Characteristics, treatments and survival of pulmonary arterial hypertension associated with congenital heart disease in China: Insights from a national multicenter prospective registry.
Feng XieRuilin QuanGangcheng ZhangHongyan TianYucheng ChenZaixin YuCaojin ZhangYuhao LiuXianyang ZhuWeifeng WuXiulong ZhuZhenwen YangQing GuChangming XiongHuijun HanYingzhang ChengJianguo HeYanqing WuPublished in: The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation (2023)
Chinese PAH-CHD patients predominantly exhibit Eisenmenger syndrome and have significantly impaired exercise tolerance and right ventricular function at diagnosis, which are closely associated with long-term survival. PAH-targeted therapy including combination therapy showed a favorable effect on survival in PAH-CHD. The long-term survival of Chinese CHD-PAH patients remains to be improved.
Keyphrases
- pulmonary arterial hypertension
- end stage renal disease
- congenital heart disease
- combination therapy
- newly diagnosed
- ejection fraction
- chronic kidney disease
- prognostic factors
- polycyclic aromatic hydrocarbons
- clinical trial
- peritoneal dialysis
- physical activity
- patient reported outcomes
- quality improvement
- resistance training
- body composition