Background And Objectives: The transformation of pulmonary arterial hypertension (PAH) treatment in Korea, ushered by targeted therapy's advent, prompted our analysis of baseline attributes, treatment trends, and survival shifts within our single-center registry.
Methods: We examined 230 patients (72.6% female, mean age 40.
This brief report reviews the clinical, procedural, and imaging data of 7 patients with p.Arg4810Lys variant of the ring finger protein 213 (RNF213) gene-related peripheral pulmonary arterial stenosis who underwent percutaneous transluminal pulmonary angioplasties (PTPAs) for demographics, clinical presentation, indications for angioplasty, and procedural and clinical outcomes. During median follow-up of 64.
View Article and Find Full Text PDFBackground And Objectives: The recent developments in chronic thromboembolic pulmonary hypertension (CTEPH) are emphasizing the multidisciplinary team. We report on the changes in clinical practice following the development of a multidisciplinary team, based on our 7 years of experience.
Methods: Multidisciplinary team was established in 2015 offering both balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) with technical upgrades by internal and external expertise.
Background: Recently, a ring-type cuffless blood pressure (BP) measuring device has been developed. This study was a prospective, single arm, first-in-human pivotal trial to evaluate accuracy of BP measurement by the new device.
Methods: The ring-type smart wearable monitoring device measures photoplethysmography signals from the proximal phalanx and transmits the data wirelessly to a connected smartphone.
Background: Despite the 2015 American College of Medical Genetics and Genomics (ACMG) and Association of Molecular Pathology (AMP) guideline, many variants of gene remain inconclusive. In line with publication of the specific variant interpretation guideline by ClinGen in 2022, we reassessed variants of uncertain significance (VUS) in gene found in our institution.
Methods: VUS found in the course of sequencing between December 2015 and April 2022 were reassessed based on -specific variant interpretation guideline, review of updated literatures and additional genetic tests including family study and/or RNA study if available.
Purpose: This regulatory post-marketing surveillance (PMS) was organized to identify the safety and effectiveness of ambrisentan in the Korean population.
Method: This was an open-label, multi-center PMS conducted from 31 institutions in Korea for 6 years from August 2015 to 2021, to evaluate the use of ambrisentan for the treatment of pulmonary arterial hypertension (PAH). Inclusion criteria are Korean subjects with the World Health Organization functional classification (WHO Fc) II or III PAH who are new users or repeated users with ambrisentan (Volibris®) Tablet 5 or 10 mg per day (age >18 years old).
Background: Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. Pulmonary arterial hypertension (PAH) and PVOD/PCH are clinically similar, but there is a risk of drug-induced pulmonary edema when PCH patients receive the PAH therapy. Therefore, early diagnosis of PVOD/PCH is important.
View Article and Find Full Text PDFWe aimed to evaluate the additive value of cardiovascular magnetic resonance imaging (CMR) and cardiopulmonary exercise test (CPET) to predict clinical outcomes in patients with HCM. We enrolled 373 patients with HCM and normal left ventricular systolic function who underwent CPET and CMR. The primary outcome was a clinical composite of all-cause death, cardiac transplantation, stroke, heart failure requiring hospitalization and defibrillator implantation.
View Article and Find Full Text PDFBackground And Objectives: Several cases involving severe right ventricular (RV) failure in advanced cancer patients have been found to be pulmonary tumor thrombotic microangiopathies (PTTMs). This study aimed to discover the nature of rapid RV failure syndrome with a suspected diagnosis of PTTM for better diagnosis, treatment, and prognosis prediction in clinical practice.
Methods: From 2011 to 2021, all patients with clinically suspected PTTM were derived from the one tertiary cancer hospital with more than 2000 in-hospital bed.
Aims: To investigate whether left arterial reservoir strain (LASr) could predict new-onset atrial fibrillation (NOAF) in patients with light-chain-type cardiac amyloidosis (ALCA).
Methods And Results: This study enrolled 427 patients with CA from two tertiary centres between 2005 and 2019. LASr was measured using a vendor-independent analysis programme.
Background And Objective: Macitentan is approved for treating pulmonary arterial hypertension. However, the real-world evidence of macitentan use is limited. Therefore, we evaluated the safety and clinical outcomes of macitentan use in clinical practice under a post-marketing surveillance.
View Article and Find Full Text PDFAims: Tricuspid valve (TV) surgery for functional tricuspid regurgitation (TR) is becoming more common, but the associated mortality remains high. Therefore, we evaluated the clinical and echocardiographic parameters associated with all-cause mortality in patients with severe functional TR who underwent TV surgery.
Methods And Results: A total of 286 patients with severe functional TR who underwent TV replacement or repair was analysed between January 2006 and December 2017.
Hypertrophic cardiomyopathy (HCM) is one of the most common inheritable cardiomyopathies. Contemporary management strategies, including the advent of implantable cardioverter-defibrillators and effective anticoagulation, have substantially improved the clinical course of HCM patients; however, the disease burden of HCM is still high in Korea. Sudden cardiac death (SCD), atrial fibrillation and thromboembolic risk, dynamic left ventricular outflow tract (LVOT) obstruction, and heart failure (HF) progression remain important issues in HCM.
View Article and Find Full Text PDFBackground: Optimizing an individual dose with careful management of adverse events (AEs) is essential in the treatment with selexipag approved for pulmonary arterial hypertension (PAH). This study aims to identify real-world practice patterns and AE characteristics of selexipag.
Research Design And Methods: This multicenter, longitudinal, observational study included Korean patients with PAH who initiated with selexipag and were followed up to 24 weeks.
Background: Few studies used nationwide data to assess the survival rates (SRs) and death risk for idiopathic pulmonary arterial hypertension (IPAH; ICD-10 I27.0) in Korea.
Methods: IPAH data (N = 9,017; female:male = 6:4) were collected from the National Health Insurance Service in Korea, from 2006 through 2017.