Clinical characteristics and prognosis of pulmonary artery hypertension secondary to congenital heart disease and idiopathic pulmonary artery hypertension
Xu Zhuoyuan
Li Qiangqiang
Liu Qian
Zhang Chen
Zhang Dan
Zhang Hongsheng
Liu Yinglong
Gu Hong
Abstract：Objective To analyze clinical manifestations,functional indexes,hemodynamic features and survival condition in patients with idiopathic pulmonary artery hypertension(IPAH) and pulmonary artery hypertension (PAH) secondary to congenital heart disease(CHD).Methods Clinical data of 88 patients with PAH who were hospitalized in Pediatric Cardiac Center of Beijing Anzhen Hospital,Capital Medical University from January 2010 to December 2016 were retrospectively reviewed,including 60 cases of IPAH and 28 cases of PAH secondary to CHD after cardiac defect correction.Clinical features and long-term prognosis of patients were analyzed.Results The average age of 88 patients was (18 ± 12) years old (0.8-50.2 years old).The median follow-up time was 4.4 years.Pulmonary artery systolic pressure,pulmonary artery mean pressure and pulmonary arteriolar resistance index estimated by echocardiography and cardiac catheterization showed no significant differences between PAH secondary to CHD group and IPAH group [(82 ± 25) mmHg vs (76 ± 22) mmHg,(58 ± 18) mmHg vs (62 ± 18)mmHg,(13 ±4)Wood/m2 vs (18 ± 10)Wood/m2] (P ＞ 0.05).The level of B-type natriuretic peptide and the ratio of right ventricular internal diameter/left ventricular internal diameter in PAH secondary to CHD group were significantly lower than those in IPAH group[18.2(9.2,25.4) ng/L vs 681.0(53.0,728.0) ng/L,(0.53 ± 0.18) vs (0.87 ± 0.41)] (P ＜ 0.05).In IPAH group,16 patients (18.2％) died during follow-up period;36 patients (60.0％) had cardiac complications.In PAH secondary to CHD group,no patient died and 5 cases(17.9％)had cardiac complications.The postoperative complication rate in PAH secondary to CHD group was significantly lower than that in IPAH group (P ＜ 0.05).The survival rate in PAH secondary to CHD group was significantly higher than that in IPAH group [100.0％ (28/28) vs 73.3 ％ (44/60)] (P ＜ 0.05).Conclusion Patients with PAH secondary to CHD have similar hemodynamic features but better heart function and prognosis compared to patients with IPAH.
Keywords：Congenital heart diseasePulmonary artery hypertensionCardiac defect correctionIdiopathic pulmonary artery hypertension
Publication Date：2017-01-01
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)
Pages：4( 1794-1797 )