Patients with idiopathic pulmonary fibrosis (IPF) usually develop hypoxemia and pulmonary hypertension when exercising. To what extent endothelium-derived vasodilating agents modify these changes is ...unknown. The study was aimed to investigate in patients with IPF whether exercise induces changes in plasma levels of endothelium-derived signaling mediators, and to assess the acute effects of inhaled nitric oxide (NO) on pulmonary hemodynamics and gas exchange, at rest and during exercise. We evaluated seven patients with IPF (6 men/1 woman; 57 ± 11 yr; forced vital capacity, 60 ± 13% predicted; carbon monoxide diffusing capacity, 52 ± 10% predicted). Levels of endothelin, 6-keto-prostaglandin-F(1α), thromboxane B(2), and nitrates were measured at rest and during submaximal exercise. Pulmonary hemodynamics and gas exchange, including ventilation-perfusion relationships, were assessed breathing ambient air and 40 ppm NO, both at rest and during submaximal exercise. The concentration of thromboxane B(2) increased during exercise (P = 0.046), whereas levels of other mediators did not change. The change in 6-keto-prostaglandin-F(1α) correlated with that of mean pulmonary arterial pressure (r = 0.94; P < 0.005). Inhaled NO reduced mean pulmonary arterial pressure at rest (-4.6 ± 2.1 mmHg) and during exercise (-11.7 ± 7.1 mmHg) (P = 0.001 and P = 0.004, respectively), without altering arterial oxygenation or ventilation-perfusion distributions in any of the study conditions. Alveolar-to-capillary oxygen diffusion limitation, which accounted for the decrease of arterial Po(2) during exercise, was not modified by NO administration. We conclude that, in IPF, some endothelium-derived signaling molecules may modulate the development of pulmonary hypertension during exercise, and that the administration of inhaled NO reduces pulmonary vascular resistance without disturbing gas exchange.
Exercise capacity declines with time and is an important determinant of health status and prognosis in patients with chronic obstructive pulmonary disease (COPD). We hypothesised that hospital ...admissions are associated with exercise capacity decline in these patients. Clinical and functional variables were collected for 342 clinically stable COPD patients. The 6-min walk distance (6MWD) was determined at baseline and after a mean±sd of 1.7±0.3 years. Information on hospitalisations during follow-up was obtained from centralised administrative databases. Linear regression was used to model changes in exercise capacity. Patients were mostly male (92%), with mean±sd age 67.9±8.6 years, post-bronchodilator forced expiratory volume in 1 s 54±17% predicted and baseline 6MWD 433±93 m. During follow-up, 6MWD decreased by 21.9±51.0 m·year(-1) and 153 (45%) patients were hospitalised at least once. Among patients admitted only for COPD-related causes (50% of those ever admitted), the proportion presenting a clinically significant loss of 6MWD was higher than in patients admitted for only nonrespiratory conditions (53% versus 29%, p=0.040). After adjusting for confounders, annual 6MWD decline was greater (26 m·year(-1), 95% CI 13-38 m·year(-1); p<0.001) in patients with more than one all-cause hospitalisation per year, as compared with those with no hospitalisations. Hospitalisations are related to a greater decline in exercise capacity in COPD.
•Imaging work-up has a crucial role in the management of patients with chronic thromboembolic pulmonary hypertension before balloon pulmonary angioplasty.•Dual-energy CT pulmonary angiography and ...cone beam CT pulmonary angiography enhance lesion characterization in patients with chronic thromboembolic pulmonary hypertension.•Cone beam CT-pulmonary angiography is a useful tool for planning and monitoring balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension.•Post-balloon pulmonary angioplasty imaging is essential for guiding long-term management of patients with chronic thromboembolic pulmonary hypertension.
Balloon pulmonary angioplasty (BPA) has recently been elevated as a class I recommendation for the treatment of inoperable or residual chronic thromboembolic pulmonary hypertension (CTEPH). Proper patient selection, procedural safety, and post-procedural evaluation are crucial in the management of these patients, with imaging work-up playing a pivotal role. Understanding the diagnostic and therapeutic imaging algorithms of CTEPH, the imaging features of patients amenable to BPA, all imaging findings observed during and immediately after the procedure and the changes observed during the follow-up is crucial for all interventional radiologists involved in the care of patients with CTEPH. This article illustrates the imaging work-up of patients with CTEPH amenable to BPA, the imaging findings observed before, during and after BPA, and provides a detailed description of all imaging modalities available for CTEPH evaluation.
Background
Portopulmonary hypertension (PoPH) is a rare condition with poorer survival compared to idiopathic/familial pulmonary arterial hypertension (IPAH/FPAH).
Aims
To compare the ...characteristics, survival, prognostic factors and management of PoPH and IPAH/FPAH patients and to assess the impact of treatment on survival of PoPH patients.
Methods
Analysis of data of prevalent and incident PoPH patients enrolled in the Spanish registry of PAH (REHAP) from January 1998 to December 2017 and comparison with IPAH/FPAH patients. Variables analysed: patient and disease (PAH and liver) characteristics, first‐line PAH‐targeted therapy, causes of death, prognostic factors and survival (according to aetiology and treatment in PoPH patients).
Results
Compared to IPAH/FPAH patients (n = 678), patients with PoPH (n = 237) were predominantly men, older and had better functional class and higher prevalence of ascites. Haemodynamics were better. Biomarkers for heart failure were worse. Age‐ and sex‐adjusted 5‐year survival rate from diagnosis was 49.3% for PoPH patients and 68.7% for IPAH patients (P < 0.001). Treated PoPH had better survival than non‐treated. PAH‐ and liver‐related causes accounted for 30.2% and 24.7% of deaths in PoPH patients. PoPH patients were less likely to receive first‐line PAH‐targeted therapy and this was associated with greater mortality. Increasing age, worse exercise capacity and ascites were independent prognostic factors of poorer survival; first‐line oral monotherapy was associated with improved survival. Eight (3.4%) PoPH patients underwent liver transplantation.
Conclusions
PoPH patients are undertreated and show poorer survival than IPAH/FPAH patients. First‐line treatment with PAH‐targeted therapy was associated with better survival. Presence of ascites was a predictor of mortality.
ObjectivesFishermen who had participated in clean-up activities of the Prestige oil spill showed an excess risk of respiratory symptoms 1–2 years later, but the long-term persistence of these health ...effects is unclear. The aim of this study was to evaluate the persistence of these respiratory symptoms 5 years after clean-up work.MethodsSubgroups of 501 fishermen who had been exposed to clean-up work and 177 non-exposed individuals were re-interviewed by telephone in 2008, including the same symptom questions as in the initial survey. Associations between participation in clean-up work and respiratory symptoms were assessed using log-binomial and multinomial regression analyses adjusting for sex, age and smoking.ResultsInformation from 466 exposed (93%) and 156 non-exposed (88%) fishermen was obtained. The prevalence of lower respiratory tract symptoms (including wheeze, shortness of breath, cough and phlegm) had slightly decreased in both groups, but remained higher among the exposed (RR 1.4, 95% CI 1.1 to 1.9). The risk of having persistent respiratory symptoms (reported both at baseline and at follow-up) increased with the degree of exposure: RR ratio 1.7 (95% CI 0.9 to 3.1) and 3.3 (95% CI 1.8 to 6.2) for moderately and highly exposed, respectively, when compared with those without any symptoms. Findings for nasal symptoms and for respiratory medication usage were similar.ConclusionsParticipation in clean-up activities of oil spills may result in respiratory symptoms that persist up to 5 years after exposure. Guidelines for preventive measures and a continued surveillance of clean-up workers of oil spills are necessary.
The wreckage of the oil tanker Prestige in November 2002 produced heavy contamination off the coast of Galicia, Spain.
To evaluate the prevalence of respiratory symptoms in local fishermen more than ...1 year after having participated in clean-up work.
Questionnaires including qualitative and quantitative information about clean-up activities and respiratory symptoms were distributed among associates of 38 fishermen's cooperatives. Both postal and telephone follow-up was performed. The association between participation in clean-up work and respiratory symptoms was evaluated using multiple logistic regression analyses, adjusted for sex, age, and smoking status.
Between January 2004 and February 2005, data were obtained from 6,780 fishermen (response rate, 76%). Sixty-three percent had participated in clean-up operations. Lower respiratory tract symptoms (LRTS) were more prevalent in clean-up workers: odds ratio (OR), 1.73; 95% confidence interval (CI), 1.54-1.94. This association was consistent for men and women, for different fishermen's cooperatives, and for different types of respiratory symptoms, and remained after excluding those who reported anxiety or believed that the oil spill had affected their health (OR, 1.57; 95% CI, 1.37-1.80). The risk of LRTS increased with the number of exposed days, exposed hours per day, and number of activities (linear trend, P < 0.0001). The excess risk of LRTS decreased when more time had elapsed since last exposure: OR, 2.33, 1.69, and 1.24 for less than 14 months, 14-20 months, and more than 20 months, respectively.
Participation in clean-up work of oil spills may result in prolonged respiratory symptoms that last 1 to 2 years after exposure.
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The impact of pulmonary hypertension (PH) on exercise tolerance in chronic obstructive pulmonary disease (COPD) has not been fully elucidated. It is necessary to characterize ...pulmonary hemodynamics in patients with moderate to severe COPD in order to improve their management. The aim of the study was to determine whether in COPD the presence of PH is associated with reduced exercise tolerance in a cohort of stable COPD patients.
Cross-sectional analysis of 174 COPD patients clinically stable: 109 without PH and 65 with PH (COPD-PH). We assessed socio-demographic data, lung function, quality of life, dyspnea, cardiopulmonary exercise testing (CPET), constant workload endurance time (CWET), and six-minute walk test (6MWT). We elaborated a logistic regression model to explore the impact of PH on exercise capacity in COPD patients.
COPD-PH patients showed lower exercise capacity both at maximal (CPET) (43(20) versus 68(27) Watts and 50(19)% versus 71(18)% predicted peak oxygen consumption (VO2peak), COPD-PH and COPD, respectively), and at submaximal tests (6MWT) (382(94) versus 486(95) m). In addition, the COPD-PH group had lower endurance time than the non-PH COPD group (265(113) s and 295(164) s, respectively).
The presence of PH is an independent factor that impairs exercise capacity in COPD.
El impacto de la hipertensión pulmonar (HTP) en la tolerancia al ejercicio en la enfermedad pulmonar obstructiva crónica (EPOC) no se ha dilucidado en su totalidad. Es necesario caracterizar la hemodinámica pulmonar de los pacientes con EPOC moderada a grave para poder mejorar su manejo. El objetivo de este estudio fue determinar si la presencia de HTP en la EPOC se asociaba con una disminución en la tolerancia al ejercicio en una cohorte de pacientes con EPOC estable.
Estudio transversal de 174 pacientes con EPOC clínicamente estables: 109 de ellos no mostraban HTP y 65 de ellos sí (EPOC-HTP). Valoramos la información sociodemográfica, la función pulmonar, la calidad de vida, la disnea, realizamos una prueba de ejercicio cardiopulmonar (PECP), medimos el tiempo de tolerancia de ejercicio constante y realizamos de marcha de seis minutos (6MWT, por sus siglas en inglés). Elaboramos un modelo de regresión logística para explorar el impacto de la HTP en la capacidad de ejercicio de los pacientes con EPOC.
Los pacientes con EPOC-HTP mostraron una menor capacidad de ejercicio, tanto en las pruebas máximas (PECP) (43(20)W frente a 68(27)W y 50(19)% frente a 71(18)% de consumo de oxígeno máximo predicho (VO2max), para pacientes con EPOC-HTP y pacientes con EPOC, respectivamente) como en las pruebas submáximas (6MWT) (382(94)m frente a 486(95)m). Además, el grupo de EPOC-HTP presentó un menor tiempo de resistencia que el grupo de EPOC sin HTP (265(113)s y 295(164)s, respectivamente).
La presencia de HTP es un factor independiente que afecta a la capacidad de ejercicio en la EPOC.
Previous studies have reported mortality rates of up to 56% associated with pregnancy in pulmonary arterial hypertension (PAH) but the management of this disease has changed considerably in recent ...years. We compiled a multinational, prospective registry to examine the contemporary outcome of pregnancies in patients with PAH. During a 3-yr period, the 13 participating centres reported 26 pregnancies. Three (12%) females died and one (4%) developed right heart failure requiring urgent heart-lung transplantation. There were eight abortions; two spontaneous and six induced. 16 (62%) pregnancies were successful, i.e. the females delivered healthy babies without complications. These females had well controlled PAH (pulmonary vascular resistance (PVR) 500 ± 352 dyn·s·cm(-5)); eight of them were long-term responders to calcium channel blockers. In contrast, the females who died or required transplantation had poorly controlled PAH (PVR 1,667 ± 209 dyn·s·cm(-5)). Pregnancy remains associated with a substantial mortality rate in PAH. However, our results indicate that the outcome of pregnancy in PAH has improved, at least when PAH is well controlled, and particularly in long-term responders to calcium channel blockers. These data must be confirmed by larger series before the general recommendation to avoid pregnancy in all patients with PAH is reconsidered.
Background and objective
Exercise capacity decline is a predictor of mortality in patients with chronic obstructive pulmonary disease (COPD). Static pulmonary hyperinflation is a key determinant of ...exercise performance, but its effect on the longitudinal decline in exercise capacity remains unknown. We aimed to study the relationship between the inspiratory capacity‐to‐total lung capacity (IC/TLC) ratio and exercise capacity decline in COPD.
Methods
We measured IC/TLC and other relevant clinical and functional variables in 342 clinically stable patients with COPD. The 6‐min walk distance (6MWD) was determined at recruitment and after a mean ± SD of 1.7 ± 0.3 years. The annual rate of change in 6MWD was calculated. Multiple imputation to account for losses during follow up was implemented, and multivariate regression was used to analyze predictive factors of 6MWD decline.
Results
Mean decline rate in the 6MWD was 21.9 ± 34.1 m/year. In the bivariate analysis, patients with lower levels of IC/TLC had greater 6MWD decline (−27.4 ± 42.5, −24.9 ± 36.5 and −13.4 ± 39.9 m/year in the first, second and third tertile of IC/TLC, respectively; P‐for‐trend = 0.018). From other potential risk factors considered, dyspnoea, health status, serum C‐reactive protein and Borg dyspnoea score at the end of the exercise test were related to exercise capacity decline. In the multivariate regression model, only IC/TLC (β = 0.7 m/year per each percentage unit of IC/TLC; P = 0.007) and dyspnoea (mMRC ≥ 2) (β = −14.6 m/year; P = 0.013) were associated with the annual rate of 6MWD change.
Conclusion
IC/TLC and dyspnoea in clinically stable patients with COPD predict their exercise capacity decline and may help to guide early therapeutic interventions.
In clinically stable patients with COPD, IC/TLC and dyspnoea, but not forced spirometric parameters, predict exercise capacity decline. This may help to screen candidates for early therapeutic interventions, including pulmonary rehabilitation.
The aims of this study were to describe the evolution of acute hepatitis C virus (HCV) infections since 2004 and to determine its associated factors. Acute HCV infections diagnosed in Barcelona from ...2004 to 2015 were included. Incidence ratios (IR) were then estimated for sex and age groups. Cases were grouped between 2004-2005, 2006-2011 and 2012-2015, and their incidence rate ratios (IRR) were calculated. In addition, risk factors for acute HCV infection were identified using multinomial logistic regression for complete, available and multiple imputed data. 204 new HCV cases were identified. Two peaks of higher IR of acute HCV infection in 2005 and 2013 were observed. Men and those aged 35-54 had higher IR. IRR for men was 2.9 times greater than in women (95% confidence intervals (CI): 1.8 ‒ 4.7). Factors related to the period 2012-2015 (versus 2006-2011) were: a) sexual risk factor for transmission versus nosocomial (relative-risk ratio (RRR): 13.0; 95% CI: 2.3 ‒ 72.1), b) higher educated versus lower (RRR: 5.4; 95% CI: 1.6 ‒ 18.7), and c) HIV co-infected versus not HIV-infected (RRR: 53.1; 95% CI: 5.7 ‒ 492.6). This is one of the few studies showing IR and RRRs of acute HCV infections and the first focused on a large city in Spain. Sexual risk for transmission between men, higher educational level and HIV co-infection are important factors for understanding current HCV epidemic. There has been a partial shift in the pattern of the risk factor for transmission from nosocomial to sexual.
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