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
Journal
International Journal of Cardiology
Journal Volume
317
Pages
49-55
Date Issued
2020
Author(s)
Weng K.-P.
Lin M.-C.
Wang J.-N.
Hwang B.-T.
Dai Z.-K.
Lin S.-M.
Chang J.-S.
Lin I.-C.
Hua Y.-C.
Wu J.-M.
TACHYON investigators
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.
SDGs
Other Subjects
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
Publisher
Elsevier Ireland Ltd
Type
journal article