Outcome of infants with right atrial isomerism: is prognosis better with normal pulmonary venous drainage?
OBJECTIVE
To compare the outcome of infants and children who have right atrial isomerism and normal pulmonary venous drainage with those who have anomalous drainage, and to determine factors associated with poor outcome.


METHODS
Retrospective review of management and outcome of 116 infants and children determined to have right atrial isomerism between January 1980 and December 2000.


METHODS
Tertiary paediatric cardiac centre.


RESULTS
The 116 patients presented at a median of one day (range 1 day to 3.7 years) with cyanosis in the majority (96%). No interventions were planned in 31 (27%) patients, all of whom died. The early surgical mortality for pulmonary venous repair was 25% (2 of 8), Fontan procedure 26% (5 of 19), cavopulmonary shunting 7.7% (1 of 13), and systemic pulmonary arterial shunt insertion 1.9% (1 of 53). Late mortality was related to infection (n = 10), sudden death of unknown aetiology (n = 7, 5 with history of arrhythmia), and documented arrhythmia (n = 1). Patients with obstructed anomalous pulmonary venous drainage had the worst survival (p < 0.001). The mean (SEM) survival estimates for those with normal pulmonary venous drainage at 1, 5, 10, and 15 years was 81 (5.3)%, 67 (6.6)%, 60 (7.8)%, and 43 (12)%, respectively, similar to those for patients with non-obstructed anomalous drainage (p = 0.06). Independent risk factors for mortality included pulmonary venous obstruction (relative risk 3.8, p = 0.001) and a single ventricle (relative risk 2.9, p = 0.016). An analysis of only patients with normal pulmonary venous drainage identified no risk factors for mortality.


CONCLUSIONS
The long term outcome of infants and children with right atrial isomerism in association with a normal pulmonary venous drainage remains unfavourable. Sepsis and sudden death that may potentially be related to cardiac arrhythmia are major causes of late mortality.
Cheung
Y F
YF
Division of Paediatric Cardiology, Department of Paediatrics, Grantham Hospital, The University of Hong Kong, Hong Kong, People's Republic of China. xfcheung@hkucc.hku.hk
Cheng
V Y
VY
Chau
A K
AK
Chiu
C S
CS
Yung
T C
TC
Leung
M P
MP
D000465:Algorithms; D002423:Cause of Death; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000473:pathology; D005260:Female; D006325:Heart Atria / Q000002:abnormalities* / Q000601:surgery; D018610:Heart Bypass, Right / Q000379:methods; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care / Q000379:methods; D011379:Prognosis; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000502:physiology*; D011668:Pulmonary Veno-Occlusive Disease / Q000209:etiology / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis