Angiosarcoma is a rare malignant vascular tumor. Pulmonary involvement is usually attributable to metastasis from other primary sites, primary pulmonary angiosarcoma therefore being quite uncommon. We report a case of angiosarcoma with pulmonary involvement, probably primary to the lung, which had gone untreated for more than two years. We describe this rare neoplasm and its growth, as well as the extensive local invasion and hematogenous metastasis at presentation. We also discuss its poor prognosis.

Coexistence between pulmonary cancer and chronic obstructive pulmonary disease (COPD) is frequent and is anticipated to be lead to high worldwide mortality in the next decades. The most powerful therapeutic approach for non-small cell lung carcinoma is lung surgical resection. However, in COPD patients, this approach bears a higher mortality and morbidity risk, thus requiring an accurate pre-operatory evaluation of the surgical risk comprising a clinical and functional assessment at rest, as well as a cardiopulmonary exercise test. In this observational study, factors associated with cardiopulmonary complications within 30 days after tumor resection surgery were investigated in a cohort of patients with COPD and lung cancer assigned to perform a cardiopulmonary exercise test. This study included 50 patients (46 men, 92.0%) with a mean age of 64.7 years old (standard deviation 7.9), forced expiratory volume in the first second (FEV1) of 61.8% (SD 19.0%) and carbon monoxide diffusing capacity (DLCO) of 46.0% (SD 14.8%). Complications were observed in eighteen patients (36.0%) including 2 deaths (4.0%). Peak oxygen uptake (VO_2peak) expressed in percentage of the predicted value was the only parameter showing a statistically significant difference between the groups with and without complications (p = 0.027). The best value of VO_2peak to discriminate complications occurrence was 61.0%. This study highlights the relevance of the cardiopulmonary exercise test in the risk assessment of pulmonary resection surgery in patients with COPD. The VO_2peak (percentage of predicted value) is shown to be associated with complications within 30 days after surgery.

Background: Home noninvasive ventilation (NIV) has been increasingly used in stable chronic obstructive pulmonary disease (COPD) with chronic hypercapnic respiratory failure (CHRF). However its effectiveness remains debatable. Aim: To describe a follow-up of COPD patients under home NIV. Methods: Retrospective descriptive study based on a prospective 3-year database that included COPD patients under home NIV between August 2011 and July 2014. Results: Within the 334 patients initially screened, 109 (32.6%) had COPD with a mean ± SD post-bronchodilator FEV1 of 38.6 ± 14.9% predicted; age of 65.6 ± 9.6 years. The mean ± SD duration of ventilation was 63.4 ± 51.1 months. Heterogeneous comorbidities that can contribute to CHRF were not excluded: obstructive sleep apnea and obesity were the most prevalent. Sixty-two (56.9%) patients started NIV during admission with acute respiratory failure. During follow-up there was a significant increase in mean inspiratory positive airway pressure (IPAP) and respiratory rate (19.5 ± 4.4 vs. 23.6 ± 5.3 cmH₂O and 10.7 ± 5.2 vs. 15.2 ± 1.4 breaths/min, respectively, p < 0.0001), with a significant improvement in hypercapnia (PaCO₂: 52.9 ± 7.7 vs. 49.5 ± 7.5 mmHg, p < 0.0001), with 93.3% of patients compliant to NIV. Admissions and days spent in hospital for respiratory illness significantly decreased after institution of NIV (respectively, 1.2 ± 1.1 vs. 0.7 ± 1.8 and 15.0 ± 16.8 vs. 8.8 ± 19.4, p < 0.001). At final evaluation, patients with severe hypercapnia (n = 47; PaCO₂ ≥50 mmHg) performing NIV at higher pressures (n = 30; IPAP ≥25 cmH₂O) were more compliant (10.1 ± 3.3 vs. 6.1 ± 3.6 h/day). Three-year mortality was 24.8% (27 of 109 patients). Conclusions: This is a real-life retrospective study in COPD patients with CHRF which results suggest benefit from home NIV. For most, NIV was effective and tolerable even at high pressures.

Objective: The identification of persistent airway obstruction is key to making a diagnosis of COPD. The GOLD guidelines suggest a fixed criterion- a post-bronchodilator FEV1/FVC ratio < 70%-to define obstruction, although other guidelines suggest that a post-bronchodilator FEV1/FVC ratio < the lower limit of normal (LLN) is the most accurate criterion. Methods: This was an observational study of individuals >= 4 0 years of age with risk factors for COPD who were referred to our pulmonary function laboratory for spirometry. Respiratory symptoms were also recorded. We calculated the prevalence of airway obstruction and of no airway obstruction, according to the GOLD criterion (GOLD+ and GOLD-, respectively) and according to the LLN criterion (LLN+ and LLN-, respectively). We also evaluated the level of agreement between the two criteria. Results: A total of 241 individuals were included. Airway obstruction was identified according to the GOLD criterion in 42 individuals (17.4%) and according to the LLN criterion in 23 (9.5%). The overall level of agreement between the two criteria was good (kappa = 0.67; 95% CI: 0.52-0.81), although it was lower among the individuals >= 70 years of age (kappa = 0.42; 95% CI: 0.12-0.72). The proportion of obese individuals was lower in the GOLD+/LLN+ category than in the GOLD+/LLN- category (p = 0.03), as was the median DLCO (p = 0.04). Conclusions: The use of the GOLD criterion appears to be associated with a higher prevalence of COPD. The agreement between the GOLD and LLN criteria also appears to be good, albeit weaker in older individuals. The use of different criteria to define airway obstruction seems to identify individuals with different characteristics. It is essential to understand the clinical meaning of discordance between such criteria. Until more data are available, we recommend a holistic, individualized approach to, as well as close follow-up of, patients with discordant results for airway obstruction.

Background: The use of home mechanical ventilation (HMV) has been increasing worldwide, driven by widening of clinical applications and improved patient survival rates. In Portugal, recent data indicate an even faster growth, although national HMV recommendations have remained unchanged for 25 years. Research question: We aimed to provide an update in clinical practice guidelines for HMV in adults, applicable to the Portuguese context, grounded on the latest available evidence and experts' opinion. Study design and methods: A multidisciplinary panel with experience in HMV in the adult population was assembled. A comprehensive literature search was conducted during March 2023 regarding specific topics: equipment, ventilatory modes and interfaces, HMV initiation, follow-up and monitoring, disease specificities (neuromuscular diseases, obesity-hypoventilation syndrome, restrictive chest wall diseases; chronic obstructive pulmonary disease, and other diseases), home mechanical invasive ventilation, and palliative and end of life care. A 2-round Delphi process was conducted to establish consensus among panel members. A minimum agreement threshold of 80% was required. Results: Out of 88 recommendations initially included in the Delphi process, 61 were selected by consensus. Conclusion: Final recommendations grounded in the current level of evidence are outlined, and the key limitations and proposals for future research are discussed.