https://scholars.lib.ntu.edu.tw/handle/123456789/525231
Title: | Congenital heart disease with pulmonary artery hypertension in an Asian cohort-initial report from TACHYON (TAiwan congenital heart disease associated with pulmonarY arterial hypertension) registry | Authors: | SHUENN-NAN CHIU Weng K.-P. Lin M.-C. Wang J.-N. Hwang B.-T. Dai Z.-K. Lin S.-M. Chang J.-S. Lin I.-C. MEI-HWAN WU CHUN-WEI LU MING-TAI LIN CHUN-AN CHEN Hua Y.-C. Wu J.-M. JOU-KOU WANG TACHYON investigators |
Issue Date: | 2020 | Publisher: | Elsevier Ireland Ltd | Journal Volume: | 317 | Start page/Pages: | 49-55 | Source: | International Journal of Cardiology | Abstract: | Background: Prospective registry studies of congenital heart disease (CHD)-associated pulmonary artery hypertension (PAH) are rare. We established a multicenter registry of CHD-PAH: the TACHYON (TAiwan Congenital Heart disease associated with pulmonarY arterial hypertension) registry. Methods: The prospective TACHYON registry was initiated in January 2016. Nine pediatric cardiology centers with 99 patients were included. Using this database, we evaluated clinical characteristics and outcomes. Results: Twelve patients with incomplete data were excluded. For the remaining 87 patients, mean age of enrollment was 37.4 (SD 18.2) years, and the male to female ratio was 60:27. PAH after defect closure accounted for 46 (52.9%) and Eisenmenger syndrome for 30 (34.5%) cases. Atrial septal defect was the most common (48.3%) disease, followed by ventricular septal defect. Mean pulmonary artery pressure was 56.7 (SD 19.4) mmHg. PAH-targeted therapy was used in 95.4% of patients. Sildenafil and bosentan were the most common drugs. After mean 23.9 months of follow-up, the 2-year Kaplan–Meier survival rate was 93.2%. According to univariate Cox regression analysis, significant risk factors included right heart failure signs, symptom progression, high-risk baseline N-terminal pro–brain natriuretic peptide (BNP)/BNP, high-risk baseline 6-min walking distance (6MWD), and high baseline hemoglobin/hematocrit level. Using the three noninvasive parameters (functional class, 6MWD, NT-pro BNP/BNP) proposed by the European Society of Cardiology, the total number of high-risk criteria predicted survival rate reliably. Conclusions: Using the TACHYON registry is feasible, but the physicians' adherences to guidelines are unsatisfactory. Midterm outcomes of PAH-target therapy are favorable and predictable using noninvasive parameters. ? 2020 Elsevier B.V. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-85087205851&doi=10.1016%2fj.ijcard.2020.05.086&partnerID=40&md5=a654e1477842685cd5adb10693d06522 https://scholars.lib.ntu.edu.tw/handle/123456789/525231 |
ISSN: | 0167-5273 | DOI: | 10.1016/j.ijcard.2020.05.086 | SDG/Keyword: | amino terminal pro brain natriuretic peptide; bosentan; hemoglobin; sildenafil; adult; Article; clinical feature; clinical outcome; cohort analysis; congenital heart disease; disease exacerbation; Eisenmenger complex; feasibility study; female; follow up; heart atrium septum defect; heart failure; heart ventricle septum defect; hematocrit; human; lung artery pressure; major clinical study; male; mortality risk; predictor variable; priority journal; protocol compliance; pulmonary hypertension; register; risk factor; six minute walking distance; survival prediction; survival rate; walking distance; child; clinical trial; congenital heart malformation; diagnostic imaging; multicenter study; pulmonary artery; pulmonary hypertension; Taiwan; Adult; Child; Female; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Male; Pulmonary Arterial Hypertension; Pulmonary Artery; Registries; Taiwan |
Appears in Collections: | 醫學系 |
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