A small proportion of patients with chronic obstructive pulmonary disease (COPD) patients present severe pulmonary hypertension (PH), defined by mean pulmonary artery pressure (mPAP) ≥35 mm Hg ...measured by right heart catheterization. Little is known about the characteristics of severe PH-COPD. The aim of the study based on a national registry was to describe this phenotype.
We prospectively included and followed patients with incident PH-COPD. Clinical, functional, hemodynamic data at inclusion and follow-up were retrieved. Survival assessed by Kaplan-Meier analysis was the primary end-point.
From 2012 to 2016, 99 patients from 13 French centers were included in the study (82 males; median age 66.0 years interquartile range 62.0-72.0). At inclusion, most patients had marked dyspnea (55.6% and 22.2% New York Heart Association class III and IV, respectively). During 12 months before inclusion, 42.9% had an exacerbation requiring a hospitalization. Pulmonary function tests showed a moderate obstructive pattern with median (interquartile range) FEV1 50.0 35.0-63.0 % predicted and low diffusing capacity for carbon monoxide, median 20.0 16.5-30.6 % predicted. The median values for PaO2 and PaCO2 on room air were 50.0 44.8-62.0 and 36.0 31.1-43.0 mm Hg. Median values of mPAP, pulmonary artery occlusion pressure, cardiac index and pulmonary vascular resistance were 42.0 37.0-48.0 mm Hg, 11.0 9.0-14.0 mm Hg, 3.0 2.4-3.6 L/min/m2, and 6.3 4.2-7.9 WU, respectively. Mean restricted survival was 15.0 13.9-16.0 months.
Severe PH-COPD is characterized by moderate airway obstruction but marked dyspnea and marked hypoxemia, low DLCO and high mPAP. This phenotype is associated with poor prognosis.
Drugs approved for the treatment of pulmonary arterial hypertension (PAH) improve long-term outcomes. These drugs have pulmonary vasodilator properties which may potentially cause a decrease in ...arterial oxyhaemoglobin saturation (SaO
) in some patients.
The present retrospective study of the French PAH Registry aimed to describe clinical characteristics and outcomes of patients showing a ≥3% decrease in SaO
while treated with PAH drugs.
We reviewed 719 PAH patients. The exclusion criteria were PAH associated with congenital heart disease and PAH with overt features of venous/capillaries involvement.
One hundred and seventy-three (24%) patients had a ≥3% decrease in SaO
. At diagnosis, they were older, with a lower diffusion capacity for carbon monoxide and a shorter 6-minute walk distance, when compared to those who did not display a ≥3% decrease in SaO
. The percentage of patients meeting the ESC/ERS low risk criteria at re-evaluation was significantly lower in those with a ≥3% decrease in SaO
and more patients started long-term oxygen therapy in this group (16%
5%, p<0.001). A≥3% decrease in SaO
was associated with a poorer survival (Hazard Ratio 1.81:95% confidence interval 1.43-2.34; p<0.0001). In a multivariate Cox analysis, a ≥3% decrease in SaO
was a prognostic factor independent of age at diagnosis and ESC/ERS risk stratification at follow-up.
When treated with PAH drugs, a large subset of patients experience a≥3% decrease in SaO
, which is associated with worst long-term outcomes and reduced survival.
Abstract
Background
No large, prospective study has investigated respiratory symptoms in patients with inflammatory bowel diseases. We aimed to describe the prevalence of and factors associated with ...respiratory symptoms in patients with inflammatory bowel disease.
Methods
In an observational, prospective, cross-sectional study, we evaluated the frequency of respiratory symptoms using a validated self-reporting questionnaire from February 2019 to February 2021 during routine follow-up outpatient visits of patients with inflammatory bowel disease followed in the Gastroenterology Department of the Nancy University Hospital. In case of a positive questionnaire, patients were systematically offered a consultation with a pulmonologist in order to investigate a potential underlying respiratory disease.
Results
There were 325 patients included, and 180 patients had a positive questionnaire (144 with Crohn’s disease). Of the included patients, 165 (50.8%) presented with respiratory symptoms, with dyspnea being the most frequent symptom (102 patients). There were 102 patients (56.7%) who benefited from a consultation in the pulmonology department: 43 (42.2%) were diagnosed with a respiratory disease, mainly asthma (n = 13) or chronic obstructive pulmonary disease (n = 10). Fourteen patients (13.7%) had obstructive sleep apnea. A body mass index increase, being a smoker or ex-smoker, and having articular extra-intestinal manifestations were independently associated with a higher prevalence of respiratory symptoms.
Conclusions
Half of patients with inflammatory bowel disease reported respiratory symptoms in our study. Patients with inflammatory bowel disease should be systematically screened, as pulmonary disease is frequently present in this population, with specific attention being given to smokers or ex-smokers and patients with extra-articular intestinal manifestations.
Lay Summary
We conducted a large-scale, prospective study, finding a high prevalence of respiratory symptoms in patients with inflammatory bowel disease, which led to a variety of respiratory diseases, including asthma and obstructive pulmonary disease. Patients should therefore be systematically screened for pulmonary manifestations.
Bone morphogenetic proteins 9 and 10 (BMP9 and BMP10), encoded by
and
, respectively, play a pivotal role in pulmonary vascular regulation.
variants have been reported in pulmonary arterial ...hypertension (PAH) and hereditary haemorrhagic telangiectasia (HHT). However, the phenotype of
and
carriers remains largely unexplored.
We report the characteristics and outcomes of PAH patients in
and
carriers from the French and Dutch pulmonary hypertension registries. A literature review explored the phenotypic spectrum of these patients.
26 PAH patients were identified: 20 harbouring heterozygous
variants, one homozygous
variant, four heterozygous
variants, and one with both
and
variants. The prevalence of
and
variants was 1.3% and 0.4%, respectively. Median age at PAH diagnosis was 30 years, with a female/male ratio of 1.9. Congenital heart disease (CHD) was present in 15.4% of the patients. At diagnosis, most of the patients (61.5%) were in New York Heart Association Functional Class III or IV with severe haemodynamic compromise (median (range) pulmonary vascular resistance 9.0 (3.3-40.6) WU). Haemoptysis was reported in four patients; none met the HHT criteria. Two patients carrying
variants underwent lung transplantation, revealing typical PAH histopathology. The literature analysis showed that 7.6% of
carriers developed isolated HHT, and identified cardiomyopathy and developmental disorders in
carriers.
and
pathogenic variants are rare among PAH patients, and occasionally associated with CHD. HHT cases among
carriers are limited according to the literature.
full phenotypic ramifications warrant further investigation.
We report the case of a 71-year-old man bearing a severe right-to-left shunt through a patent foramen ovale in the absence of elevated right-sided heart or pulmonary artery pressures. He presented ...with platypnea-orthodeoxia syndrome, but he had no pulmonary or extracardiac diseases that are known to be associated with this syndrome. Chest radiography showed a bulky aneurysm of the thoracic aorta. A peripheral contrast transesophageal echocardiography demonstrated a large right-to-left shunt through a patent foramen ovale. In addition, the atrial septum was severely deformed by an aneurysm including this patent foramen ovale. We hypothesized that the opening of the foramen ovale was the result of a mechanical deformation of the atrial septum by two contributing factors: the aneurysm of the thoracic aorta and the aneurysm of the septum itself.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Severe pulmonary hypertension occurs occasionally in patients with chronic obstructive pulmonary disease (COPD), but no detailed description of these patients is available.
To identify and ...characterize patients with COPD and severe pulmonary hypertension.
Retrospective study of 27 patients with COPD with severe pulmonary hypertension (pulmonary artery mean pressure Ppa, > or = 40 mm Hg) among 998 patients who underwent right heart catheterization between 1990 and 2002 as part of a workup for chronic respiratory failure during a period of disease stability.
Of the 27 patients, 16 had another disease capable of causing pulmonary hypertension. The remaining 11 (11 of 998, 1.1%) patients had COPD as the only cause of pulmonary hypertension, with a median Ppa of 48 mm Hg (interquartile range, 46-50). They had an unusual pattern of cardiopulmonary abnormalities with mild to moderate airway obstruction, severe hypoxemia, hypocapnia, and a very low diffusing capacity for carbon monoxide (p < 0.01 compared with a control group of patients with COPD). Exertional dyspnea was more severe (p < 0.01) and survival was shorter (p = 0.0026) than in the control subjects.
Severe pulmonary hypertension is uncommon in patients with COPD. When it occurs, another cause must be sought. COPD with severe pulmonary hypertension and no other possible cause shares features with pulmonary vascular diseases, such as idiopathic pulmonary hypertension.
To date, nothing is known about the evolution of survival in systemic sclerosis-associated pulmonary arterial hypertension (PAH) over the last decade.
This study used a multivariate Cox regression ...model adjusted for clinically relevant baseline confounders to assess the association between the occurrence of death and date of PAH diagnosis comparing two periods of the same duration (2006-2011 vs 2012-2017). Interactions between the two diagnosis periods and baseline variables were tested.
A total of 306 incident patients were included, 167 (54.6%) with a PAH diagnosis occurring in 2006 to 2011 and 139 (45.4%) in 2012 to 2017. No significant difference in survival was observed between patients diagnosed with PAH in 2012 to 2017 compared with those diagnosed in 2006 to 2011 (hazard ratio HR, 0.76 0.46-1.26; P = .29). A significant interaction was observed between PAH diagnosis periods and age (P = .05). When stratifying according to age (based on the median age of 70 years), a significant increase was observed in survival in patients aged ≤ 70 years between the 2006 to 2011 period and the 2012 to 2017 period (HR, 0.40 0.17-0.99; P = .046) but not in older patients (HR, 1.29 0.67-2.51; P = .44). A significantly higher proportion of initial (ie, within the first 4 months) endothelin receptor antagonist/phosphodiesterase type 5 inhibitor combination therapy was observed in younger patients diagnosed from 2012 to 2017 vs those diagnosed from 2006 to 2011 (42.9% vs 19.5%; P = .002) but not in older patients.
Over the period 2006 to 2017, survival in systemic sclerosis-associated PAH improved over time in patients aged ≤ 70 years but not in older patients. Further investigations are needed to confirm this relation, as general improvement in medical care and management may also be a possible explanation.
Few questionnaires are available for routine assessment of dyspnea. The study aimed to design a self-administered questionnaire assessing the impact of chronic dyspnea on daily activities, named ...DYSLIM (Dyspnea-induced Limitation).
The development followed 4 steps: 1: selection of relevant activities and related questions (focus groups); 2: clinical study: internal and concurrent validity vs. modified Medical Research Council (mMRC), Baseline Dyspnea Index (BDI) and Saint George Respiratory Questionnaire (SGRQ); 3: item reduction; 4: responsiveness.
Eighteen activities (from eating to climbing stairs) were considered with 5 modalities for each: doing the task slowly, taking breaks, seeking assistance, changing habits, and activity avoidance. Each modality was graded from 5 (never) to 1 (very often). Validation study included 194 patients: COPD (FEV1 ≥ 50% pred: n = 40; FEV1 < 50% pred: n = 65); cystic fibrosis (n = 30), interstitial lung disease (n = 30), pulmonary hypertension (n = 29). Responsiveness was evaluated by post-pulmonary rehabilitation data in 52 COPD patients.
Acceptability was high and short term (7 days) reproducibility was satisfactory (Kappa mostly above 0.7). Concurrent validity was high vs. mMRC (Spearman correlation coefficient, r = 0.71), BDI (r = - 0.75) and SGRQ (r = - 0.79). The reduced questionnaire with 8 activities (from cleaning to climbing stairs) and 3 modalities (slowly, seeking help, changing habits) showed a comparable validity and was chosen as the final short version. Effect size of rehabilitation was good for both the full (0.57) and short (0.51) versions. A significant correlation was also found between changes of SGRQ and DYSLIM post rehabilitation: r = - 0.68 and r = - 0.60 for full and reduced questionnaires, respectively.
The DYSLIM questionnaire appears promising for the evaluation of dyspnea-induced limitations in chronic respiratory diseases and seems suitable for use in various contexts.
•Dyslim is a self-administered questionnaire assessing dyspnea-induced limitation.•Development of the questionnaire followed modern psychometric rules.•Dyslim is unidimensional, reproducible, responsive, with a high concurrent validity.•The short version comprises 8 questions with 3 modalities, requiring less than 3 min.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPUK, ZAGLJ, ZRSKP
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