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

OBJECTIVES: Recently, low systolic blood pressure (SBP) was found to be associated with an increased risk of death from vascular diseases in a rural elderly population in Korea. However, evidence on the association between low SBP and vascular diseases is scarce. The aim of this study was to prospectively examine the association between low SBP and mortality from all causes and vascular diseases in older middle-aged Korean men. METHODS: From 2004 to 2010, 94 085 Korean Vietnam War veterans were followed-up for deaths. The adjusted hazard ratios (aHR) were calculated using the Cox proportional hazard model. A stratified analysis was conducted by age at enrollment. SBP was self-reported by a postal survey in 2004. RESULTS: Among the participants aged 60 and older, the lowest SBP (<90 mmHg) category had an elevated aHR for mortality from all causes (aHR, 1.9; 95% confidence interval [CI], 1.2 to 3.1) and vascular diseases (International Classification of Disease, 10th revision, I00-I99; aHR, 3.2; 95% CI, 1.2 to 8.4) compared to those with an SBP of 100 to 119 mmHg. Those with an SBP below 80 mmHg (aHR, 4.5; 95% CI, 1.1 to 18.8) and those with an SBP of 80 to 89 mmHg (aHR, 3.1; 95% CI, 0.9 to 10.2) also had an increased risk of vascular mortality, compared to those with an SBP of 90 to 119 mmHg. This association was sustained when excluding the first two years of follow-up or preexisting vascular diseases. In men younger than 60 years, the association of low SBP was weaker than that in those aged 60 years or older. CONCLUSIONS: Our findings suggest that low SBP (<90 mmHg) may increase vascular mortality in Korean men aged 60 years or older.
Aged ; Asian Continental Ancestry Group ; *Blood Pressure ; Cause of Death ; Cohort Studies ; Follow-Up Studies ; Humans ; Hypertension/mortality/physiopathology ; Male ; Middle Aged ; Proportional Hazards Models ; Prospective Studies ; Republic of Korea ; Surveys and Questionnaires ; Vascular Diseases/mortality/*physiopathology ; Veterans Health

Elevated blood pressure (BP) is the most common cause of cardiovascular disease. Salt intake has a strong influence on BP, and plasma sodium (pNa) is increased with progressive increases in salt intake. However, the associations with pNa and BP had been reported inconsistently. We evaluated the association between pNa and BP, and estimated the risks of all-cause-mortality according to pNa levels. On the basis of data collected from health checkups during 1995-2009, 97,009 adult subjects were included. Positive correlations between pNa and systolic BP, diastolic BP, and pulse pressure (PP) were noted in participants with pNa > or =138 mM/L (P<0.001). In participants aged > or =50 yr, SBP, DBP, and PP were positively associated with pNa. In participants with metabolic syndrome components, the differences in SBP and DBP according to pNa were greater (P<0.001). A cumulative incidence of mortality was increased with increasing pNa in women aged > or =50 yr during the median 4.2-yr-follow-up (P<0.001). In women, unadjusted risks for mortality were increased according to sodium levels. After adjustment, pNa > or =145 mM/L was related to mortality. The positive correlation between pNa and BP is stronger in older subjects, women, and subjects with metabolic syndrome components. The incidence and adjusted risks of mortality increase with increasing pNa in women aged > or =50 yr.
Adult ; Blood Pressure/*physiology ; Cardiovascular Diseases/blood/*mortality ; Female ; Humans ; Hypertension/*physiopathology ; Incidence ; Male ; Metabolic Syndrome X/blood ; Middle Aged ; Risk ; Risk Factors ; Sex Factors ; Sodium/*blood

To investigate the correlations between lipid ratio/oxidative stress status in chronic obstructive pulmonary disease (COPD) patients and pulmonary hypertension as well as the prognosis.
 Methods: A total of 120 patients with COPD were randomly selected and served as the COPD group and 30 healthy persons were selected as the control group. The ratios of low density lipoprotein (LDL)/high-density lipoprotein (HDL), triglyceride (TG)/HDL and total cholesterol (TC)/HDL were measured. The superoxide dismutase (SOD) activity, malondialdehyde (MDA) content and total antioxidant capacity (T-AOC) level in the control group and COPD patients were detected. Pulmonary hypertension incidence and 3-year survival rate for COPD patients were statistically analyzed. Spearman rank correlation method was used to analyze relationship between lipid ratio /oxidative stress status and pulmonary hypertension.
 Results: Compared with control group, the ratios of LDL/HDL, TG/HDL and TC/HDL, and the serum MDA level in the COPD group were increased, while the serum SOD and T-AOC level in the COPD group were decreased; compared with stable period, lipid ratios and MDA levels in the acute period were elevated, while serum SOD and T-AOC levels were reduced (P<0.05). Pulmonary hypertension incidence and 3-year survival rates in the COPD group were 56.67% and 81.67% respectively; the lipid ratios and serum MDA levels in COPD patients with pulmonary hypertension were elevated compared with that in COPD patients without pulmonary hypertension; the serum SOD and T-AOC levels in COPD patients with pulmonary hypertension were reduced compared with that in patients without pulmonary hypertension (P<0.05). Spearman rank correlation analysis showed that ratios of LDL/HDL, TG/HDL and TC/HDL, and the serum MDA levels in COPD patients were positively correlated with 3-years pulmonary hypertension incidence (r=0.752, 0.748, 0.752, 0.748; P<0.05), and negatively correlated with 3-years survival rate (r=-0.722, -0.751, -0.736, -0.748; P<0.05); serum SOD and T-AOC levels in COPD patients were negatively correlated with 3-years pulmonary hypertension (r=-0.711, -0.734; P<0.05), and positively correlated with 3-year survival rate (r=0.726, 0.733; P<0.05). 
 Conclusion: Blood lipid ratio and oxidative stress levels in COPD patients are elevated while antioxidant abilities were attenuated. The lipid ratio and oxidative stress status in COPD patients is closely related to the prognosis of pulmonary hypertension. Therefore, blood lipid ratio and oxidative stress status may be used in evaluation of pulmonary hypertension and prognosis for COPD patients.
Biomarkers ; blood ; Cholesterol ; blood ; physiology ; Female ; Humans ; Hypertension, Pulmonary ; physiopathology ; Lipids ; Lipoproteins, HDL ; blood ; physiology ; Lipoproteins, LDL ; physiology ; Male ; Malondialdehyde ; Oxidative Stress ; physiology ; Prognosis ; Pulmonary Disease, Chronic Obstructive ; blood ; mortality ; physiopathology ; Superoxide Dismutase ; Triglycerides ; blood ; physiology

BACKGROUND/AIMS: This meta-analysis compared the effects of amlodipine besylate, a charged dihydropyridine-type calcium channel blocker (CCB), with other non-CCB antihypertensive therapies regarding the cardiovascular outcome. METHODS: Data from seven long-term outcome trials comparing the cardiovascular outcomes of an amlodipine-based regimen with other active regimens were pooled and analyzed. RESULTS: The risk of myocardial infarction was significantly decreased with an amlodipine-based regimen compared with a non-CCB-based regimen (odds ratio [OR], 0.91; 95% confidence interval [CI], 0.84 to 0.99; p = 0.03). The risk of stroke was also significantly decreased (OR, 0.84; 95% CI, 0.79 to 0.90; p < 0.00001). The risk of heart failure increased slightly with marginal significance for an amlodipine-based regimen compared with a non-CCB-based regimen (OR, 1.14; 95% CI, 0.98 to 1.31; p = 0.08). However, when compared overall with beta-blockers and diuretics, amlodipine showed a comparable risk. Amlodipine-based regimens demonstrated a 10% risk reduction in overall cardiovascular events (OR, 0.90; 95% CI, 0.82 to 0.99; p = 0.02) and total mortality (OR, 0.95; 95% CI, 0.91 to 0.99; p = 0.01). CONCLUSIONS: Amlodipine reduced the risk of total cardiovascular events as well as all-cause mortality compared with non-CCB-based regimens, indicating its benefit for high-risk cardiac patients.
Amlodipine/*therapeutic use ; Antihypertensive Agents/*therapeutic use ; Blood Pressure/*drug effects ; Calcium Channel Blockers/*therapeutic use ; Chi-Square Distribution ; Clinical Trials as Topic ; Heart Failure/etiology/mortality/*prevention & control ; Humans ; Hypertension/complications/diagnosis/*drug therapy/mortality/physiopathology ; Myocardial Infarction/etiology/mortality/*prevention & control ; Odds Ratio ; Risk Factors ; Stroke/etiology/mortality/*prevention & control ; Treatment Outcome

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

BACKGROUND/AIMS: Intra-abdominal hypertension (IAH) is being increasingly reported in patients with severe acute pancreatitis (SAP) with worsened outcomes. The present study was undertaken to evaluate intra-abdominal pressure (IAP) as a marker of severity in the entire spectrum of acute pancreatitis and to ascertain the relationship between IAP and development of complications in patients with SAP. METHODS: IAP was measured via the transvesical route by measurements performed at admission, once after controlling pain and then every 4 hours. Data were collected on the length of the hospital stay, the development of systemic inflammatory response syndrome (SIRS), multiorgan failure, the extent of necrosis, the presence of infection, pleural effusion, and mortality. RESULTS: In total, 40 patients were enrolled and followed up for 30 days. The development of IAH was exclusively associated with SAP with an APACHE II score > or =8 and/or persistent SIRS, identifying all patients who were going to develop abdominal compartment syndrome (ACS). The presence of ACS was associated with a significantly increased extent of pancreatic necrosis, multiple organ failure, and mortality. The mean admission IAP value did not differ significantly from the value obtained after pain control or the maximum IAP measured in the first 5 days. CONCLUSIONS: IAH is reliable marker of severe disease, and patients who manifest organ failure, persistent SIRS, or an Acute Physiology and Chronic health Evaluation II score > or =8 should be offered IAP surveillance. Severe pancreatitis is not a homogenous entity.
APACHE ; Acute Disease ; Adult ; Female ; Humans ; Intra-Abdominal Hypertension/*etiology ; Length of Stay ; Male ; Middle Aged ; Multiple Organ Failure/etiology ; Necrosis/etiology ; Pancreas/*pathology ; Pancreatitis/*complications/mortality/physiopathology ; Pleural Effusion/etiology ; Prospective Studies ; Severity of Illness Index ; Systemic Inflammatory Response Syndrome/etiology

We evaluated long-term results of De Vega annuloplasty measured by cylindrical sizers for functional tricuspid regurgitation (FTR) and analyzed the impact of measured annular size on the late recurrence of tricuspid valve regurgitation. Between 2001 and 2011, 177 patients (57.9+/-10.5 yr) underwent De Vega annuloplasty for FTR. Three cylindrical sizers (actual diameters of 29.5, 31.5, and 33.5 mm) were used to reproducibly reduce the tricuspid annulus. Long-term outcomes were evaluated and risk factor analyses for the recurrence of FTR > or =3+ were performed. Measured annular diameter indexed by patient's body surface area was included in the analyses as a possible risk factor. Operative mortality occurred in 8 patients (4.5%). Ten-year overall and cardiac death-free survivals were 80.5% and 90.8%, respectively. Five and 10-yr freedom rates from recurrent FTR were 96.5% and 93.1%, respectively. Cox proportional hazard model revealed that higher indexed annular size was the only risk factor for the recurrence of FTR (P=0.006). A minimal P value approach demonstrated that indexed annular diameter of 22.5 mm/m2 was a cut-off value predicting the recurrence of FTR. De Vega annuloplasty for FTR results in low rates of recurrent FTR in the long-term. Tricuspid annulus should be reduced appropriately considering patients' body size to prevent recurrent FTR.
Adult ; Age Factors ; Aged ; Body Surface Area ; Cardiac Valve Annuloplasty ; Disease-Free Survival ; Echocardiography ; Female ; Humans ; Hypertension/complications ; Male ; Middle Aged ; Postoperative Complications ; Proportional Hazards Models ; Recurrence ; Risk Factors ; Treatment Outcome ; Tricuspid Valve/*physiopathology ; Tricuspid Valve Insufficiency/etiology/mortality/*surgery

The purpose of this study was to investigate the therapeutic effects of small hairpin RNA (shRNA) targeting endothelin-converting enzyme (ECE)-1 in monocrotaline (MCT)-induced pulmonary hypertensive rats. Ninty-four Sprague-Dawley rats were divided into three groups: control (n = 24), MCT (n = 35) and shRNA (n = 35). Four-week survival rate in the shRNA group was significantly increased compared to that in the MCT group. The shRNA group showed a significant improvement of right ventricular (RV) pressure compared with the MCT group. The MCT and shRNA groups also showed an increase in RV/(left ventricle + septum) ratio and lung/body weight. Plasma endothelin (ET)-1 concentrations in the shRNA group were lower than those in the MCT group. Medial wall thickness of pulmonary arterioles were increased after MCT injection and was significantly decreased in the shRNA group. The number of intra-acinar muscular pulmonary arteries was decreased in the shRNA group. The mRNA expressions of ET-1 and ET receptor A (ETA) were significantly decreased in the shRNA group in week 4. The protein levels of ETA were decreased in the shRNA group in week 2. The protein levels of tumor necrosis factor-alpha and vascular endothelial growth factor were decreased in the shRNA group in week 4. In conclusion, the gene silencing with lentiviral vector targeting ECE-1 could be effective against hemodynamic, histopathological and gene expression changes in pulmonary hypertension.
Animals ; Aspartic Acid Endopeptidases/*antagonists & inhibitors/blood/genetics ; Body Weight ; Heart Ventricles/physiopathology ; Hypertension, Pulmonary/chemically induced/*enzymology/mortality ; Lentivirus/genetics ; Lung/anatomy & histology/metabolism/pathology ; Male ; Metalloendopeptidases/*antagonists & inhibitors/blood/genetics ; Monocrotaline/toxicity ; Pulmonary Artery/drug effects/physiopathology ; RNA, Small Interfering/*metabolism ; Rats ; Rats, Sprague-Dawley ; Receptor, Endothelin A/genetics/metabolism ; Survival Rate ; Tumor Necrosis Factor-alpha/metabolism ; Vascular Endothelial Growth Factor A/metabolism

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 +/- 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.
Adult ; Aged ; Connective Tissue Diseases/complications ; Data Collection ; *Databases, Factual ; Familial Primary Pulmonary Hypertension/*epidemiology/mortality/therapy ; Female ; Heart Defects, Congenital/complications ; Humans ; Internet ; Male ; Middle Aged ; Prognosis ; Prospective Studies ; Pulmonary Artery/*physiopathology ; *Registries ; Republic of Korea/epidemiology ; Survival Rate ; Young Adult