scholarly journals Ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis

2021 ◽  
Vol 30 (162) ◽  
pp. 200395
Author(s):  
Pierantonio Laveneziana ◽  
Paolo Palange
Keyword(s):  
Cystic Fibrosis ◽  
Prognostic Value ◽  
Respiratory Infections ◽  
Genetic Disorder ◽  
Cardiopulmonary Exercise Testing ◽  
Airflow Limitation ◽  
Exercise Intolerance ◽  
Ventilatory Efficiency ◽  
Number Of Patients ◽  
Patients Experience

Cystic fibrosis, due to the absence or abnormal function of the cystic fibrosis transmembrane conductance regulator, is the most common life-limiting autosomal recessive genetic disorder among the Caucasian population. The lungs are particularly affected due to thick and tenacious mucus causing parenchymal anomalies ranging from bronchiectasis, progressive airflow limitation, respiratory infections, lung destruction and ultimately respiratory failure. Despite the remarkable advances in treatment that have greatly improved survival, most patients experience progressive exercise curtailment, with the consequence that a growing number of patients with cystic fibrosis will be referred for exercise-based evaluations in the forthcoming years. Cardiopulmonary exercise testing, in particular, is a useful tool to assess the mechanisms of exercise intolerance in individual patients that may have treatment and prognostic implications. In this review, we will focus on ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis.

Fungal Infection in Cystic Fibrosis

2021 ◽  
Vol 17 (2) ◽  
pp. 118-124
Author(s):  
Amirmehdi Sarvestani ◽  
Mohammad Almasian ◽  
Amirhossein Nafari
Keyword(s):  
Cystic Fibrosis ◽  
Fungal Infection ◽  
Fungal Pathogens ◽  
Respiratory Infections ◽  
Fungal Infections ◽  
Genetic Disorder ◽  
Medical Science ◽  
Resistant Species ◽  
Online Databases ◽  
New Treatments

Background: The prevalence of fungal infections has been increasing in recent years. Cystic fibrosis (CF) is a genetic disorder that affects organs such as the intestines, liver, pancreas, and especially the lungs. Introduction: Fungal pathogens are becoming a challenge in CF. Advanced medical science is associated with longer life expectancy in some patient groups. Method: A review was conducted on studies found on online databases, including Google Scholar, PubMed, and Scopus. Internet-based searches were performed on these databases for cystic fibrosis, respiratory infections, and fungal infection profiling to identify all relevant studies published between 2010 and 2020. Result: Fungal pathogens most frequently isolated from the respiratory tract include the Aspergillus genus, the Candida genus, Scedosporium apiospermum, and the Rasamsonia genus. In cystic fibrosis, these organisms usually colonize the respiratory and intestinal tracts and cause hypersensitivity responses and invasive diseases. Conclusion: Fungus-patient interactions are complicated and depend on various factors. Moreover, the emergence of drug-resistant species is a serious health issue, and the development of new treatments is crucial.

scholarly journals Prognostic value of cardiopulmonary exercise testing in valvular heart disease

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
C Cortina Camarero ◽  
M Sarrion Catala ◽  
V Suberviola Sanchez-Caballero ◽  
C Beltran Herrera ◽  
E Gomez Mariscal ◽  
...  
Keyword(s):  
Heart Disease ◽  
Valvular Heart Disease ◽  
Prognostic Value ◽  
Cardiopulmonary Exercise Testing ◽  
Symptom Development ◽  
Ventilatory Efficiency ◽  
Cardiopulmonary Exercise ◽  
Peak Vo2 ◽  
Echocardiographic Parameters

Abstract Introduction Cardiopulmonary exercise testing (CEPT) is not routinely used for the assessment of valvular heart disease (VHD) patients. The cutoff values of percentage of predicted peak VO2 and ventilatory efficiency parameters that carry out a bad prognosis have been predominantly validated in heart failure. The aim of this study was to analyze the prognostic value of CPET parameters in a broad population of VHD patients. Methods 163 patients (51% female) with moderate or severe VHD who underwent a CEPT (n=197) at their physician's discretion from 2017 until 2019 were included. We calculated the net reclassification index of CPET, compared to the classical clinical or echocardiographic parameters, regarding the need for surgical indication. Also, the predictive value of CPET for death and symptom development during follow-up was estimated using regression analysis. Results At inclusion, all patients were asymptomatic or with minimal equivocal symptoms. Aortic valvular lesions were the most common (47%), followed by mitral valvular disease (44%). There was a predominance of severe valvular heart disease (71%) and most of the CEPTs were performed on a treadmill (74%). The percentage of predicted peak VO2 was 76±18% and at anaerobic threshold was 61±18%. The mean follow-up time was 15±10 months. There were 5 deaths (3%) and 24 patients became symptomatic. The net reclassification index of CPET over either clinical or echocardiographic parameters was 46%. The CEPT parameters that predicted increased risk of death were VE/VCO2 slope (p=0.009), % of predicted peak VO2 (p=0.049) and Eq CO2 at anaerobic threshold (p=0.047). None of the CEPT parameters was predictive of symptom development during follow-up, however, in the subgroup of patients who became clearly symptomatic, the ventilatory efficiency parameters were similar to the cut off values that confer bad prognosis (see Table in Figure). Conclusion In our series, CPET added prognostic mortality value to patients with VHD. The cut off values used for patients with HF can also be applied in a VHD population. In addition, CEPT clearly improved the clinical decision for surgical referral. Nevertheless, these results need to be validated in a broader population. FUNDunding Acknowledgement Type of funding sources: None.

scholarly journals Exercise Stroke Volume in Adult Cystic Fibrosis: A Comparison of Acetylene Pulmonary Uptake and Oxygen Pulse

2018 ◽  
Vol 12 ◽  
pp. 117954841879056 ◽  
Author(s):  
Erik H Van Iterson ◽  
Sarah E Baker ◽  
Courtney M Wheatley ◽  
Wayne J Morgan ◽  
Thomas P Olson ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Stroke Volume ◽  
Cardiopulmonary Exercise Testing ◽  
Exercise Intolerance ◽  
Peak Exercise ◽  
Oxygen Pulse ◽  
Pulmonary Uptake ◽  
Hemodynamic Assessment ◽  
Valid Estimate ◽  
The Impact

Cardiac hemodynamic assessment during cardiopulmonary exercise testing (CPET) is proposed to play an important role in the clinical evaluation of individuals with cystic fibrosis (CF). Cardiac catheterization is not practical for routine clinical CPET. Use of oxygen pulse (O2pulse) as a noninvasive estimate of stroke volume (SV) has not been validated in CF. This study tested the hypothesis that peak exercise O2pulse is a valid estimate of SV in CF. Measurements of SV via the acetylene rebreathe technique were acquired at baseline and peak exercise in 17 mild-to-moderate severity adult CF and 25 age-matched healthy adults. We calculated [Formula: see text]. Baseline relationships between SV and O2pulse were significant in CF ( r = .80) and controls ( r = .40), persisting to peak exercise in CF ( r = .63) and controls ( r = .73). The standard error of estimate for O2pulse-predicted SV with respect to measured SV was similar at baseline (14.1 vs 20.1 mL) and peak exercise (18.2 vs 13.9 mL) for CF and controls, respectively. These data suggest that peak exercise O2pulse is a valid estimate of SV in CF. The ability to noninvasively estimate SV via O2pulse during routine clinical CPET can be used to improve test interpretation and advance our understanding of the impact cardiac dysfunction has on exercise intolerance in CF.

scholarly journals Evolution and adaptation of Pseudomonas aeruginosa in the paranasal sinuses of people with cystic fibrosis

2020 ◽  
Author(s):  
Catherine R. Armbruster ◽  
Christopher W. Marshall ◽  
Jeffrey A. Melvin ◽  
Anna C. Zemke ◽  
Arkadiy I. Garber ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Disease ◽  
Respiratory Infections ◽  
Genetic Disorder ◽  
List Type ◽  
Host Restriction ◽  
Single Clone ◽  
Lung Infections ◽  
End Stage

AbstractPeople with the genetic disorder cystic fibrosis (CF) harbor lifelong respiratory infections, with morbidity and mortality frequently linked to chronic lung infections dominated by the opportunistically pathogenic bacterium Pseudomonas aeruginosa. During chronic CF lung infections, a single clone of P. aeruginosa can persist for decades and dominate end-stage CF lung disease due to its propensity to adaptively evolve to the respiratory environment, a process termed “pathoadaptation”. Chronic rhinosinusitis (CRS), chronic inflammation and infection of the sinonasal space, is highly prevalent in CF and the sinuses may serve as the first site in the respiratory tract to become colonized by bacteria that then proceed to seed lung infections. We identified three evolutionary genetic routes by which P. aeruginosa evolves in the sinuses of people with CF, including through the evolution of mutator lineages and proliferative insertion sequences and culminating in early genomic signatures of host-restriction. Our findings raise the question of whether a significant portion of the pathoadaptive phenotypes previously thought to have evolved in response to selective pressures in the CF lungs may have first arisen in the sinuses and underscore the link between sinonasal and lung disease in CF.Graphical abstract and highlightsPseudomonas aeruginosa undergoes adaptive evolution in the sinuses of people with CFOver time, pathoadapted strains display early signatures of genome degradation consistent with recent host restrictionMutations previously thought to occur in CF lungs may have first evolved in sinuses

Fungal infection in cystic fibrosis

2021 ◽  
Vol 17 ◽  
Author(s):  
Amirmehdi Sarvestani ◽  
Mohammad Almasian ◽  
Amirhossein Nafari
Keyword(s):  
Cystic Fibrosis ◽  
Fungal Infection ◽  
Fungal Pathogens ◽  
Respiratory Infections ◽  
Fungal Infections ◽  
Genetic Disorder ◽  
Medical Science ◽  
Resistant Species ◽  
Online Databases ◽  
New Treatments

Background: The prevalence of fungal infections has been increasing in recent years. Cystic fibrosis (CF) is a genetic disorder that affects organs such as the intestines, liver, pancreas, and especially the lungs. Introduction: Fungal pathogens are becoming a challenge in CF. Advanced medical science is associated with longer life expectancy in some patient groups. Method: A review was conducted on studies found on such online databases as Google Scholar, PubMed, and Scopus. Internet-based searches were performed on these databases for cystic fibrosis, respiratory infections, and fungal infection profiling to identify all relevant studies published between 2010 and 2020. Result: Fungal pathogens most frequently isolated from the respiratory tract include the Aspergillus genus, the Candida genus, Scedosporium apiospermum, and the Rasamsonia genus. In cystic fibrosis, these organisms usually colonize the respiratory and intestinal tracts and cause hypersensitivity responses and invasive diseases. Conclusion: Fungus-patient interactions are complicated and depend on various factors. Moreover, the emergence of drug-resistant species is a serious health issue, and the development of new treatments is crucial.

scholarly journals Exercise intolerance in chronic thromboembolic pulmonary hypertension after pulmonary angioplasty

2020 ◽  
Vol 56 (1) ◽  
pp. 1901982
Author(s):  
Hanako Kikuchi ◽  
Ayumi Goda ◽  
Kaori Takeuchi ◽  
Takumi Inami ◽  
Takashi Kohno ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Oxygen Consumption ◽  
Exercise Capacity ◽  
Cardiopulmonary Exercise Testing ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Exercise Intolerance ◽  
Peak Oxygen Consumption ◽  
Ventilatory Efficiency ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

IntroductionExercise pulmonary hypertension is common in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who experience shortness of breath during exercise and reduced exercise capacity despite normalised pulmonary arterial pressure (PAP) at rest; however, the relationship between exercise pulmonary hypertension and exercise capacity remains unclear. Here we aimed to determine whether exercise pulmonary hypertension is related to exercise capacity and ventilatory efficiency in CTEPH patients with normalised resting haemodynamics after pulmonary balloon angioplasty (BPA).Patients and methodsIn total, 249 patients with CTEPH treated with BPA (mean±sd age 63±14 years; male:female 62:187) with normal mean PAP (mPAP) (<25 mmHg) and pulmonary arterial wedge pressure (≤15 mmHg) at rest underwent cardiopulmonary exercise testing with right heart catheterisation. mPAP–cardiac output (CO) during exercise was plotted using multipoint plots. Exercise pulmonary hypertension was defined by a mPAP–CO slope >3.0.ResultsAt rest, pulmonary vascular resistance was significantly higher in the exercise pulmonary hypertension group (n=116) than in the non-exercise pulmonary hypertension group (n=133). Lower peak oxygen consumption (13.5±3.8 versus 16.6±4.7 mL·min−1·kg−1; p<0.001) was observed in the former group. The mPAP–CO slope was negatively correlated with peak oxygen consumption (r= −0.45, p<0.001) and positively correlated with the minute ventilation versus carbon dioxide output slope (r=0.39, p<0.001).ConclusionsImpaired exercise capacity and ventilatory efficiency were observed in patients with CTEPH who had normalised PAP at rest but exercise pulmonary hypertension.

Reduced exercise ventilatory efficiency in adults with cystic fibrosis and normal to moderately impaired lung function

2019 ◽  
Vol 127 (2) ◽  
pp. 501-512 ◽  
Author(s):  
Marcello Di Paolo ◽  
Elisabetta Teopompi ◽  
Daniela Savi ◽  
Ernesto Crisafulli ◽  
Chiara Longo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Lung Disease ◽  
Exercise Testing ◽  
Minute Ventilation ◽  
Cardiopulmonary Exercise Testing ◽  
Normal Lung ◽  
Dynamic Hyperinflation ◽  
Ventilatory Efficiency ◽  
Impaired Lung Function

Despite being a hallmark and an independent prognostic factor in several cardiopulmonary diseases, ventilatory efficiency—i.e., minute ventilation/carbon dioxide output relationship (V̇e/V̇co2)—has never been systematically explored in cystic fibrosis (CF). To provide a comprehensive frame of reference regarding measures of ventilatory efficiency in CF adults with normal to moderately impaired lung function and to confirm the hypothesis that V̇e/V̇co2 is a sensitive marker of early lung disease. CF patients were divided into three groups, according to their spirometry: normal (G1), mild impairment (G2), and moderate impairment (G3) in lung function. All participants underwent incremental cardiopulmonary exercise testing on a cycle ergometer. Lowest V̇e/V̇co2 ratio (nadir) and the slope and the intercept of the linear region of the V̇e/V̇co2 relationship were contrasted in a two-center retrospective analysis, involving 72 CF patients and 36 healthy controls (HC). Compared with HC, CF patients had significantly higher V̇e/V̇co2 nadir, slope, and intercept ( P < 0.001, P < 0.001, and P = 0.049, respectively). Subgroup analysis revealed significant differences in nadir ( P = 0.001) and slope ( P = 0.012) values even between HC and G1. Dynamic hyperinflation related negatively with slope ( P = 0.045) and positively with intercept ( P = 0.001), while no impact on nadir was observed. Ventilatory inefficiency is a clear feature of adults with CF, even among patients with normal spirometry. V̇e/V̇co2 nadir seems to be the most reliable metric to describe ventilatory efficiency in CF adults. Further prospective studies are needed to clarify whether V̇e/V̇co2 could represent a useful marker in the evaluation of early lung disease in CF. NEW & NOTEWORTHY This is the first study to investigate ventilatory efficiency in a cohort of adult cystic fibrosis (CF) patients with nonsevere lung disease. The finding of impaired ventilatory efficiency in patients with normal lung function confirms the higher sensitivity of exercise testing in detecting early lung disease compared with spirometry. Dynamic hyperinflation plays a significant role in determining the behavior of V̇e/V̇co2 slope and intercept values with increasing lung function impairment. Apparently free from interference from mechanical constraints, V̇e/V̇co2 nadir seems the most reliable parameter to evaluate ventilatory efficiency in CF adults.

scholarly journals Airway clearance physiotherapy improves ventilatory dynamics during exercise in patients with cystic fibrosis: a pilot study

2018 ◽  
Vol 104 (1) ◽  
pp. 37-42 ◽  
Author(s):  
Fernanda Maria Vendrusculo ◽  
Zoe Johnstone ◽  
Elaine Dhouieb ◽  
Márcio V F Donadio ◽  
Steven Cunningham ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pilot Study ◽  
Exercise Capacity ◽  
Ventilatory Threshold ◽  
Minute Ventilation ◽  
Cardiopulmonary Exercise Testing ◽  
Airflow Limitation ◽  
Dynamic Hyperinflation ◽  
Airway Clearance ◽  
Significant Difference

BackgroundAirflow limitation and dynamic hyperinflation may limit exercise capacity in patients with cystic fibrosis (CF). The aim was to investigate whether the undertaking of airway clearance physiotherapy (ACT) prior to cardiopulmonary exercise testing (CPET) results in improvements in exercise capacity.MethodsA prospective randomised, cross-over pilot study was performed in children aged >9 years. Spirometry, plethysmography and CPET were performed on two separate occasions—one test with ACT prior to CPET and the other without.Results12 patients with CF were included in the study with a mean (SD) age of 12.83 (1.85) years. No significant difference in peak oxygen uptake (VO2) was found between the tests. However, lower minute ventilation (VE) and ventilatory equivalents (VEVO2 and VEVCO2) at ventilatory threshold (VT) were noted when ACT was undertaken prior to CPET. The mean(SD) VE (L/min) at VT was 26.67 (5.49) vs 28.92 (6.3) (p=0.05), VEVO2 (L/min) at VT was 24.5 (1.75) vs 26.05 (2.5) (p=0.03) and VEVCO2 (L/min) at VT was 26.58 (2.41) vs 27.98 (2.11) (p=0.03).ConclusionsThese pilot data suggest that ACT prior to exercise may lead to improved ventilatory dynamics during exercise in individuals with CF.

Sign in / Sign up

Export Citation Format

Share Document