IS PROGNOSIS BETTER IN INFANTS AND CHILDREN WITH RIGHT ATRIAL
ISOMERISM AND NORMAL PULMONARY VENOUS DRAINAGE?
Cheung YF, Cheng
YW, Chiu SW*, Yung TC, Chau AKT, Leung MP
Divisions of Paediatric
Cardiology and Cardiothoracic Surgery*, Grantham Hospital, The University
of Hong Kong, China
Objective: In contrast to the uniformly
poor prognosis of patients with right atrial isomerism and obstructed
anomalous pulmonary venous (PV) connection, the outcome of those with
normal PV connection is undefined.
We compared the outcome of the 2 groups and determined factors
associated with poor outcome.
Methods: The clinical data of 116
patients (71 boys) diagnosed with right atrial isomerism between 1980 and
2000, the largest clinical series to date, were reviewed. Actuarial survival was assessed by
Kaplan-Meier estimates, while effects of covariates on survival were
analyzed by Cox regression model.
Results: The median age at presentation
was 1 day (range 1 day to 3.7 years).
The usual combination of cardiac morphologic anomalies included
levocardia (70%), common atrium (59%), common atrioventricular valve (92%),
single main ventricle (83%), single outlet with pulmonary atresia (41%) or
double-outlet right ventricle (25%), pulmonary outflow obstruction (83%), anomalous
PV drainage (51%) and PV obstruction (15%). Surgery was not planned in 27% (31/116) of patients who
all died. The early surgical
mortality for PV repair was 25% (2/8), Fontan procedure 26% (5/19),
cavopulmonary shunt 7.7% (1/13), and systemic-pulmonary arterial shunt 1.9%
(1/53). Late mortality was
related to infection (n=10), sudden death of unknown aetiology (n=7) and
arrhythmia (n=1). The mean
(SE) survival estimates of patients with normal PV connection was 81(5.3)%,
67(6.6)%, 60(7.8)% and 43 (12)% at 1, 5, 10 and 15 years, respectively,
similar to those with non-obstructed anomalous PV connection (p=0.06). All except 1 patient with
obstructed PV connection died.
Independent risk factors for increased mortality were PV obstruction
(relative risk [RR] 3.8, p=0.001) and a single ventricle (RR 2.9, p=0.016).
Conclusion: The long-term outcome of
children with right atrial isomerism in association with normal PV
obstruction remains unfavourable, despite avoiding the high-risk PV repair.