MEI-HWAN WUJOU-KOU WANGLue H.-C.2021-01-052021-01-0520020022-3476https://www.scopus.com/inward/record.uri?eid=2-s2.0-0036161748&doi=10.1067%2fmpd.2002.120510&partnerID=40&md5=86ec234782ced194150e6868239221cahttps://scholars.lib.ntu.edu.tw/handle/123456789/538460Objective: To define the risk of sudden death in patients with right isomerism (asplenia) after palliation. Study design: A total of 154 patients with right isomerism was identified from 1980 to 1999. Sudden death was defined as acute cardiovascular collapse from which death occurred within 24 hours. Results: A total of 620 patient-years were evaluated. The 1-year and 5-year survival was 72% and 50%, respectively. There were 22 sudden unexpected deaths (14%, 35 events/1000 patient-years). Sudden death tended to occur in infancy or early childhood (12 ± 9 months; median, 9 months). The mechanisms were classified as sudden tachyarrhythmic in 2 (9%), sudden cardiac but nontachyarrrhythmic (sudden onset severe cyanosis) in 15 (68%), and sudden noncardiac in 5 (23%)(fulminant sepsis with positive blood culture: streptococcus pneumonia (3), Eschericia coli (1), and yeast-like organism (1)). The incidence of sudden death steadily decreased with age until the age of 3 years. Conclusion: The incidence of sudden death in patients with right isomerism after initial palliation remained high. Sudden death was related to complex cardiac anomalies, a susceptibility to fulminant infection, and arrhythmia.[SDGs]SDG3article; asplenia; blood culture; cause of death; congenital heart disease; cyanosis; disease severity; Escherichia coli; female; heart death; heart disease; heterotaxy syndrome; human; incidence; infant; isomerism; major clinical study; male; newborn; palliative therapy; preschool child; priority journal; sepsis; Streptococcus pneumonia; sudden death; survival rate; tachycardia; yeastjournal article10.1067/mpd.2002.120510118157702-s2.0-0036161748