Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

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 40.6±17.4 years) diagnosed and/or treated between 1980 and 2021 in our PAH clinic. Given targeted therapy’s introduction and active use since 2007, we compared diagnostic classification, demographics, and treatment patterns at that juncture. Survival analysis encompassed PAH types and the overall population. For historical survival comparison, 50 non-registry patients were retrospectively added, and age-sex matching enabled pooled analysis. Results: Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) constituted the largest subset (43.0%), trailed by connective tissue disease-associated PAH (CTD-PAH, 29.6%) and idiopathic PAH (IPAH, 19.1%). Post-2007, CTD-PAH proportions surged, notably with an elevated initiation rate of targeted therapy (95.4%). Overall survival rates at 1, 5, and 10 years stood at 91.3%, 77.4%, and 65.8%, respectively, with CHD-PAH exhibiting superior survival to idiopathic or CTD-PAH. Age-sex matching analysis indicated survival disparities between those starting immediate targeted therapy vs. conservative treatment upon diagnosis, especially driven by IPAH. Conclusions In the post-introduction of the targeted therapy era, patients with PAH promptly started treatment right away, and higher survival rates of patients who started initial PAH-targeted therapy were demonstrated. The transition towards early treatment initiation might have likely contributed to the elevated survival rates observed in Korea’s PAH patient cohort.

Purpose: To determine the risk of incidence, prevalence, survival, and death risk for Marfan syndrome (MFS) in Korea. Methods: MFS (10th revision of the International Statistical Classification of Diseases [ICD-10] code: Q87.4) data were collected from the Korea National Health Insurance Service between 2006 and 2017. Simple and multiple Cox proportional hazards analyses were performed. The death data of Koreans from 2006 to 2018 were used. Results: The mean age of patients with MFS was 27.2± 16.8 years, 26.5± 15.9 years for males and 28.2± 18.1 years for females (P < 0.01), and the proportion of males was 59.5%. The age-standardized incidence and prevalence in 2017 were 0.51 and 5.70 per 100,000 persons, respectively. The 10-year survival rate was 92.7%. Adjusted hazard ratio increased significantly with age, male sex, heart failure, ischemic stroke, hemorrhagic stroke, chronic kidney disease, malignant neoplasm, aortic dissection, and abdominal aortic aneurysm. Conclusion Although the overall 10-year survival rate of MFS was over 90%, the adjusted hazard ratio was significantly higher in the older age group, male sex, and individuals with heart failure, stroke, chronic kidney disease, malignant neoplasm, aortic dissection, and abdominal aortic aneurysm. These results suggest the need for meticulous surveillance of aortopathy in patients with MFS, as it may portend a worse prognosis in aortic dissection and aneurysm, and in older age groups with MFS.

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 40.6±17.4 years) diagnosed and/or treated between 1980 and 2021 in our PAH clinic. Given targeted therapy’s introduction and active use since 2007, we compared diagnostic classification, demographics, and treatment patterns at that juncture. Survival analysis encompassed PAH types and the overall population. For historical survival comparison, 50 non-registry patients were retrospectively added, and age-sex matching enabled pooled analysis. Results: Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) constituted the largest subset (43.0%), trailed by connective tissue disease-associated PAH (CTD-PAH, 29.6%) and idiopathic PAH (IPAH, 19.1%). Post-2007, CTD-PAH proportions surged, notably with an elevated initiation rate of targeted therapy (95.4%). Overall survival rates at 1, 5, and 10 years stood at 91.3%, 77.4%, and 65.8%, respectively, with CHD-PAH exhibiting superior survival to idiopathic or CTD-PAH. Age-sex matching analysis indicated survival disparities between those starting immediate targeted therapy vs. conservative treatment upon diagnosis, especially driven by IPAH. Conclusions In the post-introduction of the targeted therapy era, patients with PAH promptly started treatment right away, and higher survival rates of patients who started initial PAH-targeted therapy were demonstrated. The transition towards early treatment initiation might have likely contributed to the elevated survival rates observed in Korea’s PAH patient cohort.

Purpose: Few studies have assessed the prevalence of temporomandibular disorders (TMD) in the Korean population. Methods: We used cohort data from the Korean National Health Insurance Service between 2012 and 2020. The data consisted of main diagnoses related to TMD (International Classification of Diseases 10th Revision [ICD-10] code: K07.6 and K07.6x). The age-standardized prevalence of TMD was calculated using the estimated Korean population in 2020 as a reference. Results: The age-standardized prevalence of TMD increased from 604 persons per 100,000 persons in 2012 to 869 persons in 2020. In 2020, the overall age-standardized prevalence was 1,355 persons in the 10 to 19 years age group, 1,809 persons in the 20 to 29 years age group, and 979 persons in the 30 to 39 years age group. The age-standardized prevalence was approximately 1.5 times higher in females than in males (698 persons in males vs. 1,040 persons in females). Among the specific TMD subtypes, the age-standardized prevalence of internal derangement of the temporomandibular joint and pain in the temporomandibular joint, not classified elsewhere, was higher than that of other specific TMDs. Conclusion The overall age-standardized prevalence of TMD was higher in the 10s, 20s, and 30s age groups and in females between 2012 and 2020. The age-standardized prevalence of internal derangement of temporomandibular joint was the highest among specific TMD subtypes.

Purpose: This study aimed to assess the survival rate (SR) and death risk for associated pulmonary arterial hypertension (aPAH; 10th revision of the International Statistical Classification of Diseases [ICD-10], I27.2) in Koreans. Methods: The data were collected from the Korean National Health Insurance Service from 2006 through 2017 (n= 15,448). We analyzed the SR using the Kaplan-Meier method and carried out Cox proportional hazards analyses. Results: Patients’ mean age upon aPAH diagnosis was 60.1±24.0 years, and 60.7% of the patients were female. The 10-year SR of aPAH was 46.3% (95% confidence interval, 45.0 to 47.6). The factors associated with an increase in the adjusted death risk included age of 0 to 9 years, advancing age, male sex, lower income level, and comorbidities including diabetes mellitus, myocardial infarction, heart failure, hemorrhagic stroke, chronic kidney disease, malignant neoplasm, hereditary hemorrhagic telangiectasia, and systemic lupus erythematosus. Conclusion The 10-year SR of aPAH was over 46%. The risk of death from aPAH was significantly higher with advancing age, sex, lower income level, and comorbidities.

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 40.6±17.4 years) diagnosed and/or treated between 1980 and 2021 in our PAH clinic. Given targeted therapy’s introduction and active use since 2007, we compared diagnostic classification, demographics, and treatment patterns at that juncture. Survival analysis encompassed PAH types and the overall population. For historical survival comparison, 50 non-registry patients were retrospectively added, and age-sex matching enabled pooled analysis. Results: Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) constituted the largest subset (43.0%), trailed by connective tissue disease-associated PAH (CTD-PAH, 29.6%) and idiopathic PAH (IPAH, 19.1%). Post-2007, CTD-PAH proportions surged, notably with an elevated initiation rate of targeted therapy (95.4%). Overall survival rates at 1, 5, and 10 years stood at 91.3%, 77.4%, and 65.8%, respectively, with CHD-PAH exhibiting superior survival to idiopathic or CTD-PAH. Age-sex matching analysis indicated survival disparities between those starting immediate targeted therapy vs. conservative treatment upon diagnosis, especially driven by IPAH. Conclusions In the post-introduction of the targeted therapy era, patients with PAH promptly started treatment right away, and higher survival rates of patients who started initial PAH-targeted therapy were demonstrated. The transition towards early treatment initiation might have likely contributed to the elevated survival rates observed in Korea’s PAH patient cohort.