title: The clinical characteristics of sudden cardiac arrest in asymptomatic patients with congenital heart disease.
abstract: Sudden cardiac arrest (SCA) is a major cause of death in patients with congenital heart disease (CHD). Systemic ventricular dysfunction is a reported risk factor for SCA. We retrospectively analyzed the medical records of 46 patients (age >6 years) who experienced SCA. The following underlying cardiac defects were observed: biventricular repair with affected subpulmonary right ventricle (n = 18, 39 %), biventricular repair with systemic right ventricle and Eisenmenger syndrome (n = 6 each, 13 %), Fontan circulation and unrepaired CHD (n = 5 each, 11 %), and others (n = 6, 13 %). Twenty-one patients (46 %) had no history of arrhythmias, and 21 of 43 (49 %) showed systemic ventricular ejection fraction >55 %. According to the New York Heart Association classification, 18 patients (39 %) were class I and 28 (61 %) were class II/III. SCA occurred at a younger age in class I (16 ± 5 years) than in the other classes (23 ± 10 years; P = 0.004). QRS duration was similar between the groups (136 ± 38 vs. 141 ± 50 ms; P not significant). Seven patients in class I (15 % of all SCAs) had no history of arrhythmias or features of hemodynamic abnormalities. The proportion of patients with biventricular repair and affected subpulmonary right ventricle was higher than that of patients with other defects, and the majority of SCA patients had more complicated defects than a simple repaired ventricular septal defect or an atrial septal defect. No symptoms of heart failure, history of arrhythmias, or features of hemodynamic abnormalities were observed in 15 % of the patients who experienced SCA. Prolonged QRS duration might be a predictor of SCA even in asymptomatic CHD patients. Prevention of SCA in CHD patients may require more detailed evaluation than is typically considered necessary.
authors.affiliation: Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka, 565-8565, Japan, ayamiya@ncvc.go.jp.
authors.forename: Aya
authors.initials: A
authors.lastname: Miyazaki
authors.forename: Heima
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Hideo
authors.initials: H
authors.lastname: Ohuchi
authors.forename: Kenji
authors.initials: K
authors.lastname: Yasuda
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Tsujii
authors.forename: Michio
authors.initials: M
authors.lastname: Matsuoka
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yazaki
authors.forename: Etsuko
authors.initials: E
authors.lastname: Tsuda
authors.forename: Osamu
authors.initials: O
authors.lastname: Yamada
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000150:complications*; D058070:Asymptomatic Diseases / Q000453:epidemiology; D002648:Child; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000209:etiology*; D004541:Eisenmenger Complex / Q000150:complications; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006333:Heart Failure / Q000150:complications; D006345:Heart Septal Defects, Ventricular / Q000150:complications; D006352:Heart Ventricles / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D013318:Stroke Volume; D018754:Ventricular Dysfunction; D055815:Young Adult