Survey of the current status and management of Eisenmenger syndrome: A Japanese nationwide survey

• Published in: Journal of cardiology Vol. 63; no. 4; pp. 286 - 290
• Main Authors: Inohara, Taku, MD, Niwa, Koichiro, MD, PhD, FJCC, Yao, Atsushi, MD, PhD, Inuzuka, Ryo, MD, PhD, Sakazaki, Hisanori, MD, Ohuchi, Hideo, MD, PhD, FJCC, Inai, Kei, MD, PhD, FJCC
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01.04.2014
• Subjects: Adult; Aged; Angiotensin Receptor Antagonists - therapeutic use; Angiotensin-Converting Enzyme Inhibitors - therapeutic use; Anticoagulants - therapeutic use; Cardiovascular; Congenital heart disease; Digitalis Glycosides - therapeutic use; Drug Therapy, Combination; Eisenmenger Complex - classification; Eisenmenger Complex - drug therapy; Eisenmenger Complex - epidemiology; Eisenmenger Complex - etiology; Eisenmenger syndrome; Endothelin Receptor Antagonists; Female; Heart failure; Humans; Incidence; Japan - epidemiology; Male; Middle Aged; Molecular Targeted Therapy; Practice Guidelines as Topic; Prevalence; Pulmonary hypertension; Pulmonary vasodilator; Surveys and Questionnaires; Young Adult; Japan; Heart failure; Congenital heart disease; Pulmonary vasodilator; Eisenmenger syndrome; Pulmonary hypertension
• ISSN: 0914-5087; 1876-4738
• DOI: 10.1016/j.jjcc.2013.08.014

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.