Obstructive sleep apnea is a common disorder in which respiratory flow decreases or disappears despite respiratory effort due to occlusion of the upper respiratory tract during sleep. Oxidative stress and systemic inflammatory reaction induced by the obstruction cause complications such as hypertension, coronary artery disease, and diabetes and increase cancer incidence. Furthermore, in patients with interstitial lung disease, obstructive sleep apnea has a very high prevalence and is thought to have a close pathophysiological and clinical correlation. In other words, obstructive sleep apnea could be the cause or a complication of interstitial lung disease ; when these two afflictions coexist, the prognosis of the patient is worse. In patients with interstitial lung disease with obstructive sleep apnea, CPAP treatment significantly improved sleep and quality of life, as well as improved morbidity and mortality in a recent study. Therefore, early diagnosis and treatment of obstructive sleep apnea in patients with interstitial lung disease are very important, and additional studies designed to include patients with idiopathic pulmonary fibrosis as well as patients with advanced interstitial lung disease should be performed.
Coronary Artery Disease ; Early Diagnosis ; Humans ; Hypertension ; Idiopathic Pulmonary Fibrosis ; Incidence ; Lung Diseases, Interstitial* ; Mortality ; Oxidative Stress ; Prevalence ; Prognosis ; Quality of Life ; Respiratory System ; Sleep Apnea, Obstructive* ; Sleep Wake Disorders

Aspiration of meconium produces a syndrome (Meconium Aspiration Syndrome MAS) characterized by hypoxia, hypercapnia, and acidosis. Perinatal hypoxia, acute airway obstruction, pulmonary inflammation, pulmonary vasoconstriction, pulmonary hypertension, and surfactant inactivation all play a role in the pathogenesis of MAS. Most aspiration of meconium probably occurs before birth. Following aspiration, meconium may migrate to the peripheral airway, usually take about 2 hours as demonstrated in animal experiment, leading to airway obstruction and subsequent lung inflammation and pulmonary hypertension. The presence of meconium in the endotracheal aspirate automatically establishes the diagnosis of MAS. Clinical diagnosis can be made in any infant born with meconium staining of amniotic fluid who develops respiratory distress at or shortly after birth and has positive radiographic findings. Prevention of intrauterine hypoxia, early cleaning (suctioning) of the airway, and prevention and treatment of pulmonary hypertension are essential in the management of MAS. Recent studies suggest that avoidance of post-term delivery may reduce the risk of intrauterine hypoxia and the incidence of MAS. Routine intrapartum naso-and oropharyngeal suction does not appear to affect the incidence and outcome of MAS. Endotracheal suction at birth is considered a controversial procedure and only reserved for infants who have severe retraction at birth suggesting an upper airway obstruction. High frequency oscillatory ventilation with nitric oxide or surfactant may improve mortality. Mortality of MAS has improved; the causes of death are related primarily to hypoxic respiratory failure associated with irreversible pulmonary hypertension. Morbidity is affected mostly by perinatal hypoxia.
Acidosis ; Airway Obstruction ; Amniotic Fluid ; Animal Experimentation ; Anoxia ; Cause of Death ; Diagnosis ; Female ; Humans ; Hypercapnia ; Hypertension, Pulmonary ; Incidence ; Infant ; Infant, Newborn ; Meconium Aspiration Syndrome* ; Meconium* ; Mortality ; Nitric Oxide ; Parturition ; Pneumonia ; Respiratory Insufficiency ; Resuscitation* ; Suction ; Vasoconstriction ; Ventilation

Pulmonary artery sarcomas (PAS) are rare malignant neoplasms. Right heart failure due to tumour location is the main cause of death in PAS patients. The hemodynamic influence of PAS may effect prognosis, but this has not been proven. We aimed to identify the clinical characteristics and prognostic factors of PAS in Korea, their association with pulmonary hypertension (PH). PAS patients treated at the Asan Medical Center between 2000 and 2014 were reviewed. We examined demographic characteristics, diagnostic and treatment modalities. Potential prognostic factors were evaluated by univariate and multivariate analysis. Twenty patients were diagnosed with PAS. Ten patients were male, the median age was 54 years (range, 33–75 years). The most common symptom observed was dyspnea (65%). The most common histologic type was spindle cell sarcoma (30%). Ten patients had a presumptive diagnosis of pulmonary embolism (PE) and received anticoagulation therapy. Seventeen patients underwent surgery, but only 5 patients had complete resection. Eleven patients received post-operative treatment (chemotherapy = 3, radiotherapy = 5, chemoradiotherapy = 3). PH was observed in 12 patients before treatment and in 6 patients after treatment. Overall median survival was 24 months. Post-treatment PH was associated with poor prognosis (HR 9.501, 95% CI 1.79–50.32; P = 0.008) while chemotherapy was negatively associated with mortality (HR 0.102, 95% CI 0.013–0.826; P = 0.032) in univariate analysis. Post-treatment PH was also associated with poor prognosis in multivariate analysis (HR 5.7, 95% CI 1.08–30.91; P = 0.041). PAS patients are frequently misdiagnosed with PE in Korea. Post-treatment PH is associated with a poor prognosis.
Cause of Death ; Chemoradiotherapy ; Chungcheongnam-do ; Diagnosis ; Drug Therapy ; Dyspnea ; Heart Failure ; Hemodynamics ; Humans ; Hydrogen-Ion Concentration ; Hypertension, Pulmonary ; Korea* ; Male ; Mortality ; Multivariate Analysis ; Prognosis ; Pulmonary Artery* ; Pulmonary Embolism ; Radiotherapy ; Sarcoma*

BACKGROUND/AIMS: The diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is difficult for numerous reasons and is related with a poor prognosis. In Korea, the incidence of CTEPH and its clinical features are unknown. Thus, in this study, we evaluated the clinical characteristics and outcomes of CTEPH in a Korean cohort. METHODS: This study included South Korean patients diagnosed with CTEPH between September 2008 and October 2011. Baseline characteristics, treatments and outcomes were analyzed. RESULTS: A total of 134 patients were included in this study with 76 females (56.7%). Their median age was 58.3 +/- 15.9 years and dyspnea (112 patients, 83.5%) was the most common presenting symptom. Sixty-three patients (47%) had a history of acute pulmonary embolism or deep vein thrombosis, and six (4.5%) had pulmonary tuberculosis. In total, 28 patients (21%) underwent pulmonary thromboendarterectomy (PTE), and 99 patients had medical therapy. During the study period, 18 patients (13.4%) died. In a multivariate analysis, higher hemoglobin (relative risk [RR], 1.516; 95% confidence interval [CI], 1.053 to 2.184; p = 0.025) and lower total cholesterol levels (RR, 0.982; 95% CI, 0.965 to 0.999; p = 0.037) were associated with increased mortality. CONCLUSIONS: This was the first national cohort study of Korean patients with CTEPH. Accurate diagnosis, characterization and distributions of CTEPH are imperative for prompt treatment in patients, particularly those undergoing PTE.
Adult ; Aged ; Antihypertensive Agents/therapeutic use ; Chi-Square Distribution ; Chronic Disease ; Drug Therapy, Combination ; Endarterectomy ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*epidemiology/mortality/therapy ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Prevalence ; Prospective Studies ; Pulmonary Embolism/diagnosis/*epidemiology/mortality/therapy ; Registries ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome

BACKGROUND/AIMS: Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for IPAH, the benefits are uncertain, particularly in Korea, where it has not been studied. The purpose of this study was to evaluate survival outcomes of Korean patients with IPAH treated with warfarin. METHODS: We performed a retrospective cohort study of patients diagnosed previously with pulmonary arterial hypertension (PAH) at the Asan Medical Center in Korea, between January 1994 and February 2013. We excluded patients with associated PAH, patients who did not undergo right heart catheterization (RHC), and patients with a positive vasoreactivity test. Patients in the study cohort were classified into a "warfarin group" and a "non-warfarin group," according to the treatment they received during the first year after diagnosis. RESULTS: We identified 31 patients with IPAH and a negative vasoreactivity test on RHC. Median patient age was 36.0 years, and 23 patients (74.2%) were female. The median time from the onset of symptoms to diagnosis was 19.0 months, and the most common presenting symptom was dyspnea. Survival rates of the patients at 1, 3, 5, and 10 years were 90.2%, 79.5%, 62.7%, and 34.8%, respectively. The mean survival period was 12.0 years in the warfarin group and 6.1 years in the non-warfarin group. Warfarin treatment had significant survival benefits in patients with IPAH (p = 0.023). CONCLUSIONS: Warfarin treatment substantially improved survival outcomes in Korean cases of IPAH.
Adult ; Anticoagulants/*therapeutic use ; Asian Continental Ancestry Group ; Familial Primary Pulmonary Hypertension/diagnosis/*drug therapy/ethnology/mortality ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Proportional Hazards Models ; Protective Factors ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome ; Warfarin/*therapeutic use

BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients.
Adolescent ; Adult ; Cardiomegaly/diagnosis/epidemiology ; Chi-Square Distribution ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*mortality ; Kaplan-Meier Estimate ; Lung Diseases, Interstitial/diagnosis/mortality ; Lupus Erythematosus, Systemic/diagnosis/*mortality ; Lupus Nephritis/diagnosis/mortality ; Male ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Raynaud Disease/diagnosis/epidemiology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Young Adult

BACKGROUNDIdiopathic pulmonary fibrosis (IPF) is a lethal chronic interstitial lung disease (ILD) of unknown cause and having a variable and unpredictable course. This study aimed to summarize the clinical features and follow-up outcomes and to identify potential factors useful for the assessment of prognosis in IPF.

METHODSTwo hundred and ten patients hospitalized and diagnosed as IPF in our unit from January 1999 to June 2007 were enrolled into this study. The baseline demographic, clinical, radiologic and physiologic characteristics were summarized. Clinical follow-up data until February 2010 were collected, and the median survival time and 1-, 2-, and 5-year survival rates, as well as the influences of the summarized baseline variables on the prognosis were analyzed.

RESULTSThe age at diagnosis as IPF was (64 ± 10) years, the duration before diagnosis of 106 patients (50%) was shorter than 2 years, and 73% were males. One hundred and forty-five patients (69%) had a history of smoking with a median pack-year of 18. Eighty-nine patients (42%) had emphysema and 62 patients (29%) pulmonary arterial hypertension (PAH). One hundred and twenty-four patients were followed up, of which 99 patients died from various causes including respiratory failure related to IPF (93%). The follow-up period was (21 ± 23) months. The median survival time was 38 months. The 1-, 2-, and 5-year survival rates were 61%, 52%, and 39%, respectively. Multivariate analysis showed clubbing, PAH, duration from initial onset to diagnosis, and forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) were independent prognostic indicators of IPF.

CONCLUSIONIPF patients who have clubbing, PAH, a higher FEV1/FVC, and a short duration from initial onset to diagnosis have a poorer outcome.

Aged ; Emphysema ; diagnosis ; mortality ; physiopathology ; Female ; Humans ; Hypertension, Pulmonary ; diagnosis ; pathology ; physiopathology ; Idiopathic Pulmonary Fibrosis ; diagnosis ; mortality ; physiopathology ; Male ; Middle Aged

We aimed to identify a vasoreactive subset of patients with idiopathic pulmonary arterial hypertension (IPAH) in Korea and to show their clinical characteristics and prognosis. Data on patients who were diagnosed with IPAH at Asan Medical Center between January 1994 and March 2013 were retrospectively collected. Acute vasodilator testing was performed with inhaled nitric oxide during diagnostic right heart catheterization. A positive acute response was defined as a reduction in mean pulmonary arterial pressure (PAP) > or =10 mmHg to an absolute level of mean PAP <40 mmHg without a decrease in cardiac output. Among a total of 60 IPAH patients included for analysis, 9 (15%) showed a positive acute response to acute vasodilator testing. Acute responders showed significantly lower peak velocity of a tricuspid regurgitation jet on echocardiography (4.1+/-0.3 m/s vs. 4.6+/-0.6 m/s; P=0.01) and significantly lower mean PAP hemodynamically (47+/-10 mmHg vs. 63+/-17 mmHg; P=0.003) than non-responders at baseline. The survival rate of acute responders was 88% at 1, 3, 5, and 10 yr, respectively, which was significantly higher than that of non-responders (85%, 71%, 55%, and 40%, respectively; P=0.029). In conclusion, Korean IPAH patients with vasoreactivity showed better baseline hemodynamic features and survival than those without vasoreactivity.
Adolescent ; Adult ; Aged ; Female ; Humans ; Hypertension, Pulmonary/*diagnosis/*mortality/physiopathology ; Male ; Middle Aged ; Prevalence ; Prognosis ; Reproducibility of Results ; Republic of Korea/epidemiology ; Risk Factors ; Sensitivity and Specificity ; Survival Rate ; Vascular Resistance/*drug effects ; Vasodilator Agents/*diagnostic use ; Young Adult

Despite the necessity of surgical aortic valve replacement, many patients with symptomatic severe aortic stenosis (AS) cannot undergo surgery because of their severe comorbidities. In these high-risk patients, percutaneous transcatheter aortic valve implantation (TAVI) can be safely accomplished. However, no study has shown that TAVI can be performed for patients with severe AS accompanied by acute decompensated heart failure. In this case report, 1 patient presented a case of severe pulmonary hypertension with decompensated heart failure after diagnosis with severe AS, and was successfully treated via emergency TAVI. Without any invasive treatment, acute decompensated heart failure with severe pulmonary hypertension is common in patients with severe AS, and it can increase mortality rates. In conclusion, TAVI can be considered one of the treatment options for severe as presented as acute decompensated heart failure patients with pulmonary hypertension.
Aortic Valve Stenosis ; Aortic Valve* ; Comorbidity ; Diagnosis ; Emergencies* ; Emergency Treatment ; Heart Failure ; Heart Valve Prosthesis Implantation ; Humans ; Hypertension, Pulmonary ; Mortality

Antiphospholipid syndrome (APS) causes recurrent thromboses and morbidity during pregnancy, including fetal loss. This malady is associated with the persistent presence of anticardiolipin antibody or lupus anticoagulant. The pulmonary manifestations of antiphospholipid syndrome include pulmonary thromboembolism, pulmonary hypertension, acute respiratory distress syndrome, etc. Pulmonary thromboembolism is often the initial manifestation of antiphospholipid syndrome and a timely diagnosis is critical due to the high mortality rate. We herein report on a 19-year-old man with pulmonary thromboembolism that was caused by primary antiphospholipid syndrome. He presented with blood-tinged sputum, fever and epigastric pain, and his chest computerized tomography showed pulmonary thromboembolism. The other possible causes of pulmonary thromboembolism were excluded and the diagnosis of primary antiphospholipid syndrome was confirmed by the lupus anticoagulant that was present on two occasions six weeks apart. We also discuss the nature and management of antiphospholipid syndrome, along with a brief review of the relevant literatures.
Antibodies, Anticardiolipin ; Antiphospholipid Syndrome* ; Diagnosis ; Fever ; Humans ; Hypertension, Pulmonary ; Lupus Coagulation Inhibitor ; Mortality ; Pregnancy ; Pulmonary Embolism* ; Respiratory Distress Syndrome, Adult ; Sputum ; Thorax ; Thrombosis ; Young Adult