Background and Objectives: Pulmonary arterial hypertension (PAH) is a rare but fatal disease. Recent advances in PAH-specific drugs have improved its outcomes, although the healthcare burden of novel therapeutics may lead to a discrepancy in outcomes between developing and developed countries. We analyzed how the epidemiology and clinical features of PAH has changed through the rapidly advancing healthcare infrastructure in South Korea. Methods: PAH was defined according to a newly devised 3-component algorithm. Using a nationwide health insurance claims database, we delineated annual trends in the prevalence, incidence, medication prescription pattern, and 5-year survival of PAH in Korea. Cumulative survival and potential predictors of mortality were also assessed among 2,151 incident PAH cases. Results: Between 2002 or 2004 and 2018, the prevalence and incidence of PAH increased 75-fold (0.4 to 29.9 per million people) and 12-fold (0.5 to 6.3 per million person-years), respectively. The proportion of patients on combination PAH-specific drug therapy has also steadily increased up to 29.0% in 2018. Among 2,151 incident PAH cases (median [interquartile range] age, 50 [37–62] years; 67.2% female), the 5-year survival rate and median survival duration were 71.8% and 13.1 years, respectively. Independent predictors of mortality were age, sex, etiology of PAH, diabetes, dyslipidemia, and chronic kidney disease. Conclusions This nationwide study delineated that the prevalence and incidence of PAH have grown rapidly in Korea since the early 2000s. The use of combination therapy has also increased, and the 5-year survival rate of PAH in Korea was similar to those in western countries.

Purpose: K-MASTER project is a Korean national precision medicine platform that screened actionable mutations by analyzing next-generation sequencing (NGS) of solid tumor patients. We compared gene analyses between NGS panel from the K-MASTER project and orthogonal methods. Materials and Methods: Colorectal, breast, non–small cell lung, and gastric cancer patients were included. We compared NGS results from K-MASTER projects with those of non-NGS orthogonal methods (KRAS, NRAS, and BRAF mutations in colorectal cancer [CRC]; epidermal growth factor receptor [EGFR], anaplastic lymphoma kinase [ALK] fusion, and reactive oxygen species 1 [ROS1] fusion in non–small cell lung cancer [NSCLC], and Erb-B2 receptor tyrosine kinase 2 (ERBB2) positivity in breast and gastric cancers). Results: In the CRC cohort (n=225), the sensitivity and specificity of NGS were 87.4% and 79.3% (KRAS); 88.9% and 98.9% (NRAS); and 77.8% and 100.0% (BRAF), respectively. In the NSCLC cohort (n=109), the sensitivity and specificity of NGS for EGFR were 86.2% and 97.5%, respectively. The concordance rate for ALK fusion was 100%, but ROS1 fusion was positive in only one of three cases that were positive in orthogonal tests. In the breast cancer cohort (n=260), ERBB2 amplification was detected in 45 by NGS. Compared with orthogonal methods that integrated immunohistochemistry and in situ hybridization, sensitivity and specificity were 53.7% and 99.4%, respectively. In the gastric cancer cohort (n=64), ERBB2 amplification was detected in six by NGS. Compared with orthogonal methods, sensitivity and specificity were 62.5% and 98.2%, respectively. Conclusion The results of the K-MASTER NGS panel and orthogonal methods showed a different degree of agreement for each genetic alteration, but generally showed a high agreement rate.

Pulmonary hypertension is a rare and progressive illness with a devastating prognosis. Promising research efforts have advanced the understanding and recognition of the pathobiology of pulmonary hypertension. Despite remarkable achievements in terms of improving the survival rate, reducing disease progression, and enhancing quality of life, pulmonary arterial hypertension (PAH) is not completely curable. Therefore, an effective treatment strategy is still needed. Recently, many studies of the underlying molecular mechanisms and technological developments have led to new approaches and paradigms for PAH treatment. Management based on stem cells and related paracrine effects, epigenetic drugs and gene therapies has yielded prospective results for PAH treatment in preclinical research. Further trials are ongoing to optimize these important insights into clinical circumstances.

Purpose: Heart failure (HF) poses significant morbidity and mortality. Recently, the ventriculo-vascular coupling index (VVI) was introduced as an independent prognostic factor reflective of the overall cardiovascular performance index in HF. We aimed to determine the effectiveness of force-titration of valsartan on VVI values in HF patients. Materials and Methods: In this multicenter and prospective observational trial, the effect of valsartan was stratified according to dosages [non-ceiling dose (NCD) vs. ceiling dose (CD)] in HF patients with left ventricular ejection fraction (LVEF) <55%. Biochemical studies, including N-terminal pro-B-type natriuretic peptide (NT-proBNP), echocardiography with VVI, the treadmill test, and the activity scale index were assessed at baseline and after 24 weeks of treatment. Results: One-hundred thirty-eight patients were force-titrated to either a CD group (n=81) or a NCD group (n=57). The mean age of the study participants was 59 years and 66% were male. After 6 months of follow up, left ventricular mass index (LVMI) values had significantly improved in the CD group but not in the NCD group. Intriguingly, in HF patients with a reduced ejection fraction (HFrEF) (n=52, LVEF <40%), a significant improvement in VVI was only observed in the CD group (from 2.4±0.6 to 1.8±0.5, p<0.001). Conclusion CDs of valsartan for 6 months showed better improvement in VVI, as well as LVMI, in patients with HFrEF, compared with NCDs.

Purpose: Heart failure (HF) poses significant morbidity and mortality. Recently, the ventriculo-vascular coupling index (VVI) was introduced as an independent prognostic factor reflective of the overall cardiovascular performance index in HF. We aimed to determine the effectiveness of force-titration of valsartan on VVI values in HF patients. Materials and Methods: In this multicenter and prospective observational trial, the effect of valsartan was stratified according to dosages [non-ceiling dose (NCD) vs. ceiling dose (CD)] in HF patients with left ventricular ejection fraction (LVEF) <55%. Biochemical studies, including N-terminal pro-B-type natriuretic peptide (NT-proBNP), echocardiography with VVI, the treadmill test, and the activity scale index were assessed at baseline and after 24 weeks of treatment. Results: One-hundred thirty-eight patients were force-titrated to either a CD group (n=81) or a NCD group (n=57). The mean age of the study participants was 59 years and 66% were male. After 6 months of follow up, left ventricular mass index (LVMI) values had significantly improved in the CD group but not in the NCD group. Intriguingly, in HF patients with a reduced ejection fraction (HFrEF) (n=52, LVEF <40%), a significant improvement in VVI was only observed in the CD group (from 2.4±0.6 to 1.8±0.5, p<0.001). Conclusion CDs of valsartan for 6 months showed better improvement in VVI, as well as LVMI, in patients with HFrEF, compared with NCDs.

0.9 between all observers). Receiver operating characteristic curve analysis showed that the predictive power for CAD was improved when max-CIMT and plaque information (plaque≥2) was added [area under the curve (AUC): 0.838] to the traditional clinical CV risk factors (AUC: 0.769). The cutoff values for CAD prediction with the standard device and the WHUS device were 1.05 mm (AUC: 0.807, sensitivity: 0.78, specificity: 0.53) and 1.10 mm (AUC: 0.725, sensitivity: 0.98, specificity: 0.27), respectively.CONCLUSION: max-CIMT measured by a WHUS device showed excellent agreement and repeatability, compared with standard ultrasound. Combined max-CIMT and plaque information added predictive power to the traditional clinical CV risk factors in detecting high-risk CAD patients.]]>
Carotid Artery, Common ; Carotid Intima-Media Thickness ; Coronary Angiography ; Coronary Artery Disease ; Humans ; Mass Screening ; Risk Factors ; ROC Curve ; Sensitivity and Specificity ; Ultrasonography ; Wireless Technology

Background: There is little data regarding types of idiopathic premature ventricular complex (PVC) according to heart rate dependence. Methods: One hundred and sixty-eight patients with idiopathic PVC were enrolled in this study. Evaluation of the number of PVCs and total ventricular beats, and the density of PVC was done using 24 h Holter monitoring. Patients were divided into groups as having: fast rate-dependent (Group I), slow rate-dependent (Group II), and heart rateindependent PVC (Group III) based on the relationship between the number of hourly PVC and hourly heart rate. After ß-blocker medication, 24 h Holter monitoring was repeated. Results: Among the 168 subjects, 66 (39.3%) patients were in Group I, 18 (10.7%) in Group II, and 84 (50.0%) in Group III. There were no significant differences in the baseline number of PVCs and total ventricular beats, and the density of PVC among the three groups. The number of PVCs was significantly reduced in patients with Group I (14,030 ± 11,463 beats/day vs. 7401 ± 10,464 beats/day, p < 0.001), and total ventricular beat was significantly reduced in patients with Group I (109,223 ± 17,564 beats/day vs. 96,182 ± 15,594 beats/day, p < 0.001) and Group III (106,515 ± 13,468 beats/ day vs. 97,995 ± 12,960 beats/day, p < 0.001) after ß-blocker medication. The density of PVC was significantly reduced only in patients of Group I (12.9 ± 10.3% vs. 7.4 ± 10.3%, p = 0.001) after ß-blocker medication. Conclusions The type of PVC according to the heart rate dependence should be considered when treating idi‑ opathic PVC with ß-blockers.

Pulmonary arterial hypertension (PAH) is characterized by remodeling of the distal pulmonary arteries resulting in high pulmonary vascular resistance and, eventually, right ventricular heart failure. Although current advances in PAH therapy have improved outcomes, poor survival remains a reality worldwide, including Korea. One of the most important issues in PAH is the late diagnosis, since screening or diagnostic efforts are often overlooked due to the rarity of disease. Data from Korean registries and observational cohorts show that delayed detection leads to increased morbidity. Additionally, low percentages of Korean patients are committed to intensive PAH-targeted therapy. Current Korean health insurance policies' lack of coverage for new PAH-targeted drugs and upfront combination therapy may also hamper the improvement of treatment outcomes. Understanding individual variability in response to therapeutics through deep phenotyping is a novel strategy that should be considered when treating PAH. Overall, early detection of PAH by proactive screening together with early, intensive, individualized PAH therapy using deep phenotyping is crucial for improving prognoses for PAH patients in Korea.