title: Survey of the current status and management of Eisenmenger syndrome: a Japanese nationwide survey.
abstract: BACKGROUND
  The management of Eisenmenger syndrome (ES) has dramatically changed since the advent of disease-targeted therapy (DTT). However, guidelines for ES management, including DTT, have not been established. We aimed to clarify the current incidence, underlying disease, and management of ES in Japan, using a nationwide survey.
  
  
  METHODS
  A written questionnaire was sent to members of the Japanese Society for Adult Congenital Heart Disease, through which information was obtained from 86 institutions.
  
  
  RESULTS
  A total of 251 patients with ES (80.5% cases≥20 years of age) were followed as of February 2012; DTT was performed in 124 (49.4%) patients. Unrepaired simple anatomy was reported as an underlying condition in 165 patients (65.7%). Among patients with ES, 55 (21.9%), 128 (51%), 53 (21.1%), and 12 (4.8%) were classified into functional classes I, II, III, and IV, respectively. DTT was routinely performed at 52 (60.5%) institutions, but there were variations in the DTT therapeutic strategy at these institutions. Combined therapy was more often used than monotherapy; an endothelin receptor antagonist was the most frequently prescribed medication. There were institutional differences regarding heart failure treatment and indications for anticoagulation. Digitalis and angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers were widely used, but beta-blockers were infrequently used to manage heart failure.
  
  
  CONCLUSIONS
  This survey describes the current status, including prevalence and underlying disease, and variations in the practical management of ES in Japan. The results will help in the creation of future guidelines for ES management.
authors.affiliation: Department of Cardiology, Keio University School of Medicine, Tokyo, Japan; Department of Cardiology, Cardiovascular Center, St. Luke's International Hospital, Tokyo, Japan. Electronic address: taku.inohara@gmail.com.
authors.forename: Taku
authors.initials: T
authors.lastname: Inohara
authors.affiliation: Department of Cardiology, Cardiovascular Center, St. Luke's International Hospital, Tokyo, Japan.
authors.forename: Koichiro
authors.initials: K
authors.lastname: Niwa
authors.affiliation: Division for Health Service Promotion, The University of Tokyo, Tokyo, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Yao
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Inuzuka
authors.affiliation: Department of Pediatric Cardiology, Hyogo Prefectural Amagasaki Hospital, Amagasaki, Japan.
authors.forename: Hisanori
authors.initials: H
authors.lastname: Sakazaki
authors.affiliation: Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan.
authors.forename: Hideo
authors.initials: H
authors.lastname: Ohuchi
authors.affiliation: Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kei
authors.initials: K
authors.lastname: Inai
mesh_terms: D000328:Adult; D000368:Aged; D057911:Angiotensin Receptor Antagonists / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D004071:Digitalis Glycosides / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004541:Eisenmenger Complex / Q000145:classification / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D065128:Endothelin Receptor Antagonists; D005260:Female; D006801:Humans; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D058990:Molecular Targeted Therapy; D017410:Practice Guidelines as Topic; D015995:Prevalence; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D000925:Anticoagulants; D004071:Digitalis Glycosides; D065128:Endothelin Receptor Antagonists
keywords: Congenital heart disease; Eisenmenger syndrome; Heart failure; Pulmonary hypertension; Pulmonary vasodilator