Sudden death in patients with right isomerism (asplenism) after palliation.

摘要: Abstract Objective: To define the risk of sudden death in patients with right isomerism (asplenia) after palliation Study design: A total 154 was identified from 1980 to 1999. Sudden defined as acute cardiovascular collapse which occurred within 24 hours. Results: 620 patient-years were evaluated. The 1-year and 5-year survival 72% 50%, respectively. There 22 unexpected deaths (14%, 35 events/1000 patient-years). tended occur infancy or early childhood (12 ± 9 months; median, months). mechanisms classified tachyarrhythmic 2 (9%), cardiac but nontachyarrrhythmic (sudden onset severe cyanosis) 15 (68%), noncardiac 5 (23%)(fulminant sepsis positive blood culture: streptococcus pneumonia (3), Eschericia coli (1), yeast-like organism (1)). incidence steadily decreased age until 3 years. Conclusion: initial remained high. related complex anomalies, a susceptibility fulminant infection, arrhythmia. (J Pediatr 2002;140:93-6)