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31st Annual Meeting Abstracts: Contemporary Management of Right Atrial Isomerism: Impact of Evolving Therapeutic Strategies
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P19. Contemporary Management of Right Atrial Isomerism: Impact of Evolving Therapeutic Strategies
T. Yun1, O. O. Al- Radi2, C. A. Caldarone*2, J. G. Coles*2, W. G. Williams*2, G. S. Van Arsdell*2. 1Asan Medical Center, Seoul, Republic of Korea, 2The Hospital for Sick Children in Toronto, Toronto, ON, Canada,
BACKGROUND:Infants with right atrial isomerism (RAI) have poor outcomes due to complex combination of cardiac anomalies. Aggressive management of total anomalous pulmonary venous drainage (TAPVD) may have a positive impact on the prognosis.
METHODS:Outcomes of all children with RAI from 1994 to present were retrospectively reviewed. Management of TAPVD evolved from no repair or conventional surgical tecnique to primary sutureless repair (SR) upon initial palliation. Cox survival models were used to identify variables associated with reduced survival.
RESULTS:There were 55 children (34 males). Median age at initial visit was 2 days. Withdrawal of treatment occurred in 11 (11/55, 20%) during an interval of institutional bias towards no treatment . Thirteen patients (13/55, 24%) had palliations without TAPVD repair - 3 survived (3/13, 23%). Thirty-one patients (31/55, 56%) had operations that included TAPVD repair, of whom 13 (13/31, 42%) underwent primary SR for TAPVD. Sixteen patients survived (16/31, 52%), and their current status 1 to 10 yrs (median: 5.8 yrs) after repair is post-Fontan (7/16, 44%), post-bidrectional Glenn (6/16, 38%), and others (3, 20%). In patients who underwent TAPVD repair (n=31), two risk factors of decreased survival were identified: drainage site obstruction, infracardiac or mixed type TAPVD. After adjustment, SR was associated with improved survival, HR of 0.43, but this beneficial effect failed to reach statistical significance (p= 0.19).
CONCLUSIONS: Although mortality is still high, aggressive TAPVD repair for RAI has resulted in improved survival.The role of priamry SR for TAPVD associaved with RAI has remained to be defined.
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