Yuling Qian, Ruilin Quan, Xiaoxi Chen, Qing Gu, Changming Xiong, Huijun Han, Gangcheng Zhang, Yucheng Chen, Zaixin Yu, Hongyan Tian, Yuhao Liu, Xianyang Zhu, Shengqing Li, Caojin Zhang, Jianguo He
Chinese Academy of Medical Sciences and Peking Union Medical College and Fuwai Hospital. School of Basic Medicine Peking Union Medical College. Wuhan Asia Heart Hospital. West China Hospital and Sichuan University. Xiangya Hospital and Central South University. First Affiliated Hospital and Medical College of Xi’an Jiaotong University. Henan Provincial People’s Hospital, Central China Fuwai Hospital and Zhengzhou University. General Hospital of Northern Theater Command. Xijing Hospital and Fourth Military Medical University. Guangdong Provincial People’s Hospital and Guangdong Academy of Medical Sciences.
China
Chest
Chest 2022;
DOI: 10.1016/j.chest.2022.11.038
Abstract
Background: Registry-based studies of pediatric pulmonary arterial hypertension (PPAH) are scarce in developing countries, including China. The PPAH risk assessment tool needs further evaluation and improvement.
Research question: What are the characteristics and long-term survival of PPAH in China and what is the performance of the PPAH risk model in Chinese patients?
Study design and methods: Patients with PAH were enrolled in the national prospective multicenter registry from August 2009 through December 2019. Children 3 months to 18 years of age at the time of PAH diagnosis were analyzed.
Results: A total of 247 children with PAH were enrolled. The median patient age was 14.8 years, and 58.3% of patients were female. Most patients had a diagnosis of PAH associated with congenital heart disease (CHD; 61.5%) and idiopathic or heritable PAH (37.7%). The median time from symptom onset to PAH diagnosis was 24 months. The mean pulmonary artery pressure and pulmonary vascular resistance index were 70.78 ± 19.80 mm Hg and 21.82 ± 11.18 Wood units/m2, respectively. Patients with CHD-associated PAH experienced a longer diagnostic delay and demonstrated higher pulmonary artery pressure, but better cardiac performance, than those with idiopathic or heritable PAH. An increased number of patients received targeted therapy at the last follow-up compared with baseline. The 5- and 10-year survival rates of the entire cohort were 74.9% and 55.7%, respectively, with better survival in patients with CHD-associated PAH than in those with idiopathic or heritable PAH. Patients with low risk had better survival than those with high risk according to the simplified noninvasive risk score model with weight, function class, and echocardiographic right ventricular size, both at baseline and follow-up.
Interpretation: Patients with PPAH in China showed severely compromised hemodynamics with marked diagnostic delay. The long-term survival of PPAH is poor despite the increased usefulness of targeted drugs. The simplified noninvasive risk model demonstrated good performance for predicting survival in Chinese children with PAH.
Category
Class I. Idiopathic Pulmonary Hypertension
Class I. Heritable Pulmonary Hypertension
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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