixteen healthy participants (eight females, eight males) completed two days of exercise testing. Day 1 involved an incremental cycle exercise test starting at 40 W for females and 60 W for males, increasing by 20 W each minute until volitional exhaustion. Following the maximal exercise test on Day 1, participants completed IDT, involving 5 min of exercise at 70% of peak work rate with 500 mL dead space and external resistance (i.e., 6.8 ± 2.3 cm·H2O·s-1·L-1 inspiration, 3.8 ± 0.7 cm·H2O·s-1·L-1 expiration). Day 2 consisted of an incremental exercise test identical to Day 1. At maximal exercise, there were no differences in oxygen uptake (V̇O2; 44.7 ± 7.7vs. 46.5 ± 6.3 mL·kg-1·min-1), minute ventilation (120 ± 35vs. 127 ± 38 L·min-1), dyspnea (6.5 [4, 8.5] vs. 6 [4.25, 8.75]), or leg discomfort (6 [5, 8.75] vs. 7 [5, 9]) between days (all p > 0.05). At 60%-80% of peak V̇O2 (V̇O2peak), dyspnea was significantly lower on Day 2 (-0.75 [-1.375, 0] for 60% and -0.5 [0, -2] for 80%, p < 0.05) despite no differences in relevant physiological variables. The onset of perceived dyspnea occurred at a significantly higher exercise intensity on Day 2 than on Day 1 (42% ± 19%vs. 51% ± 17% V̇O2peak, respectively; p < 0.05). Except for 40% V̇O2peak (p = 0.05), RPE-L was not different at any intensities nor was the onset of perceived leg discomfort different between days (38% ± 14%vs. 43% ± 10% V̇O2peak, respectively; p = 0.10). Exposure to heightened dyspnea alters exercise-induced dyspnea perception during subsequent submaximal exercise bouts.

Exercise-induced laryngeal obstruction: a rarely recognized cause of exercise-induced dyspnea. Two case reports

Introduction: Exercise-induced laryngeal obstruction (EILO) is a transient and reversible narrowing of the larynx in response to intense physical effort. The clinical picture of EILO often resembles those of asthma or exercise induced bronchospasm. The gold standard in EILO diagnostics is nasofiberoscopic examination during intense exercise (CLE).Case Description: We present two clinical cases of athletes initially treated for asthma who were diagnosed with EILO after CLE. This allowed the initiation of phoniatric rehabilitation and initial improvement of exercise tolerance.

Sensitivity of FEV1 and Clinical Parameters in Children With a Suspected Asthma Diagnosis

Asthma is the most common chronic disease in children and a robust diagnosis is crucial to optimize patient care and reduce its burden. To diagnose asthma in children, the Global Initiative for Asthma (GINA) recommendations propose a 12% improvement in forced expiratory volume in 1 second (FEV1) after a bronchodilation test. Nevertheless, such a criterion is rarely confirmed in these patients in clinical practice. The objective of this study was to evaluate the sensitivity of spirometric and clinical parameters in identifying children with possible asthma. The VERI-VEMS Study is a multicenter international retrospective cohort study. Data were collected, from January 2008 until January 2019, for all consecutive children (aged 5-18 years), with a diagnosis of asthma, who performed a spirometry at the time of the diagnosis. We compared the sensitivity of the reversibility criterion proposed by GINA guidelines, with other spirometric and clinical variables, using physician-diagnosed asthma and response to treatment as the standard. The study included 871 children. The reversibility criterion of 12% of FEV1 showed a sensitivity of 30.4%. The 3 best spirometric or clinical criteria were the presence of dry cough, or wheezing or atopy and dry cough, or wheezing or exercise-induced dyspnea, with a sensitivity reaching 99.5%, with no added value of the spirometric parameters in the calculation of the cumulated sensitivity for the diagnosis of pediatric asthma. Postbronchodilator reversibility of 12%, although essential for patients' follow-up, has an insufficient low sensitivity in reaching a diagnosis of asthma in pediatric patients, compared with a combination of clinical symptoms, that show a better sensitivity. Further studies on specificity will help clarify the role of this change in the diagnostic paradigm in formally diagnosing children with asthma.

A novel phenotype in an Italian family with a rare progranulin mutation

IntroductionProgranulin (PGRN) is a secreted glycoprotein encoded in humans by the GRN gene, located on chromosome 17q21. Several nonsense and missense pathogenetic GRN mutations have been described.ObjectiveWe herein describe two sisters carrying a rare GRN mutation with extremely different clinical features and family history of dementia and behavioral disorders, with a novel presentation with stridor and dysphonia.MethodsPatients underwent a multidimensional assessment including neurological and neuropsychological evaluation, structural and functional imaging, and genetic screening.ResultsThe younger sister presented at the age of 64 with inspiratory stridor, dysphonia and exercise-induced dyspnea. Transnasal fiberoptic laryngoscopy showed bilateral adduction of the vocal cords at rest and paradoxical further adduction of the vocal cords during forced inspiration, suggesting the hypothesis of an adductor laryngeal dystonia. The older sister presented at the age of 63 with a rapidly progressive corticobasal syndrome. The only clinical feature common to both sisters was a dysexecutive syndrome. The c.893G > A mutation in exon 9 of GRN was found in heterozygosis in both sisters, causing a missense Arginine to Histidine substitution in position 298 of the protein (p.R298H).ConclusionsOur report supports the pathogenicity of the GRN p.R298H mutation, which is first detected in two members from the same family, showing an extremely different phenotypes. Moreover, we report the first case of an FTD-associated mutation presenting with inspiratory stridor and dysphonia linked to adductor laryngeal dystonia, thus expanding the clinical spectrum of GRN-related disorders.

Unexplained exercise-related dyspnea in type 2 diabetes predicts pulmonary arterial hypertension during exercise

Abstract Funding Acknowledgements Type of funding sources: None. Purpose To evaluate whether type 2 diabetes mellitus (T2DM) patients who suffer from unexplained exercise-induced dyspnea display cardiac dysfunction during exercise. Background Patients with type 2 diabetes mellitus (T2DM) frequently suffer from unexplained dyspnea. It is not fully understood whether these patients have a worse exercise capacity and cardiac dysfunction than non-dyspneic patients. If so, it would be clinically relevant to evaluate cardiac function in T2DM patients with unexplained dyspnea and initiate treatment accordingly. Methods 47 T2DM patients with unexplained dyspnea and 50 asymptomatic T2DM patients underwent echocardiography with simultaneous cardiopulmonary exercise testing (CPETecho). Left ventricular (LV) function (cardiac output, LV ejection fraction, stroke volume), estimated LV filling pressures (E/e’) and mean pulmonary artery pressures (mPAP) were assessed at rest, low- and high-intensity exercise by using colloid contrast. Breath-by-breath analyses were used to monitor exercise intensity and evaluate exercise capacity (V̇O2peak). Results The groups had similar characteristics and glycemic control (p&amp;gt;0.05). LV function and E/e’ were similar between groups during all stages of exercise (p&amp;gt;0.05). However, mPAP increased disproportionally (mPAP/CO &amp;gt; 3) during exercise in 62% of the patients with dyspnea. The mPAP/CO slope was significantly steeper in the patients with, as opposed to without dyspnea (Pinteraction=0.013). Moreover, a steeper mPAP/cardiac output slope (β= -1.86, 95% CI -2.75, -0.98) was an independent predictor of lower V̇O2peak (multivariate model R²:0.54). Conclusion In T2DM patients with unexplained exertional dyspnea, simultaneous cardiopulmonary exercise testing and echocardiography frequently reveal a reduced exercise capacity associated with exercise-induced pulmonary arterial hypertension. Dyspneic T2DM patients possibly have early left atrial and LV diastolic dysfunction. Timely preventive and therapeutic interventions may improve clinical outcomes in these patients.

Breath Tools: A Synthesis of Evidence-Based Breathing Strategies to Enhance Human Running.

Running is among the most popular sporting hobbies and often chosen specifically for intrinsic psychological benefits. However, up to 40% of runners may experience exercise-induced dyspnoea as a result of cascading physiological phenomena, possibly causing negative psychological states or barriers to participation. Breathing techniques such as slow, deep breathing have proven benefits at rest, but it is unclear if they can be used during exercise to address respiratory limitations or improve performance. While direct experimental evidence is limited, diverse findings from exercise physiology and sports science combined with anecdotal knowledge from Yoga, meditation, and breathwork suggest that many aspects of breathing could be improved via purposeful strategies. Hence, we sought to synthesize these disparate sources to create a new theoretical framework called “Breath Tools” proposing breathing strategies for use during running to improve tolerance, performance, and lower barriers to long-term enjoyment.

Dyspnea in Post-COVID Syndrome following Mild Acute COVID-19 Infections: Potential Causes and Consequences for a Therapeutic Approach.

Dyspnea, shortness of breath, and chest pain are frequent symptoms of post-COVID syndrome (PCS). These symptoms are unrelated to organ damage in most patients after mild acute COVID infection. Hyperventilation has been identified as a cause of exercise-induced dyspnea in PCS. Since there is a broad overlap in symptomatology with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), causes for dyspnea and potential consequences can be deduced by a stringent application of assumptions made for ME/CFS in our recent review papers. One of the first stimuli of respiration in exercise is caused by metabolic feedback via skeletal muscle afferents. Hyperventilation in PCS, which occurs early on during exercise, can arise from a combined disturbance of a poor skeletal muscle energetic situation and autonomic dysfunction (overshooting respiratory response), both found in ME/CFS. The exaggerated respiratory response aggravating dyspnea does not only limit the ability to exercise but further impairs the muscular energetic situation: one of the buffering mechanisms to respiratory alkalosis is a proton shift from intracellular to extracellular space via the sodium–proton-exchanger subtype 1 (NHE1), thereby loading cells with sodium. This adds to two other sodium loading mechanisms already operative, namely glycolytic metabolism (intracellular acidosis) and impaired Na+/K+ATPase activity. High intracellular sodium has unfavorable effects on mitochondrial calcium and metabolism via sodium–calcium-exchangers (NCX). Mitochondrial calcium overload by high intracellular sodium reversing the transport mode of NCX to import calcium is a key driver for fatigue and chronification. Prevention of hyperventilation has a therapeutic potential by keeping intracellular sodium below the threshold where calcium overload occurs.

Adiposity increases weight-bearing exercise-induced dyspnea despite favoring resting lung hyperinflation in COPD.

ObjectivesOur aim was to study the associations between resting lung hyperinflation, weight-bearing exercise-induced dyspnea and adipose distribution in obese and normal-weight COPD patients.MethodsWe performed a comparison between 80 obese COPD patients (COPDOB) with 80 age- and FEV1 matched normal-weight COPD patients (COPDNW). Dyspnea was assessed by the mMRC scale and the Borg dyspnea score before and after a 6 min walk test. Further characterization included spirometry, body plethysmography and metronome paced tachypnea (MPT) to estimate dynamic hyperinflation. Body composition was assessed with bioelectrical impedance analysis. Associations between dyspnea scores and BMI and body composition groups were studied using logistic regression models.ResultsCOPDOB patients had attenuated increases in TLC, FRC and RV compared to COPDNW patients (p < 0.01). The groups had comparable 6 min walking distance and ΔFRC upon MPT (p > 0.05). Compared to COPDNW, COPDOB patients reported more often a mMRC ≥ 2 (65 vs 46%; p = 0.02; OR 3.0, 95% CI 1.4–6.2, p < 0.01) and had higher ΔBorg upon 6MWT: 2.0 (SEM 0.20) vs. 1.4 (SEM 0.16), p = 0.01; OR for ΔBorg ≥ 2: 2.4, 95% CI 1.1–5.2, p = 0.03. Additional logistic regression analyses on the associations between body composition and dyspnea indicated that increased body fat percentage, fat mass index and waist-to-hip ratio were associated with higher ORs for mMRC ≥ 2 and ΔBorg upon 6MWT ≥ 2.ConclusionDespite its beneficial effect on resting lung hyperinflation, adiposity is associated with increased weight-bearing exercise-induced dyspnea in COPD.

Self-reported exercise-induced dyspnea and airways obstruction assessed by oscillometry and spirometry in adolescents.

BackgroundSelf‐reported exercise‐induced dyspnea (EID) is common among adolescents. Possible underlying pathologies are exercise‐induced bronchoconstriction (EIB) and laryngeal obstruction (EILO). The forced oscillation technique (FOT) may evaluate exercise‐induced changes in airway caliber.AimTo investigate in adolescents the relationship between EID, EIB (post‐exercise fall in forced expiratory volume in 1s (FEV1)≥10%), EILO, and post‐exercise challenge changes in FOT parameters.MethodsOne hundred and forty‐three subjects (97 with EID) of 13–15 years old underwent a standardized exercise challenge with FOT measurement and spirometry repeatedly performed between 2 and 30 min post‐exercise. EILO was studied in a subset of 123 adolescents. Subjects showing greater changes than the healthy subgroup in the modulus of the inspiratory impedance were considered FOT responders.ResultsEID‐nonEIB subjects presented similar post‐exercise changes in all FOT parameters to nonEID‐nonEIB adolescents. Changes in all FOT parameters correlated with FEV1 fall. 45 of 97 EID subjects responded neither by FEV1 nor FOT to exercise. 19 and 18 subjects responded only by FEV1 (onlyFEV1responders) or FOT (onlyFOTresponders), respectively. Only a lower baseline forced vital capacity (FVC)%predicted and a higher FEV1/FVC distinguished the onlyFEV1responders from onlyFOTresponders. FOT parameters did not present specific post‐exercise patterns in EILO subjects.ConclusionFOT can be used to identify post‐exercise changes in lower airway function. However, EID has a modest relation with both FEV1 and FOT responses, highlighting the need for objective testing. More research is needed to understand whether onlyFEV1responders and onlyFOTresponders represent different endotypes.

400 Refractory pulmonary hypertension in a young woman

Abstract Aims Clinical case—Twenty-four years old Moroccan woman. Family history: parents and three siblings in good health. Methods and results Past medical history—In 2016, when she was 19 years old, she developed worsening exercise-induced dyspnoea. A right heart catheterization (RHC) was performed with evidence of increased median pulmonary artery pressure (mPAP 60 mmHg, wedge pressure 8 mmHg), cardiac index 2.27 l/min/m2. She was diagnosed with idiopathic pulmonary hypertension and combination therapy with sildenafil and macitentan was started with partial improvement. In August 2019, she became pregnant and vasodilatory therapy was suspended. The pregnancy was complicated by premature labor with foetal death. Specific therapy with sildenafil and macitentan was then restarted. Due to further clinical and haemodynamic impairment, triple combination therapy with selexipag was initiated. However, symptomatic deterioration progressed, and she was referred to a Pulmonary Hypertension Referral Center where HRTC of the chest showed centrilobular ground-glass opacities and interlobular septal thickening. Based on the imaging that was highly suspicious pulmonary veno-occlusive disease (PVOD), and the severe haemodynamic impairment (with pulmonary vascular resistance at RHC &amp;gt; 20 WU), assessment for lung transplantation was started. Recent medical history—she was transferred to our Center with Transplant Unit for lung transplant assessment. Pulmonary function testing demonstrated a restrictive disorder with severe reduction of DLCO (14%). At 6-min walking test, she could walk 100 m with desaturation up to 90% on O2 therapy. Evaluated by our multidisciplinary team, indications for lung transplantation were confirmed and she entered the transplant waiting list. At the end of February 2021: further clinical and haemodynamic deterioration with respiratory distress, reduction in urinary output and signs and symptoms of right-side heart failure; she was hospitalized in intensive care unit and required extra-corporeal membrane oxygenation (ECMO) circulatory support. Selexipag was suspended. On echocardiography: severe right ventricle hypertrophy and dilatation, tricuspid regurgitation velocity &amp;gt;4 m/s. Few days later, she underwent bilateral lung transplantation; anatomo-pathological evaluation of explanted organs confirmed PVOD. However, during post-operative monitoring, she suffered from two episodes of cardiac arrest on VT/VF which required multiple DC-shocks. Since no triggering causes were identified, an ICD was implanted for secondary prevention. Conclusions PVOD is a rare disease with clinical presentation and haemodynamic profile often similar to pulmonary arterial hypertension (and, therefore, the diagnosis is challenging) but the clinical course is more severe. The diagnosis requires clinical history and physical examination, together with multimodality imaging and functional testing. Bronchoalveolar lavage might be necessary. Patients do not usually respond satisfactorily to vasodilatory therapy but rather they are at high risk of drug-induced pulmonary oedema. Some case reports and case series have reported a slight benefit and/or clinical stabilization with vasodilatory therapy in selected patients; therefore, specific therapy can be used but cautiously and in experienced referral centres. Definitive therapy is lung transplantation (or heart-lung transplantation) and a multidisciplinary team is necessary for the appropriate management of these complicated patients. Ventricular arrhythmias are rare in patients with Group 1 pulmonary hypertension. In our specific clinical case, we suspect that ventricular arrhythmias might be related to the severely hypertrophic, and potentially fibrotic, right ventricle.

Modifiable factors associated with health-related quality of life among lung cancer survivors following curative intent therapy

ObjectiveThe number of lung cancer survivors is increasing along with advances in screening, diagnosis, and treatment. Following curative intent therapy, many lung cancer survivors experience significant health-related quality of life (HRQL) impairments. We sought to identify potentially modifiable factors that contribute to the HRQL of these patients. Materials and methodsIn this cross-sectional observational study of disease-free, stage I-IIIA lung cancer survivors following curative intent therapy, we used a conceptual model to examine factors that included behavioral, objective functional and physiologic, self-rated function and symptom burden, specific comorbidities, and non-modifiable demographic and clinical lung cancer-related characteristics. We assessed HRQL using the valid and prognostic European Organization for Research and Treatment of Cancer Quality of Life (QoL) Core 30 global health/QoL subscale. We used univariable and multivariable linear regression modeling with backward elimination of potentially modifiable and non-modifiable factors, and interpreted clinically and statistically significant, consistent, and independent modifiable factors as meaningful. ResultsAmong 75 participants at a median of 12 months since treatment completion, the mean (standard deviation) C30 global health/QoL score was 62.7 (23.3) points (0–100 scale range). In multivariable analysis, with and without non-modifiable factors, we identified three clinically and statistically significant, consistent, and independent factors (unstandardized β range) associated with global health/QoL: 1) abnormal exercise-induced dyspnea (−9.23 to −10.0 points); 2) impaired self-rated role function (or inability to perform work or daily activities and pursuing leisure-time activities) (−12.6 to −16.4 points); and 3) abnormal insomnia (or trouble sleeping) (−12.6 to −16.4 points). ConclusionWe identified meaningful modifiable factors associated with the HRQL of disease-free, stage I-IIIA lung cancer survivors following curative intent therapy. Interventions to improve the HRQL of these patients should aim to reduce exercise-induced dyspnea, improve role function – the ability to perform work and other daily including leisure-time activities, and control insomnia.

Development and validation of the Exercise-Induced Laryngeal Obstruction Dyspnea Index (EILODI)

Exercise-induced laryngeal obstruction (EILO) causes exertional dyspnea and is important for its effect on quality of life, diagnostic confusion with exercise-induced asthma, and health care resource utilization. There is no validated patient-reported outcome measure specific to EILO. We sought to develop, validate, and define a minimal clinically important difference for a patient-reported outcome measure to be used with adolescents and young adults with EILO. A multidisciplinary group created a preliminary measure, modified by a 10-member participant focus group, with 20 items scored along a 5-point Likert scale. Asubsequent cohort of participants recruited from a clinic, aged 12 to 21 years, with confirmed EILO by continuous laryngoscopy during exercise testing (1) completed the measure at 3 points in time over 28 days and (2) provided anchoring data in the form of a daily exercise log and categorical self-assessments of clinical improvement. Thirty additional participants without exertional dyspnea served as controls. Two hundred nineteen subjects with mild to severe EILO participated in the exploratory factor analysis, which identified 7 factors within the preliminary outcome measure. After a process of item reduction, a 12-item metric with a total score ranging from 0 to 48 was developed. Mean scores of patients with EILO and healthy controls at baseline were 28.8± 7.4 and 4.5± 7.4, respectively. Aminimal clinically important difference of 6 was determined by comparison of index change with changes in categorical self-assessments of improvement. This is the first patient-reported outcome measure specifically designed for adolescents and young adults with EILO.

Multimodal Approach of Isolated Pulmonary Vasculitis: A Single-Institution Experience

BackgroundIsolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. MethodsData were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. ResultsWe identified 9 patients (6 women; median age, 48 years [range, 23-55]) with IPV. The diagnosis was confirmed after histopathologic examination of all surgical materials. The mean duration of disease before surgery was 88.0 ± 70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in 6 patients and unilateral in 3. No deaths occurred; however 1 patient had pulmonary artery stenosis, and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30 mm Hg (range, 19-67) to 21 mm Hg (range, 15-49) after surgery (P < .05). Pulmonary vascular resistance also improved significantly from 270 dyn/s/cm–5 (range, 160-1600) to 153 dyn/s/cm–5 (range, 94-548; P < .05). After a median follow-up of 41 months, all but 1 patient had improved to the New York Heart Association functional class I. ConclusionsIPV can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients.

Exercise-Induced Dyspnea in Collegiate Athletes: Are We Missing the Diagnosis?

Exercise-induced dyspnea (EID) can disrupt an athlete's participation and performance in their given sport. Differential diagnosis of EID is often completed using subjective report and may be inaccurate, therefore increasing the frustration and stress of the athlete. This nonexperimental research study was used to determine prevalence of EID and related respiratory symptoms in athletes at a small, Division I university. An anonymous survey was provided to athletes at Murray State University as they registered for participation in sports for the 2020-2021 school year. Data from this survey was analyzed as to reported physician-given diagnosis of a respiratory disorder as well as reported symptoms of EID. Results showed that athletes with a physician-given diagnosis often did not report symptoms or responses to medications that support that diagnosis. Additionally, athletes frequently reported symptoms of EID without a formal diagnosis of a respiratory disorder. These findings provide preliminary insight and pilot data that may be used to understand the prevalence of EID in collegiate athletes and the need for improved methods of diagnosis for etiologies of EID.