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Right atrial isomerism (RAI) itself predicts poor outcome for children with a normal pulmonary venous connection, conclude the authors of a large study in Hong Kong. In a retrospective review of surgical management and outcome of RAI in a tertiary paediatric cardiac centre over 20 years, Cheung et al analysed risk factors for mortality and survival rates in 116 infants and children with a normal pulmonary venous connection or abnormal connection, either obstructed or non-obstructed.
Most children presented early (median age 1 day, range 1 day-3.7 years). Various corrective surgical procedures were used (27% (4/15) children with an obstructed connection, 60% (27/45) with a non-obstructed connection, and 77% (43/56) with a normal connection). These included systemic pulmonary shunt, pulmonary vein repair, cavopulmonary shunt, Fontan procedure, banding of the pulmonary artery, and inserting a pacemaker. Most deaths were late deaths from infection (10) or sudden deaths associated with arrhythmia (six of eight children). Survival was similar in children with normal and non-obstructed abnormal pulmonary venous drainage (mean (SEM) survival at 1, 5, 10, 15 years 81 (5.3)%, 67 (6.6)%, 60 (7.8)%, and 43 (12)% respectively).
Overall, obstructed abnormal pulmonary venous drainage carried increased risk of mortality (relative risk 3.8, 95% confidence interval 1.7 to 8.3; p=0.001) but no independent risk factor was found for children with a normal connection, when analysed separately.
RAI—even with a normal pulmonary venous connection—has a poor prognosis. Apart from rigorous antibiotic prophylaxis and treatment, identifying predictors of arrhythmia might bring further benefit.