Baseline characteristics of the Korean registry of pulmonary arterial hypertension

KORPAH Investigators

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 ± 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.

Original languageEnglish
Pages (from-to)1429-1438
Number of pages10
JournalJournal of Korean medical science
Volume30
Issue number10
DOIs
Publication statusPublished - 2015 Jan 1

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Pulmonary Hypertension
Registries
Connective Tissue Diseases
Survival Rate
Doppler Echocardiography
Therapeutics
Cardiac Catheterization
Systemic Lupus Erythematosus
Internet
Early Diagnosis
Heart Diseases
Epidemiology
Survival
Incidence

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

@article{25dceae9a67545cf9681e58f29478294,
title = "Baseline characteristics of the Korean registry of pulmonary arterial hypertension",
abstract = "Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 ± 15.7 yr, and 503 (80.5{\%}) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8{\%}) and Doppler echocardiography (n = 376, 60.2{\%}). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8{\%}, 25.4{\%}, and 23.2{\%}, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4{\%}. In total, 380 (60.8{\%}) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9{\%}) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8{\%}, 87.8{\%}, and 84.4{\%}, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.",
author = "{KORPAH Investigators} and Chung, {Wook Jin} and Park, {Yong Bum} and Jeon, {Chan Hong} and Jung, {Jo Won} and Ko, {Kwang Phil} and Choi, {Sung Jae} and Seo, {Hye Sun} and Lee, {Jae Seung} and Jung, {Hae Ok} and Chang, {Sung A.} and Lee, {Sang Do} and Lee, {Sang Min} and Kim, {Ki Jo} and Kang, {Kwi Young} and Lee, {Choong Won} and Lim, {Mie Jin} and Park, {Yong Beom} and Won Park and Park, {Sung Hwan} and Jun, {Jae Bum} and Hojoong Kim and Yoo, {Byung Su} and Lee, {Young Ho} and Jung, {Sang Youn} and Kichul Shin and Lee, {Sung Ji} and Choi, {Hyo Jin} and Kim, {Hyun A.} and Suh, {Chang Hee} and Ji, {Jong Dae} and Koh, {Eun Mi} and Cha, {Hoon Suk} and Lee, {Kwang Hoon} and Yonggil Kim and Lee, {Shin Seok} and Nah, {Seong Su} and Kwon, {Seong Ryul} and Yoon, {Bo Young} and Lee, {Eun Young} and Kim, {Hyun Sook} and Moon, {Ki Won} and Park, {Yong Wook} and Shin, {Jin Ho} and Ahn, {Joong Kyong} and Yoo, {Dae Hyun} and Jaejoon Lee and Kim, {Seong Ho} and Kim, {Gi Beom} and Kang, {I. Seok} and Choi, {Jae Young}",
year = "2015",
month = "1",
day = "1",
doi = "10.3346/jkms.2015.30.10.1429",
language = "English",
volume = "30",
pages = "1429--1438",
journal = "Journal of Korean Medical Science",
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}

Baseline characteristics of the Korean registry of pulmonary arterial hypertension. / KORPAH Investigators.

In: Journal of Korean medical science, Vol. 30, No. 10, 01.01.2015, p. 1429-1438.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Baseline characteristics of the Korean registry of pulmonary arterial hypertension

AU - KORPAH Investigators

AU - Chung, Wook Jin

AU - Park, Yong Bum

AU - Jeon, Chan Hong

AU - Jung, Jo Won

AU - Ko, Kwang Phil

AU - Choi, Sung Jae

AU - Seo, Hye Sun

AU - Lee, Jae Seung

AU - Jung, Hae Ok

AU - Chang, Sung A.

AU - Lee, Sang Do

AU - Lee, Sang Min

AU - Kim, Ki Jo

AU - Kang, Kwi Young

AU - Lee, Choong Won

AU - Lim, Mie Jin

AU - Park, Yong Beom

AU - Park, Won

AU - Park, Sung Hwan

AU - Jun, Jae Bum

AU - Kim, Hojoong

AU - Yoo, Byung Su

AU - Lee, Young Ho

AU - Jung, Sang Youn

AU - Shin, Kichul

AU - Lee, Sung Ji

AU - Choi, Hyo Jin

AU - Kim, Hyun A.

AU - Suh, Chang Hee

AU - Ji, Jong Dae

AU - Koh, Eun Mi

AU - Cha, Hoon Suk

AU - Lee, Kwang Hoon

AU - Kim, Yonggil

AU - Lee, Shin Seok

AU - Nah, Seong Su

AU - Kwon, Seong Ryul

AU - Yoon, Bo Young

AU - Lee, Eun Young

AU - Kim, Hyun Sook

AU - Moon, Ki Won

AU - Park, Yong Wook

AU - Shin, Jin Ho

AU - Ahn, Joong Kyong

AU - Yoo, Dae Hyun

AU - Lee, Jaejoon

AU - Kim, Seong Ho

AU - Kim, Gi Beom

AU - Kang, I. Seok

AU - Choi, Jae Young

PY - 2015/1/1

Y1 - 2015/1/1

N2 - Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 ± 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.

AB - Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 ± 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.

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