title: Eisenmenger syndrome: not always inoperable.
abstract: Recently, advanced therapies for pulmonary arterial hypertension have become available, and have been effective in reducing pulmonary vascular resistance and symptoms in patients with Eisenmenger syndrome, previously thought to be inoperable. This review summarizes the current knowledge  on the pathophysiology and treatment of Eisenmenger syndrome. The recent introduction of targeted therapies in pulmonary arterial hypertension has led to a renewed insight in the pathophysiology and treatment of Eisenmenger syndrome. Patients with Eisenmenger syndrome using a diagnostic-treatment-and-repair  strategy are amenable to surgery after successful treatment with advanced therapy. With continued improvements in the diagnosis, preoperative management, refinement of surgical techniques and intra- and postoperative management strategies, the patients with Eisenmenger syndrome selected using  a diagnostic-treatment-and-repair strategy are operable with safety and efficacy in the current era with advanced pulmonary arterial hypertension therapy. Future directions of Eisenmenger syndrome may be the combination of reversal of pulmonary vascular remodeling and correction.
authors.affiliation: Department of Cardiothoracic Surgery, Affiliated Ruikang Hospital of Guangxi Traditional Chinese Medical College, Nanning, Guangxi Zhuang Autonomous Region, China. b2007117@hotmail.com
authors.forename: Jing-Bin
authors.initials: JB
authors.lastname: Huang
authors.forename: Jian
authors.initials: J
authors.lastname: Liang
authors.forename: Li-Yan
authors.initials: LY
authors.lastname: Zhou
mesh_terms: D004541:Eisenmenger Complex / Q000503:physiopathology* / Q000601:surgery*; D016232:Endothelins / Q000037:antagonists & inhibitors; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000601:surgery; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D016232:Endothelins; D014665:Vasodilator Agents