RESUMO
Acute right ventricular failure secondary to acutely increased right ventricular afterload (acute cor pulmonale) is a life-threatening condition that may arise in different clinical settings. Patients at risk of developing or with manifest acute cor pulmonale usually present with an acute pulmonary disease (e.g. pulmonary embolism, pneumonia, and acute respiratory distress syndrome) and are managed initially in emergency departments and later in intensive care units. According to the clinical setting, other specialties are involved (cardiology, pneumology, internal medicine). As such, coordinated delivery of care is particularly challenging but, as shown during the COVID-19 pandemic, has a major impact on prognosis. A common framework for the management of acute cor pulmonale with inclusion of the perspectives of all involved disciplines is urgently needed.
Assuntos
Cardiologia , Insuficiência Cardíaca , Doença Cardiopulmonar , Humanos , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/etiologia , Doença Cardiopulmonar/terapia , Pandemias , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Ventrículos do CoraçãoRESUMO
Objective: To analyze the combination clinical value of plasma brain natriuretic peptide and serum glycated hemoglobin (HbAlc) in chronic pulmonary heart disease. Methods: A total of 200 patients with chronic pulmonary heart disease admitted to our hospital from January 2021 to January 2022 were selected as the observation group, and 200 healthy subjects were selected as the control group during the same period. All subjects were examined by an ECG vector map and plasma BNP, and HbAlc levels were detected to analyze the value and clinical significance of each index in single diagnosis and combined diagnosis. Results: Plasma BNP and HbAlc levels in the observation group were significantly higher than those in the control group (P < 0.05). There were 154 BNP positive, 146 HbAlc positive, 164 parallel combined diagnosis positive, and 132 serial combined diagnosis positive. Sensitivity of series combination diagnosis was significantly higher than other indexes (P < 0.05); especially, parallel combination diagnosis was significantly higher than other indexes (P < 0.05). Besides, area under the ROC curve of parallel combination diagnosis and series combination diagnosis was significantly higher than that of each index alone diagnosis (P < 0.05). Conclusion: In the diagnosis of chronic pulmonary heart disease, the combination of plasma BNP and HbAlc can effectively improve the diagnostic specificity and sensitivity, as well as improve the area under the ROC curve.
Assuntos
Insuficiência Cardíaca , Doença Cardiopulmonar , Doença Crônica , Insuficiência Cardíaca/diagnóstico , Humanos , Peptídeo Natriurético Encefálico , Doença Cardiopulmonar/diagnóstico , Curva ROC , Sensibilidade e EspecificidadeRESUMO
AIMS AND OBJECTIVES: Patients with pulmonary thromboembolism (PTE) are commonly admitted to hospital and generally have a prolonged hospital stay in this part of the world. We aimed to determine different clinical and laboratory parameters that are associated with prolonged hospital stay in our set-up and to analyse effectiveness of Pulmonary Embolism Severity Index (PESI) score as a predictor of prolonged hospital stay in patients with PTE. MATERIALS AND METHODS: It was a hospital based observational prospective study. Confirmed cases of PTE defined as patients with evidence of thrombus on CT pulmonary angiogram (CTPA) were included in this study. Depending on the length of hospital stay, patients were divided into two cohorts: Shorter Hospital stay (less than mean i.e., < 10 Days) and Prolonged Hospital stay (longer than mean i.e., ≥ 10 Days). Logistic regression analysis was done to identify predictors of prolonged hospital stay. RESULTS: 150 patients were included in the study with 67 patients (44.67%) having shorter hospital stay (<10 days) and 83 patients (55.33%) having prolonged hospital stay (≥10 days). On multivariate regression analysis, parameters that were found to be statistically significant were hypotension at presentation, decreased level of consciousness, pco2 < 30 mmHg, presence of S1Q3T3 pattern on electrocardiogram (ECG) and high risk PESI (class III-V). CONCLUSION: PESI class can be effectively used to predict prolonged hospital stay in patients with pulmonary embolism. Patients with hypotension at presentation, decreased level of consciousness, pco2 less than 30 mmHg, and S1Q3T3 on ECG are more likely to have prolonged hospital stay in our healthcare setup.
Assuntos
Tempo de Internação , Embolia Pulmonar , Doença Aguda , Dióxido de Carbono/sangue , Angiografia por Tomografia Computadorizada , Transtornos da Consciência/etiologia , Eletrocardiografia , Humanos , Hipotensão/etiologia , Estudos Prospectivos , Embolia Pulmonar/sangue , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/terapia , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/etiologia , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de DoençaRESUMO
We studied the effects of heart rate reduction by ivabradine to the ongoing therapy in patients with chronic obstructive pulmonary disease (COPD) and cor pulmonale.100 patients of COPD with cor pulmonale with sinus heart rate ≥ 90 bpm were randomly assigned to either ivabradine 5 mg twice daily (50 patients) or placebo (50 patients) alongwith standard therapy. Assessment was done at baseline and after 6 months which included 6 min walk test (6MWT), dyspnea scoring by modified borg scale, Lung function test by forced expiratory volume in 1 s (FEV1) and pulmonary artery systolic pressure (PASP) by echocardiogram. The drug group showed a significant reduction in heart rate from 95.1 ± 8.2 bpm to 71.1 ± 6.2 bpm (p < 0.001). This group also showed significant improvement in 6-min walk distance and dyspnea on modified Borg scale (p < 0.001) at 6 months follow up. However no significant difference was found between both groups regarding PASP or FEV1 at 6 months.
Assuntos
Ivabradina/uso terapêutico , Doença Pulmonar Obstrutiva Crônica , Doença Cardiopulmonar , Frequência Cardíaca , Humanos , Artéria Pulmonar , Doença Pulmonar Obstrutiva Crônica/complicações , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/tratamento farmacológicoAssuntos
Influenza Humana/complicações , Doença Cardiopulmonar , Respiração Artificial , Síndrome do Desconforto Respiratório , Choque , Bloqueio de Ramo/diagnóstico , Bloqueio de Ramo/etiologia , Pressão Venosa Central , Angiografia por Tomografia Computadorizada/métodos , Ecocardiografia/métodos , Feminino , Humanos , Vírus da Influenza A/isolamento & purificação , Influenza Humana/diagnóstico , Influenza Humana/fisiopatologia , Pessoa de Meia-Idade , Assistência ao Paciente/métodos , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/etiologia , Doença Cardiopulmonar/fisiopatologia , Respiração Artificial/efeitos adversos , Respiração Artificial/métodos , Síndrome do Desconforto Respiratório/etiologia , Síndrome do Desconforto Respiratório/fisiopatologia , Síndrome do Desconforto Respiratório/terapia , Choque/etiologia , Choque/fisiopatologia , Choque/terapiaRESUMO
The pediatric population accounts for a small portion of those with severe disease related to COVID-19. There are few published reports of hypercoagulable states in children with COVID-19. We describe an 11-year-old male with nephrotic syndrome who required inpatient treatment for COVID-19 pneumonia eight weeks prior. He returned to the emergency department with vomiting, tachypnea and was found to have a pulmonary embolism. In this case report, we discuss the risk factors for, presentation and evaluation of hypercoagulable state and its relation to COVID-19 in a pediatric patient.
Assuntos
COVID-19/sangue , Síndrome Nefrótica/complicações , Embolia Pulmonar/diagnóstico , Doença Cardiopulmonar/diagnóstico , Trombofilia/sangue , COVID-19/complicações , Criança , Angiografia por Tomografia Computadorizada , Eletrocardiografia , Fibrinolíticos/uso terapêutico , Heparina/uso terapêutico , Hospitalização , Humanos , Unidades de Terapia Intensiva Pediátrica , Masculino , Readmissão do Paciente , Obesidade Infantil/complicações , Embolia Pulmonar/sangue , Embolia Pulmonar/complicações , Embolia Pulmonar/tratamento farmacológico , Doença Cardiopulmonar/tratamento farmacológico , Doença Cardiopulmonar/etiologia , SARS-CoV-2 , Taquipneia , Trombofilia/complicações , Trombofilia/tratamento farmacológico , Ativador de Plasminogênio Tecidual/uso terapêutico , VômitoRESUMO
Cor pulmonale is a disease of the heart characterised by dilatation of the right ventricle and paradoxical movement of the interventricular septum. The diagnosis depends on echocardiography even if pulmonary artery catheterisation suggests it. It is secondary to pulmonary disease or a disorder of the pulmonary circulation. These two mechanisms, which are often connected, involve pulmonary hypertension as the origin of a systolic and diastolic overload of the right ventricle, which then leads to the alterations of its structure and performance. Acute cor pulmonale is usually secondary to an acute respiratory distress syndrome or to a pulmonary embolism but it can also be seen in primary lactic acidosis, a vaso-occlusive crisis in a patient with sickle cell anaemia, severe acute asthma, and entry of air or injected crushed tablets into the circulation. Chronic cor pulmonale is the terminal stage of pulmonary hypertension. Clinically these patients are dyspnoeic with signs of chronic right heart failure. They should have an echocardiogram confirming the cardiac involvement. Certain precipitating factors, such as infection of any origin, have been reported, leading to acute on chronic cor pulmonale that has a particularly high mortality.
Assuntos
Doença Cardiopulmonar/etiologia , Disfunção Ventricular Direita/complicações , Doença Crônica , Diagnóstico Diferencial , Ecocardiografia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/prevenção & controle , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/etiologia , Embolia Pulmonar/terapia , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/terapia , Síndrome do Desconforto Respiratório/diagnóstico , Síndrome do Desconforto Respiratório/etiologia , Síndrome do Desconforto Respiratório/terapia , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/terapiaAssuntos
Aquaporina 2/metabolismo , Hipertensão Pulmonar/complicações , Rim/metabolismo , Doença Cardiopulmonar/diagnóstico , Animais , Aquaporina 2/urina , Arginina Vasopressina/sangue , Arginina Vasopressina/metabolismo , Biomarcadores/metabolismo , Biomarcadores/urina , Modelos Animais de Doenças , Humanos , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/induzido quimicamente , Hipertensão Pulmonar/urina , Masculino , Monocrotalina/toxicidade , Doença Cardiopulmonar/sangue , Doença Cardiopulmonar/etiologia , Doença Cardiopulmonar/urina , Ratos , Eliminação RenalRESUMO
PURPOSE: Canine and feline cardiopulmonary disease is an emergent condition in several European countries, particularly in non-endemic regions, although it is often underestimated because of the limitations of the common diagnostic techniques. The aim of this study was to evaluate the performance of Mini-FLOTAC to detect cardiopulmonary larvae from dogs and cats compared to reference techniques such as Baermann and FLOTAC. METHODS: A total of 20 fresh faecal samples were collected from ten dogs and ten cats naturally infected with Angiostrongylus vasorum, and Aelurostrongylus abstrusus and Troglostrongylus spp., respectively. Three replicates were performed for each technique. FS3 (zinc sulfate, specific gravity = 1.200) was used as flotation solution for Mini-FLOTAC and FLOTAC. The results were expressed as the arithmetic mean of LPG (larvae per gram of faeces). RESULTS: The mean LPG calculated by means of Mini-FLOTAC was significantly higher (P < 0.05) than that obtained through the Baermann technique for A. vasorum, A. abstrusus and Troglostrongylus spp., whereas it was significantly higher (P < 0.05) than that obtained through the FLOTAC technique only for A. vasorum and A. abstrusus. CONCLUSIONS: Mini-FLOTAC can be considered a valid alternative for the detection of metastrongyloid larvae of dogs and cats, overcoming the limitation of time required by the Baermann test.
Assuntos
Doenças do Gato/parasitologia , Doenças do Cão/parasitologia , Metastrongyloidea/isolamento & purificação , Doença Cardiopulmonar/veterinária , Infecções por Strongylida/veterinária , Angiostrongylus/isolamento & purificação , Animais , Doenças do Gato/diagnóstico , Gatos , Diagnóstico Diferencial , Doenças do Cão/diagnóstico , Cães , Fezes/parasitologia , Larva , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/parasitologia , Infecções por Strongylida/diagnóstico , Infecções por Strongylida/parasitologiaAssuntos
Acalasia Esofágica/diagnóstico , Doença Cardiopulmonar/diagnóstico , Diagnóstico Diferencial , Ecocardiografia , Acalasia Esofágica/complicações , Acalasia Esofágica/diagnóstico por imagem , Acalasia Esofágica/cirurgia , Feminino , Miotomia de Heller , Humanos , Pessoa de Meia-Idade , Doença Cardiopulmonar/complicações , Doença Cardiopulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Cor pulmonale is right ventricular hypertrophy and/or dilation caused by pulmonary hypertension (PH) due to diseases affecting the lung function and structure. Recently, the definition of PH was revised from a mean pulmonary arterial pressure (mPAP) >25 mmHg to an mPAP >20 mmHg based on the Nice statement; this might expedite the detection of cor pulmonale. However, the only treatment for cor pulmonale for the past 3 decades has been to maintain the lung function and oxygen saturation. Chronic obstructive pulmonary disease (COPD) is the leading cause of cor pulmonale. Cor pulmonale in COPD is generally known to occur due to loss of vascular bed and chronic hypoxic pulmonary vasoconstriction (HPV) due to alveolar wall destruction. However, a recent study suggested that in some patients with COPD, the pulmonary vascular alterations include components that are primary lesions of the pulmonary artery. These alterations may be similar to the remodeling that occurs in pulmonary arterial hypertension (PAH). Although, there is no evidence supporting the treatment of COPD patients with PH using drugs approved for PAH, such drugs may be effective in the treatment of a selected group of COPD patients, whose disease includes PAH-like vascular components. To distinguish these patients, it is necessary to understand the histopathology of COPD and renew our understanding of the concept of cor pulmonale, which treats the heart and lung as a single unit. Herein, we review the recent histopathological concepts of COPD with respect to the progression of cor pulmonale.
Assuntos
Anti-Hipertensivos/uso terapêutico , Uso Off-Label , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Doença Cardiopulmonar/tratamento farmacológico , Doença Cardiopulmonar/etiologia , Humanos , Hipertensão Pulmonar/complicações , Doença Pulmonar Obstrutiva Crônica/complicações , Doença Pulmonar Obstrutiva Crônica/patologia , Doença Cardiopulmonar/diagnósticoRESUMO
The term cor pulmonale has traditionally been used as a synonym for right heart failure due to chronic respiratory diseases, although this condition is less frequently seen in the modern era because of the use of long-term oxygen therapy along with aggressive measures directed at optimizing ventilation and gas exchange. The mechanisms by which adaptation or maladaptation of right heart structure and function in the broader setting of pulmonary vascular disease, either intrinsic to the pulmonary circulation or due to respiratory diseases, have garnered considerable interest along with the development of medical and surgical treatments for pulmonary hypertension. Thus, the right heart is no longer considered an "innocent bystander" in pulmonary hypertension, but rather a key component in its pathophysiology. Furthermore, the status of right heart function is a major determinant of outcome. Accordingly, the right heart has become a potential, appealing target for novel therapies to treat hypertensive pulmonary vascular disease.
Assuntos
Hipertensão Pulmonar/diagnóstico , Doença Cardiopulmonar/diagnóstico , Insuficiência Cardíaca/etiologia , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Doença Cardiopulmonar/complicações , Doença Cardiopulmonar/fisiopatologia , Função Ventricular DireitaRESUMO
Almost 70 years ago, Drs. Baldwin, Cournand, and Richards defined chronic pulmonary insufficiency by the presence of respiratory symptoms, radiologic evidence of pulmonary emphysema on chest radiography, and physiologic gas trapping. A decade later, airflow obstruction on spirometry was added to the definition and insufficiency became a disease. Contemporary studies are reviving the diagnostic approach described by these early luminaries, with researchers finding that symptomatic smokers with preserved spirometry have increased exacerbations and that smokers and non-smokers with normal spirometry but emphysema on chest computed tomography have increased mortality. Hence, the Baldwin-Cournand-Richards concept of disease defined by respiratory symptoms, radiologic findings, and physiology-regardless of spirometric criteria-is being rediscovered. Baldwin, Cournand, and Richards also stated that "functionally, it is obvious that the pulmonary and circulatory apparatus are one unit," and they defined combined cardiopulmonary insufficiency as chronic pulmonary insufficiency with (left or right) cardiac and pulmonary artery enlargement. They appreciated the complexity of these interactions, which include the potential role of gas trapping in heart failure with reduced ejection fraction; the impact of emphysema on blood flow in heart failure with preserved ejection fraction; multiple contributions to cor pulmonale with increased pulmonary artery pressure; and cor pulmonale parvus in emphysema; all of which may be amenable to specific therapeutic interventions. Given the complexity of heart-lung interactions originally identified by Baldwin, Cournand, and Richards and the potentially large therapeutic opportunities, large-scale studies are still warranted to find specific therapies for subphenotypes of combined cardiopulmonary insufficiency.
Assuntos
Pulmão/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Enfisema Pulmonar/diagnóstico , Doença Cardiopulmonar/diagnóstico , Volume Expiratório Forçado , Humanos , Pulmão/diagnóstico por imagem , Doença Pulmonar Obstrutiva Crônica/complicações , Enfisema Pulmonar/complicações , Doença Cardiopulmonar/complicações , Fumar/efeitos adversos , Espirometria , Tomografia Computadorizada por Raios X , Função Ventricular DireitaAssuntos
Ventrículos do Coração/diagnóstico por imagem , Hemodinâmica/fisiologia , Ventilação com Pressão Positiva Intermitente/efeitos adversos , Doença Cardiopulmonar/etiologia , Diagnóstico Diferencial , Ecocardiografia , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/fisiopatologia , Função Ventricular Direita/fisiologiaRESUMO
Chronic cor pulmonale is defined as right ventricular failure secondary to pulmonary hypertension. Our study reviewed all cases of chronic cor pulmonale secondary to respiratory diseases in a ten-year period (2004-20014) in the Department of Child Health, Korle-Bu Teaching Hospital, Ghana. Nine cases of chronic cor pulmonale were recorded during the period. The age range was 1-9 years (average age = 3 years). Obstructive sleep apnoea secondary to adenoid hypertrophy was the commonest cause of pulmonary hypertension.
Assuntos
Hipertensão Pulmonar/complicações , Doença Cardiopulmonar/etiologia , Criança , Pré-Escolar , Doença Crônica , Países em Desenvolvimento , Feminino , Gana/epidemiologia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Lactente , Masculino , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/epidemiologia , Estudos RetrospectivosRESUMO
A pulmonary artery to aorta ratio (PA:A) >1 is a proxy of pulmonary hypertension. It is not known whether this measure carries prognostic information in the general population and in individuals with chronic obstructive pulmonary disease (COPD).Between 2003 and 2006, 2197 participants from the population-based Rotterdam Study (mean±sd age 69.7±6.7â years; 51.3% female), underwent cardiac computed tomography (CT) scanning with PA:A quantification, defined as the ratio between the diameters of the pulmonary artery and the aorta. COPD was diagnosed based on spirometry or clinical presentation and obstructive lung function measured by a treating physician. Cox regression was used to investigate the risk of mortality.We observed no association between 1-sd increase of PA:A and mortality in the general population. Larger PA:A was associated with an increased risk of mortality in individuals with COPD, particularly in moderate-to-severe COPD (hazard ratio 1.36, 95% CI 1.03-1.79). We demonstrated that the risk of mortality in COPD was driven by severe COPD, and that this risk increased with decreasing diffusing capacity.Larger PA:A is not associated with mortality in an older general population, but is an independent determinant of mortality in moderate-to-severe COPD. Measuring PA:A in CT scans obtained for other indications may yield important prognostic information in individuals with COPD.
Assuntos
Aorta , Hipertensão Pulmonar , Artéria Pulmonar , Capacidade de Difusão Pulmonar , Doença Pulmonar Obstrutiva Crônica , Doença Cardiopulmonar , Idoso , Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Estudos de Coortes , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Capacidade de Difusão Pulmonar/métodos , Capacidade de Difusão Pulmonar/fisiologia , Doença Pulmonar Obstrutiva Crônica/complicações , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Doença Pulmonar Obstrutiva Crônica/mortalidade , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Doença Cardiopulmonar/diagnóstico , Doença Cardiopulmonar/etiologia , Doença Cardiopulmonar/fisiopatologia , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Espirometria/métodos , Tomografia Computadorizada por Raios X/métodosRESUMO
We report the case of a previously healthy man who presented with subacute dyspnoea after a long drive. He developed hypoxic respiratory failure, thought secondary to a massive pulmonary embolism and was treated with tissue plasminogen activator but died in the hospital despite aggressive medical measures. Autopsy revealed pulmonary tumour thrombotic microangiopathy (PTTM) from papillary renal cell carcinoma. PTTM is a rare clinicopathological syndrome that clinically results in symptoms of dyspnoea and right heart failure. Pathologically, a localised paraneoplastic process evolves from tumour microemboli in the pulmonary arterioles, resulting in fibrocellular proliferation and narrowing of the vessels, causing subacute right heart failure. To our knowledge, this is the first case of PTTM due to papillary renal cell carcinoma.
Assuntos
Carcinoma de Células Renais/patologia , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/patologia , Neoplasias Pulmonares/secundário , Pulmão/patologia , Doença Cardiopulmonar/etiologia , Microangiopatias Trombóticas/etiologia , Adenocarcinoma/complicações , Adenocarcinoma/patologia , Autopsia , Carcinoma de Células Renais/complicações , Insuficiência Cardíaca/diagnóstico , Humanos , Hipertensão Pulmonar/diagnóstico , Pulmão/irrigação sanguínea , Neoplasias Pulmonares/complicações , Masculino , Pessoa de Meia-Idade , Células Neoplásicas Circulantes , Artéria Pulmonar/patologia , Embolia Pulmonar/diagnóstico , Doença Cardiopulmonar/diagnóstico , Insuficiência Respiratória/diagnóstico , Insuficiência Respiratória/etiologia , Microangiopatias Trombóticas/diagnósticoRESUMO
In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause.