Abstract
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.
Highlights
Pulmonary arterial hypertension (PAH) is a disease characterized by remodeling of the small pulmonary arteries, which leads to a progressive increase in pulmonary vascular resistance and right-sided heart failure and death.[1,2,3,4,5,6]
Demographic and baseline characteristics of all patients From January 2008 to December 2016, we identified 1,307 patients diagnosed with PAH (ICD codes I27.0x and I27.2x) for the first time with at least one drug prescription (CCB, prostacyclin analogues, endothelin receptor antagonists (ERA), or phosphodiesterase-5 inhibitors (PDE5-i)) (Fig 1)
We report the clinical data of 1,307 patients with PAH in Korea in this article
Summary
Pulmonary arterial hypertension (PAH) is a disease characterized by remodeling of the small pulmonary arteries, which leads to a progressive increase in pulmonary vascular resistance and right-sided heart failure and death.[1,2,3,4,5,6] Over the past decade, PAH-specific drugs such as prostanoids, endothelin receptor antagonists (ERA), and phosphodiesterase-5 inhibitors (PDE5-i) that can alter the natural history of PAH, have been developed [7,8,9,10,11]; albeit not satisfactorily, clinical outcome of PAH has improved.[12,13,14]Several PAH registries including the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) have provided basic information on PAH in terms of its epidemiology, clinical characteristics, and prognosis.[15,16,17,18] most data have derived from registries of Western populations, leaving the Asian population incompletely evaluated. Pulmonary arterial hypertension (PAH) is a disease characterized by remodeling of the small pulmonary arteries, which leads to a progressive increase in pulmonary vascular resistance and right-sided heart failure and death.[1,2,3,4,5,6] Over the past decade, PAH-specific drugs such as prostanoids, endothelin receptor antagonists (ERA), and phosphodiesterase-5 inhibitors (PDE5-i) that can alter the natural history of PAH, have been developed [7,8,9,10,11]; albeit not satisfactorily, clinical outcome of PAH has improved.[12,13,14]. The natural history of PAH in the era of PAH-specific drugs and a comprehensive investigation of the use of PAH-specific medications have not been adequately investigated. We assessed the prognosis and the pattern of using PAH-specific drugs in incident PAH patients, using the Korean Health Insurance Review and Assessment Service (HIRA) database of medical claims
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