Background and Objectives: Inherited arrhythmia (IA) is a more common cause of sudden cardiac death in Asian population, but little is known about the genetic background of Asian IA probands. We aimed to investigate the clinical characteristics and analyze the genetic underpinnings of IA in a Korean cohort. Methods: This study was conducted in a multicenter cohort of the Korean IA Registry from 2014 to 2017. Genetic testing was performed using a next-generation sequencing panel including 174 causative genes of cardiovascular disease. Results: Among the 265 IA probands, idiopathic ventricular fibrillation (IVF) and Brugada Syndrome (BrS) was the most prevalent diseases (96 and 95 cases respectively), followed by long QT syndrome (LQTS, n=54). Two-hundred-sixteen probands underwent genetic testing, and 69 probands (31.9%) were detected with genetic variant, with yield of pathogenic or likely pathogenic variant as 6.4%. Left ventricular ejection fraction was significantly lower in genotype positive probands (54.7±11.3 vs. 59.3±9.2%, p=0.005). IVF probands showed highest yield of positive genotype (54.0%), followed by LQTS (23.8%), and BrS (19.5%). Conclusions There were significant differences in clinical characteristics and genetic yields among BrS, LQTS, and IVF. Genetic testing did not provide better yield for BrS and LQTS. On the other hand, in IVF, genetic testing using multiple gene panel might enable the molecular diagnosis of concealed genotype, which may alter future clinical diagnosis and management strategies.

Background: Current international guidelines recommend against deep sedation as it is associated with worse outcomes in the intensive care unit (ICU). However, in Korea the prevalence of deep sedation and its impact on patients in the ICU are not well known. Methods: From April 2020 to July 2021, a multicenter, prospective, longitudinal, noninterventional cohort study was performed in 20 Korean ICUs. Sedation depth extent was divided into light and deep using a mean Richmond Agitation–Sedation Scale value within the first 48 hours. Propensity score matching was used to balance covariables; the outcomes were compared between the two groups. Results: Overall, 631 patients (418 [66.2%] and 213 [33.8%] in the deep and light sedation groups, respectively) were included. Mortality rates were 14.1% and 8.4% in the deep and light sedation groups (P = 0.039), respectively. Kaplan-Meier estimates showed that time to extubation (P < 0.001), ICU length of stay (P = 0.005), and death P = 0.041) differed between the groups. After adjusting for confounders, early deep sedation was only associated with delayed time to extubation (hazard ratio [HR], 0.66; 95% confidence inter val [CI], 0.55– 0.80; P < 0.001). In the matched cohort, deep sedation remained significantly associated with delayed time to extubation (HR, 0.68; 95% 0.56–0.83; P < 0.001) but was not associated with ICU length of stay (HR, 0.94; 95% CI, 0.79–1.13; P = 0.500) and in-hospital mortality (HR, 1.19; 95% CI, 0.65–2.17; P = 0.582). Conclusion In many Korean ICUs, early deep sedation was highly prevalent in mechanically ventilated patients and was associated with delayed extubation, but not prolonged ICU stay or in-hospital death.

The phenotypic variability of variant Turner syndrome is wide, ranging from characteristic clinical features to those that are hardly distinguishable from the general population. A 4-year-old girl presented with multiple brownish macules and patches on the trunk and upper extremities as well as axillary freckles. Exome sequencing and chromosomal microarray testing revealed a microdeletion at Xp22.33p22.11 leading to a diagnosis of Turner syndrome. Here we describe an unusual case of variant Turner syndrome with multiple café-au-lait spots.

Purpose: Here we report a case of posterior chamber intraocular lens (IOL) dislocation into the subconjunctival space (pesudophacocele) following ocular trauma.Case summary: A 66-year-old male presented with ocular pain and decreased vision in the right eye following trauma with a metallic rod. The patient had a history of trabeculectomy, glaucoma drainage device implantation, transscleral cyclophotocoagulation, and cataract surgery for uveitic glaucoma and a cataract in the right eye. On examination, vision was hand movement, the intraocular pressure was 3 mmHg, and subconjunctival hemorrhage and hyphema were observed. After the resolution of hemorrhage, uveal tissue prolapse was seen nasally behind the corneal limbus and the IOL was found to be dislocated into the nasal subconjunctival space. There were no changes in the filtering bleb and tube compared to the pre-trauma status. The IOL was removed through a conjunctival incision because the patient refused any active treatment. Conclusions Pesudophacocele developed in a patient who had a history of glaucoma and cataract surgery in the injured eye. The IOL could not be assessed immediately after the trauma because of subconjunctival hemorrhage and hyphema. When the status of IOL is unclear or suspected to be dislocated after trauma, the possibility of pseudophacocele should be considered, in addition to the dislocation into the vitreous cavity.

Background and Objectives: We investigated whether extra-pulmonary vein (PV) ablation targeting a high maximal slope of the action potential duration restitution curve (Smax) improves the rhythm outcome of persistent atrial fibrillation (PeAF) ablation. Methods: In this open-label, multi-center, randomized, and controlled trial, 178 PeAF patients were randomized with 1:1 ratio to computational modeling-guided virtual Smax ablation (V-Smax) or empirical ablation (E-ABL) groups. Smax maps were generated by computational modeling based on atrial substrate maps acquired during clinical procedures in sinus rhythm. Smax maps were generated during the clinical PV isolation (PVI). The V-Smax group underwent an additional extra-PV ablation after PVI targeting the virtual high Smax sites. Results: After a mean follow-up period of 12.3±5.2 months, the clinical recurrence rates (25.6% vs. 23.9% in the V-Smax and the E-ABL group, p=0.880) or recurrence appearing as atrial tachycardia (11.1% vs. 5.7%, p=0.169) did not differ between the 2 groups. The postablation cardioversion rate was higher in the V-Smax group than E-ABL group (14.4% vs. 5.7%, p=0.027). Among antiarrhythmic drug-free patients (n=129), the AF freedom rate was 78.7% in the V-Smax group and 80.9% in the E-ABL group (p=0.776). The total procedure time was longer in the V-Smax group (p=0.008), but no significant difference was found in the major complication rates (p=0.497) between the groups. Conclusions Unlike a dominant frequency ablation, the computational modeling-guided V-Smax ablation did not improve the rhythm outcome of the PeAF ablation and had a longer procedure time.

Background: Green nail syndrome (GNS) is characterized by a greenish discoloration of the nail with accompanying nail apparatus disorders, such as onycholysis or paronychia. To date, data on the clinical characteristics of GNS are limited, especially in Korea. Objective: This study aimed to investigate the clinical characteristics of GNS. Methods: We retrospectively reviewed the medical records and clinical photographs of 78 patients with 91 GNS lesions diagnosed at the Pusan National University Hospitals (Busan and Yangsan) from 2009 to 2021. Results: Among 78 patients, 47 (60.3%) were female and 31 male (39.7%), with a mean age of 53.1 years (23∼82 years). This study included 56 fingernail (61.5%) and 35 toenail lesions (38.5%). Thumbnails and great toenails were the most common site, with 62 lesions (68.1%). Most patients presented predisposing conditions, such as frequent exposure to wet conditions (55.1%), immunosuppressive conditions (26.9%), and trauma history (29.5%). The most common colors, shapes, and eccentricities of GNS were blackish-green (31.9%), reverse triangle (53.8%), and distal eccentricity (42.9%), respectively. The GNS was accompanied by various nail diseases that mostly (80.2%) preceded the syndrome, and onycholysis was the most common (83.5%). Based on the involvement of the nail surface or subsurface, GNS can be divided into the superficial (15.4%), subungual (73.6%), and mixed (11.0%) subtypes, each of which shows different clinical features, such as multiplicity, color, shape, and eccentricity. Conclusion This is the largest-scale study showing the clinical characteristics of GNS and can be helpful for dermatologists who usually and primarily treat GNS.

Purpose: To evaluate the dynamic range of retinal nerve fiber layer (RNFL) and optic nerve head (ONH) parameters measured using optical coherence tomography (OCT) in conditions ranging from nonglaucomatous status to advanced glaucoma by longitudinal observation. Methods: A total of 15 eyes from 12 participants with glaucoma progression from a nonglaucomatous status to advanced glaucoma were included. The RNFL and ONH parameters were compared between the nonglaucomatous and advanced stages within the same eye. The absolute and relative changes in OCT parameters were analyzed. Results: The median highest intraocular pressure was 42.5 mmHg (interquartile range, 37.5 to 54.5 mmHg), and the final mean deviation of the visual field test was –24.68 dB (interquartile range, –23.93 to –31.13 dB). The median relative changes in RNFL thickness were –40.6% in the overall area, and –51.9%, –21.4%, –51.1%, and –41.8% in the superior, nasal, inferior, and temporal quadrants, respectively (all p < 0.05). Relative changes in the rim area, disc area, average cup to disc ratio, vertical cup to disc ratio, and cup volume were –56.64%, 0.59%, 62.10%, 66.0%, and 337.90%, respectively (all p < 0.05, except for disc area with a p-value of 0.753). Conclusions The dynamic range of the RNFL thickness ranged from 40.6% to 51.9%, and the dynamic range of the ONH parameters ranged from 56.64% to 337.90%. During the course of glaucoma progression, the cup volume showed the widest dynamic range. However, the disc area did not show significant changes.

Background/Aims: Atrial fibrillation (AF)-related stroke accounts for 20% of ischemic strokes. Rivaroxaban use in AF patients for preventing stroke and systemic embolism was approved in 2013 in Korea. This study was to investigate the safety and effectiveness of rivaroxaban use in Korean patients with non-valvular AF in a real-world setting. Methods: This was an analysis of the Korean patients in Xarelto for Prevention of Stroke in Patients with Atrial Fibrillation in Asia-Pacific (XANAP), which was a prospective, observational cohort study including patients with non-valvular AF starting rivaroxaban treatment to prevent stroke or non-central nervous system systemic embolism (non-CNS SE), conducted in 10 Asian countries. Results: A total of 844 patients were enrolled in the Korean portion of the XANAP study. In XANAP Korea, the mean age was 70.1 years and 62.6% were males. The mean CHADS2 score was 2.5 and the mean CHA2DS2-VASc score was 3.8. 47% of the patients had experienced prior stroke or non-CNS SE or transient ischemic attack. 73.6% of the patients had CHADS2 score ≥ 2. Incidence proportions of 0.8% of the patients (1.1 per 100 patient-years) developed adjudicated treatment-emergent major bleeding. Death was observed in 1.2% of the patients. The incidence of non-major bleeding as well as thromboembolic event were 8.4% (11.6 per 100 patient-years) and 1.5% (2.0 per 100 patient-years), respectively. Conclusions This study reaffirmed the consistent safety profile of rivaroxaban. We found consistent results with overall XANAP population for rivaroxaban in terms of safety in non-valvular AF patients for the prevention of stroke and non-CNS SE.

Objectives: . The aim of this study was to investigate whether preserved vestibular function in the high-frequency range influences the prognosis of patients with bilateral vestibulopathy (BVP) after vestibular rehabilitation. Methods: . Twenty-four patients followed up with vestibular rehabilitation were recruited. The enrolled patients were divided into two groups according to the preservation of the high-frequency vestibulo-ocular reflex (VOR) based on the video head impulse test (vHIT). The results of computerized dynamic posturography and the Dizziness Handicap Inventory (DHI) survey collected at baseline and at the 6-month follow-up after vestibular rehabilitation therapy were analyzed. Results: . Both groups showed significantly increased composite and DHI scores after follow-up with vestibular rehabilitation. The group with preserved high-frequency VOR showed a better composite score (p=0.064) and vestibular score (p= 0.008) than the group with lost high-frequency VOR at the 6-month follow up. The DHI score significantly decreased only in the group with lost high-frequency VOR (p=0.047). Among the three vestibular function tests (caloric test, rotary chair test, and vHIT) used to diagnose BVP, only vHIT showed a significant correlation (p=0.015) with a favorable prognosis (composite score ≥70). Conclusion . Better treatment outcomes are likely in patients with BVP with preserved vestibular function in response to high-frequency stimulation, as measured by the vHIT.