BACKGROUND: Pulmonary hypertension (PH) is common in patients with idiopathic pulmonary fibrosis (IPF) and is associated with poor outcomes. This study was performed to determine the clinical efficacy of PH-specific therapeutic agents for IPF patients.METHODS: We performed a systematic review and meta-analysis using MEDLINE, EMBASE, and the Cochrane Central Register. We searched randomized controlled trials (RCTs) without language restriction until November 2018. The primary outcome was all-cause mortality to end of study.RESULTS: We analyzed 10 RCTs involving 2,124 patients, 1,274 of whom received PH-specific agents. In pooled estimates, the use of PH-specific agents was not significantly associated with reduced all-cause mortality to end of study compared with controls (hazard ratio, 0.99; 95% confidence interval [CI], 0.92, 1.06; P = 0.71; I² = 30%). When we performed subgroup analyses according to the type of PH-specific agent, sample size, age, forced vital capacity, diffusion lung capacity, and the extent of honeycombing, PH-specific agents also showed no significant association with a reduction in all-cause mortality. A small but significant improvement in quality of life, measured using the St. George Respiratory Questionnaire total score, was found in the PH-specific agent group (mean difference, −3.16 points; 95% CI, −5.34, −0.97; P = 0.005; I² = 0%). We found no significant changes from baseline in lung function, dyspnea, or exercise capacity. Serious adverse events were similar between the two groups.CONCLUSION: Although PH-specific agents provided small health-related quality-of-life benefits, our meta-analysis provides insufficient evidence to support their use in IPF patients.
Diffusion ; Dyspnea ; Humans ; Hypertension, Pulmonary ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Volume Measurements ; Mortality ; Quality of Life ; Sample Size ; Treatment Outcome ; Vasodilator Agents ; Vital Capacity

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterized by worsening lung function and dyspnea. The prognosis of IPF patients is poor, as median survival is approximately 3 years. However, recently developed IPF-specific therapies have shown improved efficacies in terms of reducing lung function decline and mortality. Therefore, the early recognition and accurate diagnosis of IPF are crucial. In 2018, new guidelines for the diagnosis of IPF were published by the Fleischner Society and by the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society (ATS/ERS/JRS/ALAT). Both guidelines emphasize the need for a thorough history taking and physical examination to exclude an alternative diagnosis, such as exposure-related or connective tissue disease. The most informative initial examination is high-resolution computed tomography, the results of which can indicate the need for bronchoalveolar lavage or surgical lung biopsy, based on a multidisciplinary discussion of the findings and the patient's clinical condition. A multidisciplinary discussion of the clinico-radiologic-pathologic findings is currently the gold standard in the diagnoisis of IPF and will allow the more effective and timely treatment of these patients.
Biopsy ; Bronchoalveolar Lavage ; Connective Tissue Diseases ; Diagnosis ; Dyspnea ; Humans ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Diseases, Interstitial ; Mortality ; Physical Examination ; Prognosis

BACKGROUND AND OBJECTIVES: Aortic valve replacement (AVR) is the treatment of choice in severe symptomatic aortic stenosis (AS) patients. However, a substantial number of elderly patients refuse AVR and treated medically. We investigated their long-term prognosis. METHODS: From January 2005 to December 2016, we analyzed elderly patients with severe symptomatic AS who refused to have AVR. RESULTS: After screening of total 534 patients, we analyzed total 180 severe symptomatic AS patients (78±7 years old, 96 males). Hypertension was the most common cardiovascular risk factor (72%) and the most common symptom was dyspnea (66%). Calculated aortic stenosis area was 0.73±0.20 cm2 and mean left ventricular ejection fraction (LVEF) was 57.8±12.2%. Total 102 patients died during follow-up period (39.1±31.0 months). One-, 3-, and 5-year all-cause mortality rate was 21.1±3.0%, 43.1±3.8%, and 56.5±4.2%, respectively. Of them, 87 died from cardiac causes, and 1-, 3-, and 5-year cardiac mortality rate was 18.0±2.9%, 38.2±3.8%, and 50.7±4.3%, respectively. Their all-cause mortality and cardiac mortality were significantly higher than those of controls. Univariate analysis showed that age, anemia, LVEF, and Log N-terminal pro B-type natriuretic peptide (NT-proBNP) were significant parameters in all-cause mortality (p < 0.001, p=0.001, p=0.039, and p=0.047, respectively) and in cardiac mortality (p < 0.001, p < 0.001, p=0.046, and p=0.026, respectively). Multivariate analysis showed that age and anemia were significant prognostic factors for cardiac and all-cause mortality. CONCLUSIONS: In elderly severe symptomatic AS patients who treated medically, their 1-, 3- and 5-year all-cause mortality rate was 21.1±3.0%, 43.1±3.8%, and 56.5±4.2%, respectively. Age and anemia were significant prognostic factors for cardiac and all-cause mortality.
Aged ; Anemia ; Aortic Valve Stenosis ; Aortic Valve ; Drug Therapy ; Dyspnea ; Follow-Up Studies ; Humans ; Hypertension ; Mass Screening ; Mortality ; Multivariate Analysis ; Natriuretic Peptide, Brain ; Prognosis ; Risk Factors ; Stroke Volume

Transfusion-related acute lung injury (TRALI) is defined as a new episode of acute lung injury that occurs during or within 6 hours of a completed transfusion, which is one of the leading causes of transfusion-related morbidity and mortality. We present a case of TRALI in a 29-year-old parturient with myelodysplastic syndrome scheduled for cesarean section. The parturient developed hypoxemia and dyspnea after preoperative transfusion of platelets following apheresis to eliminate a unit of leucocyte in order to correct thrombocytopenia. She underwent emergent caesarean section for fetal distress. After surgery, the chest radiograph showed diffuse haziness of both lung fields. Direct and indirect antiglobulin tests were negative, and hemolytic transfusion reaction was ruled out. Pro-BNP 347.3 pg/ml also excluded transfusion-associated circulatory overload. The parturient completely recovered after oxygen support for 2 days. It is important to recognize TRALI as soon as possible to minimize perioperative morbidity and mortality.
Acute Lung Injury* ; Adult ; Anoxia ; Blood Component Removal ; Cesarean Section ; Coombs Test ; Dyspnea ; Female ; Fetal Distress ; Humans ; Lung ; Mortality ; Myelodysplastic Syndromes* ; Oxygen ; Pregnancy ; Radiography, Thoracic ; Thrombocytopenia ; Transfusion Reaction

Chronic obstructive pulmonary disease (COPD) should be considered in any patient who has dyspnea, chronic cough or sputum production, and/or a history of exposure to risk factors for the disease, such as cigarette smoking, biomass exposure, and occupational dust. Spirometry is required to make the diagnosis, and a post-bronchodilator forced expiratory volume in one second/forced vital capacity ratio < 0.7 confirms the presence of persistent airflow limitation. The goal of COPD assessment is to determine the severity of the disease, including the severity of airflow limitation, the impact of the disease on the patient's health status, the risk of future events (such as exacerbations, hospital admission, or death), and comorbidities in order to guide therapy. Concomitant chronic diseases occur frequently in COPD patients, including cardiovascular disease, skeletal muscle dysfunction, metabolic syndrome, osteoporosis, depression, anxiety, and lung cancer. These comorbidities should be actively surveilled and treated appropriately when present, as they can independently influence mortality and hospitalization. Above all, further efforts are required to increase the diagnosis rate of COPD in Korea.
Anxiety ; Biomass ; Cardiovascular Diseases ; Chronic Disease ; Comorbidity ; Cough ; Depression ; Diagnosis ; Dust ; Dyspnea ; Forced Expiratory Volume ; Hospitalization ; Humans ; Korea ; Lung Neoplasms ; Mortality ; Muscle, Skeletal ; Osteoporosis ; Pulmonary Disease, Chronic Obstructive ; Respiratory Function Tests ; Risk Factors ; Smoking ; Spirometry ; Sputum ; Vital Capacity

“Thrombus-in-transit” in pulmonary embolism is associated with high mortality and refers to a free-floating clot in the right atrium or right ventricle, indicating that deep vein thrombosis is present en route to the pulmonary artery. Thrombus entrapped in a patent foramen ovale (PFO) is a rare condition and is associated with paradoxical systemic embolism. Here, we report a case of acute pulmonary embolism with thrombus-in-transit through a PFO in a 68-year-old woman with a diagnosis of metastatic pancreatic cancer undergoing palliative chemotherapy. She presented with syncope after acute onset of exertional dyspnea and was diagnosed with cardiogenic shock due to massive pulmonary embolism with thrombus-in-transit on admission to the emergency room. We treated her with systemic thrombolysis and anticoagulation therapy instead of surgical thrombectomy. We show that hemodynamically unstable pulmonary embolism with thrombus-in-transit entrapped by a PFO may be successfully treated with systemic thrombolysis without paradoxical embolism.
Aged ; Diagnosis ; Drug Therapy ; Dyspnea ; Embolism ; Embolism, Paradoxical ; Emergency Service, Hospital ; Female ; Foramen Ovale ; Foramen Ovale, Patent ; Heart Atria ; Heart Ventricles ; Humans ; Mortality ; Pancreatic Neoplasms ; Pulmonary Artery ; Pulmonary Embolism ; Shock, Cardiogenic ; Syncope ; Thrombectomy ; Thrombolytic Therapy ; Thrombosis ; Venous Thrombosis

Approximately one in four patients with chronic obstructive pulmonary disease (COPD) have asthmatic features consisting of wheezing, airway hyper-responsiveness or atopy. The Global initiative for Asthma/Globalinitiative for chronic Obstructive Lung Disease committee recently labelled these patients as having asthma-COPD overlap syndrome or ACOS. ACOS also encompasses patients with asthma, ≥40 years of age, who have been cigarette smokers (more than 5–10 pack years) or have had significant biomass exposure, and demonstrate persistent airflow limitation defined as a post-bronchodilator forced expiratory volume in 1 second (FEV₁)/forced vital capacity of <70%. Data over the past 30 years indicate that patients with ACOS have greater burden of symptoms including dyspnea and cough and show higher risk of COPD exacerbations and hospitalizations than those with pure COPD or pure asthma. Patients with ACOS also have increased risk of rapid FEV₁ decline and COPD mortality. Paradoxically, experimental evidence to support therapeutic decisions in ACOS patients is lacking because traditionally, patients with ACOS have been systematically excluded from therapeutic COPD and asthma trials to maintain homogeneity of the study population. In this study, we summarize the current understanding of ACOS, focusing on definitions, epidemiology and patient prognosis.
Asthma ; Biomass ; Cough ; Dyspnea ; Epidemiology ; Forced Expiratory Volume ; Hospitalization ; Humans ; Mortality ; Prognosis ; Pulmonary Disease, Chronic Obstructive ; Respiratory Hypersensitivity ; Respiratory Sounds ; Tobacco Products ; Vital Capacity

Chronic obstructive pulmonary disease (COPD) is characterized by persistent airflow limitation that is usually progressive. It is a major cause of morbidity and mortality worldwide, leading to substantial and increasing economic and social burden. Palliative care for COPD patients aims to reduce symptoms and exacerbations and improve exercise tolerance and quality of life. It is difficult to make a prognosis for COPD patients due to the variable illness trajectory and advanced care of patients. However, severity of breathlessness, assessment of lung function impairment, and frequency of exacerbations can help to identify palliative care needs and determine effective methods to mitigate symptoms, which is discussed in this paper. In these patients, it is recommended to provide individualized palliative care along with curative/restorative care at the onset of COPD symptoms. Before launching a palliative care system in Korea, it is necessary to prepare pulmonary rehabilitation resources, patient-centered communication, timely palliative responsiveness, and a program for effective advanced care planning. A multidisciplinary approach involving collaboration with not only the respiratory and palliative care teams but also primary care offers a new model of care for these patients and should be considered with a priority.
Cooperative Behavior ; Dyspnea ; Exercise Tolerance ; Hospice Care ; Hospices* ; Humans ; Korea ; Lung ; Mortality ; Palliative Care* ; Primary Health Care ; Prognosis ; Pulmonary Disease, Chronic Obstructive* ; Quality of Life ; Rehabilitation ; Respiratory Therapy

BACKGROUND AND OBJECTIVES: Trapped thrombus in patent foramen ovale (PFO) is a rare complication of pulmonary embolism that may lead to tragic clinical events. The aim of this study was to identify the optimal treatment for different clinical situations in patients with trapped thrombus in a PFO by conducting a literature review. SUBJECTS AND METHODS: A PubMed database search was conducted from 1991 through 2015, and 194 patients (185 articles) with trapped thrombus in a PFO were identified. Patient characteristics, paradoxical embolic events, and factors affecting 60-day mortality were analyzed retrospectively. RESULTS: Among all patients, 112 (57.7%) were treated with surgery, 28 with thrombolysis, and 54 with anticoagulation alone. Dyspnea (79.4%), chest pain (33.0%), and syncope (17.5%) were the most common presenting symptoms. Pretreatment embolism was found in 37.6% of cases, and stroke (24.7%) was the most common event. Surgery was associated with fewer post-treatment embolic events than were other treatment options (p=0.044). In the multivariate analysis, initial shock or arrest, and thrombolysis were independent predictors of 60-day mortality. Thrombolysis was related with higher 60-day mortality compared with surgery in patients who had no initial shock or arrest. CONCLUSION: This systematic review showed that surgery was associated with a lower overall incidence of post-treatment embolic events and a lower 60-day mortality in patients with trapped thrombus in a PFO. In patients without initial shock or arrest, thrombolysis was related with a higher 60-day mortality compared with surgery.
Chest Pain ; Dyspnea ; Embolism ; Foramen Ovale, Patent* ; Humans ; Incidence ; Mortality ; Multivariate Analysis ; Pulmonary Embolism ; Retrospective Studies ; Shock ; Stroke ; Syncope ; Thrombosis*

Atrial septal defect (ASD) is one of the common congenital anomalies of the heart in humans. Its complications depend on the size of the defect and can manifest at any age. The common symptoms of ASD include dyspnea and fatigue. Most of the ASDs are associated with morbidity and mortality, Earlier the treatment, it is better to the patient. I saw a large ostium secundum defect in the heart of an adult female cadaver during dissection classes for undergraduate medical students. The interatrial septum had large defect at the region where fossa ovalis should have been located. It was about 1.25 inches in diameter and oval in shape. This type of large septal defect might result in cyanosis, stroke or death of the patient at any age.
Adult ; Cadaver* ; Cyanosis ; Dyspnea ; Fatigue ; Female* ; Heart ; Heart Septal Defects, Atrial ; Humans ; Middle Aged* ; Mortality ; Stroke ; Students, Medical