Differences of cardiac output measurements by open-circuit acetylene uptake in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: a cohort study

<p>Abstract</p> <p>Background</p> <p>As differences in gas exchange between pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) have been demonstrated, we asked if cardiac output measurements determined by acetylene (C₂H₂) uptake significantly differed in these diseases when compared to the thermodilution technique.</p> <p>Method</p> <p>Single-breath open-circuit C₂H₂uptake, thermodilution, and cardiopulmonary exercise testing were performed in 72 PAH and 32 CTEPH patients.</p> <p>Results</p> <p>In PAH patients the results for cardiac output obtained by the two methods showed an acceptable agreement with a mean difference of -0.16 L/min (95% CI -2.64 to 2.32 L/min). In contrast, the agreement was poorer in the CTEPH group with the difference being -0.56 L/min (95% CI -4.96 to 3.84 L/min). Functional dead space ventilation (44.5 ± 1.6 vs. 32.2 ± 1.4%, p < 0.001) and the mean arterial to end-tidal CO₂gradient (9.9 ± 0.8 vs. 4.1 ± 0.5 mmHg, p < 0.001) were significantly elevated among CTEPH patients.</p> <p>Conclusion</p> <p>Cardiac output evaluation by the C₂H₂technique should be interpreted with caution in CTEPH, as ventilation to perfusion mismatching might be more relevant than in PAH.</p

Successful long-term treatment of persistent pulmonary air leak in pneumocystis jirovecii pneumonia by unidirectional endobronchial valves

Spontaneous pneumothorax is a rare complication of pneumocystis jirovecii pneumonia. We report a patient with pneumocystis jirovecii pneumonia and therapy-refractory, right-sided pneumothorax due to persistent air leak (PAL) despite prolonged chest tube placement and multiple pleurodesis attempts. Due to the patient's morbidity, we evaluated if the PAL can be sealed by unidirectional endobronchial valves (EBVs). After occlusion of the right upper lobe by a balloon catheter, the air leak flow-rate decreased from 800 ml/min to 250 ml/min. Zephyr EBVs (ZEBVs) were placed in the segmental right upper lobe bronchi and subsequently, a complete resolution of the pneumothorax was noted. During 30 months of follow-up, neither recurrence of pneumothorax nor any adverse events of EBV treatment were noted. We conclude that ZEBV placement might be an effective and well-tolerated treatment option for PAL secondary to pneumocystis jirovecii pneumonia with promising long-term results

The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry

Background: Quality of life (QoL) is profoundly impaired in patients with idiopathic pulmonary fibrosis (IPF). However, data is limited regarding the course of QoL. We therefore analysed longitudinal data from the German INSIGHTS-IPF registry. Methods: Clinical status and QoL were assessed at enrollment and subsequently at 6- to 12-months intervals. A range of different QoL questionnaires including the St. George’s Respiratory Questionnaire (SGRQ) were used. Results: Data from 424 patients were included; 76.9% male; mean age 68.7 ± 9.1 years, mean FVC% predicted 75.9 ± 19.4, mean DLCO% predicted 36.1 ± 15.9. QoL worsened significantly during follow-up with higher total SGRQ scores (increased by 1.47 per year; 95% CI: 1.17 to 1.76; p &lt; 0.001) and higher UCSD-SOBQ scores and lower EQ-5D VAS and WHO-5 scores. An absolute decline in FVC% predicted of &gt; 10% was associated with a significant deterioration in SGRQ (increasing by 9.08 units; 95% CI: 2.48 to 15.67; p = 0.007), while patients with stable or improved FVC had no significantly change in SGRQ. Patients with a &gt; 10% decrease of DLCO % predicted also had a significant increase in SGRQ (+ 7.79 units; 95% CI: 0.85 to 14.73; p = 0.028), while SQRQ was almost stable in patients with stable or improved DLCO. Patients who died had a significant greater increase in SGRQ total scores (mean 11.8 ± 18.6) at their last follow-up visit prior to death compared to survivors (mean 4.2 ± 18.9; HR = 1.03; 95% CI: 1.01 to 1.04; p &lt; 0.001). All QoL scores across the follow-up period were significantly worse in hospitalised patients compared to non-hospitalised patients, with the worst scores reported in those hospitalised for acute exacerbations. Conclusions: QoL assessments in the INSIGHTS-IPF registry demonstrate a close relationship between QoL and clinically meaningful changes in lung function, comorbidities, disease duration and clinical course of IPF, including hospitalisation and mortality

Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry

Background: The INSIGHTS-IPF registry provides one of the largest data sets of clinical data and self-reported patient related outcomes including health related quality of life (QoL) on patients with idiopathic pulmonary fibrosis (IPF). We aimed to describe associations of various QoL instruments between each other and with patient characteristics at baseline. Methods: Six hundred twenty-three IPF patients with available QoL data (St George's Respiratory Questionnaire SGRQ, UCSD Shortness-of-Breath Questionnaire SoB, EuroQol visual analogue scale and index EQ-5D, Well-being Index WHO-5) were analysed. Mean age was 69.6 +/- 8.7 years, 77% were males, mean disease duration 2.0 +/- 3.3 years, FVC pred was 67.5 +/- 17.8%, DLCO pred 35.6 +/- 17%. Results: Mean points were SGRQ total 48.3, UCSD SoB 47.8, EQ-5D VAS 66.8, and WHO-5 13.9. These instruments had a high or very high correlation (exception WHO-5 to EQ-5D VAS with moderate correlation). On bivariate analysis, QoL by SGRQ total was statistically significantly associated with clinical symptoms (NYHA;p < 0.001), number of comorbidities (p < 0.05), hospitalisation rate (p < 0.01) and disease severity (as measured by GAP score, CPI, FVC and 6-min walk test;p < 0.05 each). Multivariate analyses showed a significant association between QoL (by SGRQ total) and IPF duration, FVC, age, NYHA class and indication for long-term oxygen treatment. Conclusions: Overall, IPF patients under real-life conditions have lower QoL compared to those in clinical studies. There is a meaningful relationship between QoL and various patient characteristics

Drug induced interstitial lung disease

With an increasing number of therapeutic drugs, the list of drugs that is responsible for severe pulmonary disease also grows. Many drugs have been associated with pulmonary complications of various types, including interstitial inflammation and fibrosis, bronchospasm, pulmonary edema, and pleural effusions. Drug-induced interstitial lung disease (DILD) can be caused by chemotherapeutic agents, antibiotics, antiarrhythmic drugs, and immunosuppressive agents. There are no distinct physiologic, radiographic or pathologic patterns of DILD, and the diagnosis is usually made when a patient with interstitial lung disease (ILD) is exposed to a medication known to result in lung disease. Other causes of ILD must be excluded. Treatment is avoidance of further exposure and systemic corticosteroids in patients with progressive or disabling disease

Ventilatory efficiency testing as prognostic value in patients with pulmonary hypertension

Abstract Background Increased ventilatory response has been shown to have a high prognostic value in patients with chronic heart failure. Our aim was therefore to determine the ventilatory efficiency in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension by cardiopulmonary exercise testing (CPET) identifying PH-patients with increased risk for death within 24 months after evaluation. Methods 116 patients (age: 64 ± 1 years) with a mean pulmonary arterial pressure of 35 ± 1 mmHg underwent CPET and right heart catheterization. During a follow-up of 24 months, we compared the initial characteristics of survivors (n = 87) with nonsurvivors (n = 29). Results Significant differences (p ≤ 0.005) between survivors and nonsurvivors existed in ventilatory equivalents for oxygen (42.1 ± 2.1 versus 56.9 ± 2.6) and for carbon dioxide (Ve/VCO2) (47.5 ± 2.2 versus 64.4 ± 2.3). Patients with peak oxygen uptake ≤ 10.4 ml/min/kg had a 1.5-fold, Ve/VCO2 ≥ 55 a 7.8-fold, alveolar-arterial oxygen difference ≥ 55 mmHg a 2.9-fold, and with Ve/VCO2 slope ≥ 60 a 5.8-fold increased risk of mortality in the next 24 months. Conclusions Our results demonstrate that abnormalities in exercise ventilation powerfully predict outcomes in PH. Consideration should be given to add clinical guidelines to reflect the prognostic importance of ventilatory efficiency parameters in addition to peak VO2.</p