Demographics, treatment trends, and survival rate in incident pulmonary artery hypertension in Korea: A nationwide study based on the health insurance review and assessment service database

Epidemiologic data regarding pulmonary arterial hypertension (PAH) have relied on registries from Western countries. We assessed the current status of PAH in the Korean population. The Health Insurance Review and Assessment Service (HIRA) claim database, which comprises nationwide medical insurance data of Koreans from 2008-2016, was assessed to determine the current status of PAH. Overall, 1,307 patients were newly diagnosed with PAH from 2008-2016 (0.0005%, annual incidence: 4.84 patients/1 million people/year). The mean age at diagnosis was 44±13 years (range 18-65) and patients were mostly women (n = 906, 69.3%). Cases of idiopathic PAH (51.6%) accounted for the largest proportion, followed by acquired PAH (APAH) associated with congenital heart disease (25.8%) and APAH with connective tissue disease (17.2%). Overall, 807 (61.7%) patients received a single PAH-specific treatment based on their last prescription, of which bosentan (50.6%) was the most frequently used. Only 240 (18.4%) patients received combination therapy, with the bosentan-beraprost combination (32.9%) being the most common. During the mean follow-up of 1.9 years, the 1-, 2-, 3-, and 5-year estimated survival rates were 85%, 62%, 54%, and 46%, respectively. The prevalence and incidence of PAH in the Korean population is currently comparable with that in previous registries. The 5-year survival rate was slightly higher in the Korean population than previously reported.ope

Effect of Niacin on Carotid Atherosclerosis in Patients at Low-Density Lipoprotein-Cholesterol Goal but High Lipoprotein (a) Level: a 2-Year Follow-Up Study

Objective: To examine the effect of niacin on the progression of carotid intima-media thickness (IMT) in patients with high level of lipoprotein (Lp) (a). Methods: Patients at low-density lipoprotein-cholesterol goal but with Lp (a) >25 mg/dL and mean carotid IMT >0.75 mm were included. Eligible patients were randomized at a 1:2 ratio into one of two groups for 24 months: control or 1,500 mg extended release niacin. The primary study outcomes were the percentage changes in mean and maximal carotid IMT. The percentage change in lipid profiles including Lp (a) was analyzed as a secondary study outcome. Results: Among 96 randomized patients, 31 completed the study (mean age: 65 years; male: 44%). At follow-up, the percentage change in mean carotid IMT was not significantly different between the two groups (−1.4%±15.5% and −1.1%±7.3% in the control and niacin groups, respectively, p =0.95). The percentage change in maximal carotid IMT was also similar in the two groups (0.7%±16.5% and −4.4%±11.6%, respectively, p =0.35). Elevation of high-density lipoprotein-cholesterol tended to be higher in the niacin group ( p =0.07), and there was a significant difference in the percentage change in hemoglobin A1c between the two groups (−1.9%±2.2% and 3.3%±6.7%, respectively, p =0.02). Reduction of Lp (a) was greater in the niacin-treated group compared to placebo, but the difference was not statistically significant. Conclusion: Treatment with niacin for two years did not inhibit the progression of carotid intima-media thickening in patients with high Lp (a) level. However, this study may have been underpowered to evaluate the primary study outcome.ope

Characteristics and prognostic implications of tricuspid regurgitation in patients with arrhythmogenic cardiomyopathy

Aims: Arrhythmogenic cardiomyopathy (AC) is characterized by right ventricular (RV) dilatation and dysfunction and is often seen in combination with tricuspid regurgitation (TR). The aim of this study was to investigate the characteristics and prognostic implications of TR in patients with AC. Methods and results: Clinical, echocardiographic, and cardiac magnetic resonance data of 52 patients with AC fulfilling 2010 Task Force criteria in a single centre were retrospectively evaluated. TR in AC was classified as no/mild, moderate, or severe on the basis of the current guidelines. Significant TR was defined as at least moderate TR. The primary endpoint was a composite of death, heart transplantation, and tricuspid valve surgery. There were seven patients (13.4%) with moderate TR and 13 patients (25.0%) with severe TR at initial diagnosis. Patients with severe TR showed a higher prevalence of atrial fibrillation and a higher mean NT-pro-BNP than other groups (68%, P = 0.013; 2423 ± 1578 pg/mL, P < 0.001, respectively). Patients with significant TR revealed a higher incidence of heart failure at initial presentation than did those without significant TR (30.0 vs. 3.1%, P = 0.022). Patients with severe TR showed significantly larger RV and lower RV and left ventricular functional parameters. During a mean follow-up of 4.2 years, three groups classified by TR severity considerably discriminated clinical outcomes (log rank P = 0.019). Patients with significant TR had a poorer prognosis than those with no or mild TR (42.9 vs. 3.1%, log rank P = 0.005). Cox regression analysis showed significant TR as an independent prognostic factor (hazard ratio 11.41, 95% confidential interval 1.30-99.92, P = 0.028). Conclusions: Significant TR at initial diagnosis in patients with AC is a poor prognostic factor.ope

Progression and Outcomes of Non-dysfunctional Bicuspid Aortic Valve: Longitudinal Data From a Large Korean Bicuspid Aortic Valve Registry

Background: Using echocardiographic surveillance, many patients are diagnosed with bicuspid aortic valve (BAV) without significant valve dysfunction. Limited data are available regarding the progression and outcomes of non-dysfunctional BAV. Methods and Results: We investigated 1,307 BAV patients (984 male, mean age 56 years) diagnosed from Jan 2003 through Dec 2018 in a single tertiary center. Seven hundred sixty-one patients underwent follow-up echocardiography at &amp;amp;gt;= 1 year post-diagnosis. Non-dysfunctional BAV was defined as BAV without moderate aortic stenosis (AS) or aortic regurgitation (AR). The presence of aortopathy was defined as an ascending aorta diameter &amp;amp;gt;37mm. Progression to significant BAV dysfunction, progression to severe aortopathy (ascending aorta diameter &amp;amp;gt;= 45mm), and incidence of valve or aorta operation were analyzed. One hundred eighty-seven (25%) patients showed non-dysfunctional BAV. Among them, 104 (56%) had mild AS or AR, and 81 (43%) had aortopathy at indexed echocardiography. At 6.0 +/- 3.8 years post-diagnosis, 56 (29%) progressed to dysfunctional BAV, 28 (15%) progressed to severe aortopathy, 22 (12%) underwent valve operation, and 19 (10%) experienced aorta operation. Eighty-nine percent of patients with normal BAV function and 61% of patients with mild AS or AR maintained non-dysfunctional BAV. More patients with aortopathy progressed to severe aortopathy (35 vs. 0% without aortopathy, p &amp;amp;lt; 0.001), with a higher incidence of aorta operation (21 vs. 2%, p &amp;amp;lt; 0.001). Conclusions: In patients with non-dysfunctional BAV, initial BAV function and degree of aorta dilatation might be important for progression and outcomes. Patients without any dysfunction or aortopathy tend to maintain good structure and function for 6 years

Efficacy and Safety of Udenafil for the Treatment of Pulmonary Arterial Hypertension: a Placebo-controlled, Double-blind, Phase IIb Clinical Trial

PURPOSE: Udenafil is an oral phosphodiesterase-5 inhibitor approved for the treatment of erectile dysfunction. In a multicenter, placebo-controlled, randomized Phase IIa study, the reduction of pulmonary vascular resistance index was greater with a 50-mg baseline dose of udenafil than with the 100-mg dose, the cardiac index did not decrease at most points, and the safety was excellent, suggesting that 50-mg udenafil could be used in a Phase IIb trial. METHODS: In this 16-week, double-blind, placebo-controlled study, 63 patients with pulmonary arterial hypertension were randomized to receive 50-mg udenafil or a placebo BID. The primary efficacy end point was the 6-min walking distance. The secondary efficacy end points were the Borg dyspnea score and time to clinical worsening. Patients who completed the 16-week study could participate in a long-term extension study. FINDINGS: In terms of the difference between the baseline and 16-week 6-min walking distance in both groups, the mean placebo-corrected treatment effect was 25 (58) m (P = 0.0873). Among the patients with a history of endothelin receptor antagonist therapy, the treatment effect at week 16 between the udenafil and placebo groups was 34 (60) m (P = 0.0460). However, there were no significant differences in the Borg dyspnea score and time to clinical worsening between groups. The safety profile and adverse effects of udenafil were similar to those of typical phosphodiesterase-5 inhibitors seen in previous studies. IMPLICATIONS: Udenafil has a favorable safety profile and improves exercise capacity in patients with pulmonary arterial hypertension. ClinicalTrials.gov identifier: NCT01553721.restrictio

Incremental prognostic value of triglyceride glucose index additional to coronary artery calcium score in asymptomatic low-risk population

Background: The triglyceride glucose (TyG) index has been suggested as a reliable surrogate marker of insulin resistance which is a substantial risk factor for atherosclerotic cardiovascular disease (ASCVD). Several recent studies have shown the relationship between the TyG index and cardiovascular disease; however, the role of the TyG index in coronary artery calcification (CAC) progression has not been extensively assessed especially in low-risk population. Methods: We enrolled 5775 Korean adults who had at least two CAC evaluations. We determined the TyG index using ln (fasting triglycerides [mg/dL] x fasting glucose [mg/dL]/2). The CAC progression was defined as either incident CAC in a CAC-free population at baseline or an increase of ≥ 2.5 units between the square roots of the baseline and follow-up coronary artery calcium scores (CACSs) of subjects with detectable CAC at baseline. Results: CAC progression was seen in 1,382 subjects (23.9%) during mean 3.5 years follow-up. Based on the TyG index, subjects were stratified into four groups. Follow-up CACS and incidence of CAC progression were markedly elevated with rising TyG index quartiles (group I [lowest]:17.6% vs. group II:22.2% vs. group III:24.6% vs. group IV [highest]: 31.3%, p < 0.001). In multivariate logistic regression analysis, the TyG index was independent predictor of CAC progression (odds ratio: 1.57; 95% confidence interval: 1.33 to 1.81; p < 0.001) especially in baseline CACS ≤ 100 group. Conclusion: The TyG index is an independent predictor of CAC progression in low-risk population. It adds incremental risk stratification over established factors including baseline CACS.ope

Primary cardiac chondrosarcoma mimicking chronic pulmonary thromboembolism in multimodality imaging

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An unusual cause of early aortic bioprosthetic valve failure

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Comparing the feasibility and accuracy of three-dimensional ultrasound to two-dimensional ultrasound and computed tomography angiography in the assessment of carotid atherosclerosis

AIMS: Two-dimensional ultrasound (2D-US) is the mainstay imaging technique used to evaluate carotid atherosclerosis. An automated single sweep three-dimensional ultrasound (3D-US) technique became available. We evaluated the feasibility and accuracy of 3D-US in the assessment of carotid plaques compared to those of 2D-US. Carotid computed tomography angiography (CTA) was used as a reference. METHODS AND RESULTS: Among 126 stroke patients who underwent carotid 2D-US, 73 underwent 3D-US and carotid CTA. 3D-US was pursued when there were carotid plaques or when area stenosis was ≥ 20% by 2D-US. Both 2D- and 3D-US images of the carotid arteries were acquired using a dedicated ultrasound system that was equipped with the single sweep volumetric transducer. In total, 266 arteries from 73 patients were selected for comparison of the detection rate of carotid plaques between 2D- and 3D-US. Among the 73 patients, carotid CTA detected 139 plaques. 3D-US demonstrated a higher detection rate of carotid plaques than did 2D-US (108 plaques (77.9%) vs. 70 plaques (50.4%)) when using carotid CTA as a reference standard. Carotid plaque volume (PV) of 133 vessels from 73 patients were quantitatively evaluated using both 3D-US and carotid CTA. Plaque volume of carotid artery was comparable between 3D-US and CTA (148.5 ± 133.0 mm3 vs. 154.1 ± 134.6 mm3 , P = .998, R: 0.9825, P-value for r < .001). CONCLUSION: 3D-US using a single sweep technique was a feasible and accurate method of detecting arterial plaques and assessing plaque volume.restrictio

Contemporary Multimodality Imaging for Cardiovascular Behçet Disease

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